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1. Update of the Spanish clinical practice guideline for urinary tract infection in infants and children. Summary of recommendations for diagnosis, treatment and follow-up

Author

Juan David González Rodríguez, Gloria María Fraga Rodríguez, César Joaquín García Vera, Andrés Gómez Fraile, Juan Ignacio Martín Sánchez, José María Mengual Gil, Carlos Ochoa Sangrador, Blanca Valenciano Fuentes, and Joaquín Escribano Subías

Subjects
Cistitis, Diagnóstico, Guía de práctica clínica, Infección urinaria, Niños, Pielonefritis, Pediatrics, RJ1-570
Abstract

The management of urinary tract infection (UTI) in infants and children has changed significantly over the past few decades based on scientific evidence that questioned the efficacy of strategies used to prevent kidney injury and subsequent progression to chronic kidney disease, which is very unlikely in most paediatric cases. However, there is still substantial heterogeneity in its management and uncertainty regarding the diagnosis, indication of imaging tests, treatment or follow-up in these patients. The Spanish clinical practice guideline has been updated through the review of the literature published since 2009 and a rigorous evaluation of current clinical practice aspects, taking into account the evidence on the benefits of each intervention in addition to its risks and drawbacks to attempt to provide more precise recommendations. Resumen: En las últimas décadas, se han producido profundos cambios en el manejo de la infección urinaria en pediatría, a partir de estudios que cuestionaban la eficacia de las estrategias para prevenir el daño renal y la evolución posterior a enfermedad renal crónica, muy poco probable en la mayoría de casos. No obstante, todavía existe una gran variabilidad clínica en el manejo e incertidumbre en cuestiones relativas al diagnóstico, la indicación de una exploración radiológica, el tratamiento o el seguimiento de estos niños. La actualización de la guía de práctica clínica española ha examinado en la literatura publicada desde el año 2009 y de modo riguroso aspectos de la práctica actual, considerando la evidencia de los beneficios de cada intervención, junto con los riesgos asociados y desventajas, para intentar delimitar las pautas de actuación más precisas.

Published
2024
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3. Predictors of the response to phosphodiesterase-5 inhibitors in pulmonary arterial hypertension: an analysis of the Spanish registry

Author

Agustin R. Garcia, Isabel Blanco, Lluis Ramon, Jesús Pérez-Sagredo, Francisco J. Guerra-Ramos, Clara Martín-Ontiyuelo, Olga Tura-Ceide, Francisco Pastor-Pérez, Pilar Escribano-Subías, and Joan A. Barberà

Subjects
Pulmonary arterial hypertension, Vasodilator agents, Phosphodiesterase-5 inhibitors, Sildenafil citrate, Tadalafil, Treatment outcome, Diseases of the respiratory system, RC705-779
Abstract

Abstract Background Achieving and maintaining a low-risk profile is associated with favorable outcome in pulmonary arterial hypertension (PAH). The effects of treatment on risk profile are variable among patients. Objective To Identify variables that might predict the response to treatment with phosphodiesterase-5 inhibitors (PDE-5i) in PAH. Methods We carried out a cohort analysis of the Spanish PAH registry in 830 patients diagnosed with PAH that started PDE5i treatment and had > 1 year follow-up. 644 patients started PDE-5i either in mono- or add-on therapy and 186 started combined treatment with PDE-5i and endothelin receptor antagonist (ERA). Responders were considered when at 1 year they: (1) were alive; (2) did not present clinical worsening; and (3) improved European Society of Cardiology/European Respiratory Society (ESC/ERS) risk score or remained in low-risk. Univariate and multivariate logistic regression models were used to analyze variables associated with a favorable response. Results Two hundred and ten patients (33%) starting PDE-5i alone were classified as responders, irrespective of whether it was mono- or add-on therapy. In addition to known predictors of PAH outcome (low-risk at baseline, younger age), male sex and diagnosis of portopulmonary hypertension (PoPH) or HIV-PAH were independent predictors of favorable response to PDE-5i. Diffusing capacity for carbon monoxide (DLco) ≤ 40% of predicted was associated with an unfavorable response. When PDE-5i were used in upfront combination, 58% of patients were responders. In this group, diagnosis of idiopathic PAH (IPAH) was an independent predictor of favorable response, whereas connective tissue disease-PAH was associated with an unfavorable response. Conclusion Male sex and diagnosis of PoPH or HIV-PAH are predictors of favorable effect of PDE-5i on risk profile when used as mono- or add-on therapy. Patients with IPAH respond more favorably to PDE-5i when used in upfront combination. These results identify patient profiles that may respond favorably to PDE-5i in monotherapy and those who might benefit from alternative treatment strategies.

Published
2023
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4. Assessing the clinical benefit, safety, and patient-reported outcomes with the use of the PAHcare™ digital platform in pulmonary arterial hypertension: a pilot study

Author

Gregorio Pérez Peñate, Nuria Ochoa Parra, Juan Antonio Domingo Morera, Amaya Martínez Meñaca, Marta López Ramón, Sergio Cadenas Menéndez, Fernando León Marrero, Sara Gómara de la Cal, Cristina Ghadban-Garrido, Patricia Royo Tolosana, Javier Martín Puentes, Rebeca Aldonza Aguayo, Hadis Mahdavi, Gabriela Bacchini Jeanneret, and Pilar Escribano Subías

Subjects
pulmonary arterial hypertension, digital intervention platform, mobile health (mHealth), electronic patient-reported outcome, health services research, patient support program, Public aspects of medicine, RA1-1270
Abstract

IntroductionDigital health interventions, particularly mobile health platforms, have shown promise in supporting patients with respiratory conditions, but their application in pulmonary arterial hypertension (PAH) remains limited. We aimed to assess the feasibility, acceptability, and potential clinical benefit of the novel PAHcare™ digital platform as a patient-centred intervention for PAH management through a prospective, single-arm, multicenter pilot study conducted on 53 patients diagnosed with PAH who used the platform for 6 months.MethodsThe primary objective was to assess the impact on Health-Related Quality of Life (HRQoL) through questionnaires. Secondary objectives included evaluating clinical outcomes, including disease progression, PAH signs and symptoms, the 6-min walking test, and the patient’s symptom perception. Additionally, we assessed patient satisfaction and engagement with the PAHcare™ platform, interaction with health coaches, retention, costs and healthcare resource utilisation (HCRU), and safety through monitoring device incidents.ResultsMinimal changes in HRQoL and clinical outcomes were observed over 6 months. A noteworthy 92.4% of patients actively used the platform in the first month, maintaining high usage throughout the study. Patient satisfaction was substantial, with more than half of the patients expressing excellence in service quality, willingness to reuse the platform, and fulfilment of their needs. Health coach interaction was high, with 76% of patients initiating contact within the first week. User retention rates were 70%, with prevalent ongoing usage and interaction with healthcare professionals even after the study. In terms of HCRU and costs, the study showed no significant changes in PAH-related hospital admissions, clinical visits, or tests. Finally, the low number of device-related incidents indicated platform safety.ConclusionThis pilot study provides compelling evidence supporting the feasibility and acceptability of the PAHcare™ digital platform to empower patients to manage their disease and significantly enhance their overall experience with PAH.

Published
2024
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5. Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension

Author

Alfredo Guillén-Del-Castillo, Manuel López Meseguer, Vicent Fonollosa-Pla, Berta Sáez Giménez, Dolores Colunga-Argüelles, Eva Revilla-López, Manuel Rubio-Rivas, Maria Jose Cristo Ropero, Ana Argibay, Joan Albert Barberá Mir, Xavier Pla Salas, Amaya Martínez Meñaca, Ana Belén Madroñero Vuelta, Antonio Lara Padrón, Luis Sáez Comet, Juan Antonio Domingo Morera, Cristina González-Echávarri, Teresa Mombiela, Norberto Ortego-Centeno, Manuela Marín González, Carles Tolosa-Vilella, Isabel Blanco, Pilar Escribano Subías, Carmen Pilar Simeón-Aznar, RESCLE Consortium, and REHAP Consortium

Subjects
Medicine, Science
Abstract

Abstract To assess severity markers and outcomes of patients with systemic sclerosis (SSc) with or without pulmonary arterial hypertension (PAH-SSc/non-PAH-SSc), and the impact of interstitial lung disease (ILD) on PAH-SSc. Non-PAH-SSc patients from the Spanish SSc registry and PAH-SSc patients from the Spanish PAH registry were included. A total of 364 PAH-SSc and 1589 non-PAH-SSc patients were included. PAH-SSc patients had worse NYHA-functional class (NYHA-FC), worse forced vital capacity (FVC) (81.2 ± 20.6% vs 93.6 ± 20.6%, P

Published
2022
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6. Evaluation of a digital health system (PAHcare™) for routine care of patients with pulmonary arterial hypertension: The CBS-PAH study protocol

Author

Gregorio Pérez Peñate, Nuria Ochoa Parra, Juan Antonio Domingo Morera, Amaya Martínez Meñaca, Marta López Ramón, Sergio Cadenas Menéndez, Fernando León Marrero, Sara Gómara de la Cal, Cristina Ghadban Garrido, Patricia Royo Tolosana, Javier Martin Puentes, Rebeca Aldonza Aguayo, Hadis Mahdavi, Gabriela Bacchini Jeanneret, and Pilar Escribano Subías

Subjects
pulmonary arterial hypertension, digital intervention, mobile Health, electronic patient-reported outcome, health services research, quality of life, Public aspects of medicine, RA1-1270
Abstract

IntroductionPulmonary arterial hypertension (PAH) is a rare, multifactorial, chronic condition that requires ongoing monitoring and assessment. PAHcare™ is a novel, patient-centered digital platform that provides software intended for use on patients' mobile phones (downloadable application) and web-based dashboards for use by physicians and health coaches (HC). We describe herein the protocol of a clinical study aimed at evaluating the clinical benefit and safety of PAHcare™ for the routine management of patients with PAH.Methods and analysisIn this prospective, single cohort, multicenter study, 50 patients with PAH will be recruited at six specialized PAH units from reference hospitals of the public Spanish healthcare system. The PAHcare™ digital health platform allows patients to log health and lifestyle information while also providing structured content for patient education, medication reminders, and behavioral and lifestyle coaching from a remote HC. Evaluation will be primarily focused on the impact of the platform use on the patient's health-related quality of life (HRQoL) via questionnaires completion through electronic patient-reported outcomes. Moreover, the analysis of the impact on the patient's functional status, signs and symptoms of PAH, patient costs and healthcare resource utilization, satisfaction, knowledge of the disease and its management, and adherence to and safety of the platform will be secondary outcomes. The clinical investigation started in July 2021 and is expected to end by September 2022.DiscussionThe PAHcare™ platform is anticipated to provide direct benefits to healthcare professionals, patients, and caregivers. These include the simplification of the multidisciplinary approach needed to tailor routine PAH management, enhancement of the patient/healthcare professional interaction, patient's empowerment to become more actively involved in the management and treatment of the disease, and increase of the patient's and caregiver's knowledge on PAH.

Published
2022
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7. Tromboendarterectomía pulmonar en pacientes con hipertensión pulmonar tromboembólica crónica y afectación distal

Author

Consuelo A. Gotor-Pérez, María J. López-Gude, Victoria Benito-Arnaiz, Enrique Pérez de la Sota, Jorge E. Centeno-Rodríguez, Andrea Eixerés-Esteve, Eva M. Aguilar-Blanco, Carolina Barajas-Díaz, María T. Velázquez-Martín, Marta Pérez-Núñez, José L. Pérez-Vela, Pilar Escribano-Subías, and José M. Cortina-Romero

Subjects
Pulmonary hypertension, Endarterectomy, Pulmonary embolism, Surgical procedures, Treatment outcome, Medicine, Surgery, RD1-811
Abstract

Resumen: Introducción y objetivos: La hipertensión pulmonar tromboembólica crónica es una rara consecuencia de la embolia pulmonar aguda. La cirugía de tromboendarterectomía pulmonar es el tratamiento de elección. Cuando la afectación es distal (nivel iii anatomoquirúrgico o segmentario) supone un mayor desafío quirúrgico y presenta mayor riesgo de morbimortalidad perioperatoria. Analizamos los resultados de la tromboendarterectomía pulmonar en pacientes con hipertensión pulmonar tromboembólica crónica nivel i-ii y nivel iii en nuestro centro. Métodos: Desde febrero de 1996, hemos realizado 290 tromboendarterectomías pulmonares consecutivas, 260 con seguimiento mínimo de un año (media de 67 meses [rango 17-279]). Dividimos esta población en grupo 1 (210 pacientes con hipertensión pulmonar tromboembólica crónica nivel i y ii) y grupo 2 (50 pacientes con hipertensión pulmonar tromboembólica crónica nivel iii [19%]) Resultados: La supervivencia al año fue del 94% en el grupo 1 y del 96% en el grupo 2, a los 3 años, del 93 y el 96%, y a los 5 años, del 90 y el 96%, respectivamente (p = 0,352). La mortalidad hospitalaria (6% vs. 4%, p = 0,473), la oxigenación con membrana extracorpórea perioperatoria (6% vs. 0%, p = 0,058), la tasa de ventilación mecánica de más de 48 horas (34% vs. 42%, p = 0,306) y la hipertensión pulmonar residual (resistencias vasculares pulmonares superiores a 400 dinas.s.cm−5) a los 6 meses de seguimiento (22% vs. 19%, p = 0,680) no presentaron diferencias estadísticamente significativas. Conclusiones: La tromboendarterectomía pulmonar en hipertensión pulmonar tromboembólica crónica con afectación distal o segmentaria, cuando es realizada en un centro experto, puede obtener resultados a corto-medio plazo excelentes y similares a los pacientes intervenidos con afectación más proximal. Abstract: Introduction and objectives: Chronic thromboembolic pulmonary hypertension is a rare consequence of an acute pulmonary embolism. Pulmonary thromboendarterectomy is the treatment of choice. It represents a greater surgical challenge in distal chronic thromboembolic disease (level iii, segmental). It also presents a higher risk of perioperative morbidity and mortality. We analyzed the results of pulmonary thromboendarterectomy in patients with level i-ii and level iii chronic thromboembolic pulmonary hypertension in our center. Methods: Since February 1996, we have performed 290 consecutive pulmonary thromboendarterectomy, 260 of them with a minimum follow-up of one year (mean of 67 months [rank 17-279]). We divided this population into group 1 (210 patients with chronic thromboembolic pulmonary hypertension levels i and ii) and group 2 (50 patients with chronic thromboembolic pulmonary hypertension level iii [19%]). Results: Survival rate was 94% in group 1 and 96% in group 2 after one year, 93% and 96% after 3 years, and 90% and 96% after 5 years, respectively (P=.352). No statistically significant differences were found regarding hospital mortality (6% vs. 4%, P=.473), perioperative extracorporeal membrane oxigenation (6% vs. 0%, P=.058), the rate of mechanical ventilation for more 48 hours (34 vs. 42%, P=.306) and residual pulmonary hypertension (pulmonary vascular resistance greater than 400 dynes/sec/cm−5) at 6 months of follow-up (22% vs. 19%, P=.680). Conclusions: Pulmonary thromboendarterectomy in chronic thromboembolic pulmonary hypertension with distal involvement, when performed in an expert center, can obtain short-to-medium-term results excellent and similar to those of patients operated on with more proximal involvement.

Published
2020
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8. Author Correction: Impact of interstitial lung disease on the survival of systemic sclerosis with pulmonary arterial hypertension

Author

Alfredo Guillén-Del-Castillo, Manuel López Meseguer, Vicent Fonollosa-Pla, Berta Sáez Giménez, Dolores Colunga-Argüelles, Eva Revilla-López, Manuel Rubio-Rivas, Maria Jose Cristo Ropero, Ana Argibay, Joan Albert Barberá, Xavier Pla Salas, Amaya Martínez Meñaca, Ana Belén Madroñero Vuelta, Antonio Lara Padrón, Luis Sáez Comet, Juan Antonio Domingo Morera, Cristina González-Echávarri, Teresa Mombiela, Norberto Ortego-Centeno, Manuela Marín González, Carles Tolosa-Vilella, Isabel Blanco, Pilar Escribano Subías, Carmen Pilar Simeón-Aznar, RESCLE Consortium, and REHAP Consortium

Subjects
Medicine, Science
Published
2022
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9. Performance of Prognostic Risk Scores in Chronic Heart Failure Patients Enrolled in the European Society of Cardiology Heart Failure Long-Term Registry

Author

Crespo-Leiro, M., Anker, S., Mebazaa, A., Coats, A., Filippatos, G., Ferrari, R., Maggioni, A.P., Piepoli, M.F., Amir, O., Chioncel, O., Dahlström, U., Delgado Jimenez, J.F., Drozdz, J., Erglis, A., Fazlibegovic, E., Fonseca, C., Fruhwald, F., Gatzov, P., Goncalvesova, E., Hassanein, M., Hradec, J., Kavoliuniene, A., Lainscak, M., Logeart, D., Merkely, B., Metra, M., Otljanska, M., Seferovic, P.M., Srbinovska Kostovska, E., Temizhan, A., Tousoulis, D., Ferreira, T., Andarala, M., Fiorucci, E., Folkesson Lefrancq, E., Glémot, M., Gracia, G., Konte, M., Laroche, C., McNeill, P.A., Missiamenou, V., Taylor, C., Auer, J., Ablasser, K., Dolze, T., Brandner, K., Gstrein, S., Poelzl, G., Moertl, D., Reiter, S., Podczeck-Schweighofer, A., Muslibegovic, A., Vasilj, M., Cesko, M., Zelenika, D., Palic, B., Pravdic, D., Cuk, D., Vitlianova, K., Katova, T., Velikov, T., Kurteva, T., Kamenova, D., Antova, M., Sirakova, V., Krejci, J., Mikolaskova, M., Spinar, J., Krupicka, J., Malek, F., Hegarova, M., Lazarova, M., Monhart, Z., Sobhy, M., El Messiry, F., El Shazly, A.H., Elrakshy, Y., Youssef, A., Moneim, A.A., Noamany, M., Reda, A., Abdel Dayem, T.K., Farag, N., Ibrahim Halawa, S., Abdel Hamid, M., Said, K., Saleh, A., Ebeid, H., Hanna, R., Aziz, R., Louis, O., Enen, M.A., Ibrahim, B.S., Nasr, G., Elbahry, A., Sobhy, H., Ashmawy, M., Gouda, M., Aboleineen, W., Bernard, Y., Luporsi, P., Meneveau, N., Pillot, M., Morel, M., Seronde, M.-F., Schiele, F., Briand, F., Delahaye, F., Damy, T., Eicher, J.-C., de Groote, P., Fertin, M., Lamblin, N., Isnard, R., Lefol, C., Thevenin, S., Hagege, A., Jondeau, G., Le Marcis, V., Ly, J.-F., Coisne, D., Lequeux, B., Le Moal, V., Mascle, S., Lotton, P., Behar, N., Donal, E., Thebault, C., Ridard, C., Reynaud, A., Basquin, A., Bauer, F., Codjia, R., Galinier, M., Tourikis, P., Stavroula, M., Stefanadis, C., Chrysohoou, C., Kotrogiannis, I., Matzaraki, V., Dimitroula, T., Karavidas, A., Tsitsinakis, G., Kapelios, C., Nanas, J., Kampouri, H., Nana, E., Kaldara, E., Eugenidou, A., Vardas, P., Saloustros, I., Patrianakos, A., Tsaknakis, T., Evangelou, S., Nikoloulis, N., Tziourganou, H., Tsaroucha, A., Papadopoulou, A., Douras, A., Polgar, L., Kosztin, A., Nyolczas, N., Csaba Nagy, A., Halmosi, R., Elber, J., Alony, I., Shotan, A., Vazan Fuhrmann, A., Romano, S., Marcon, S., Penco, M., Di Mauro, M., Lemme, E., Carubelli, V., Rovetta, R., Bulgari, M., Quinzani, F., Lombardi, C., Bosi, S., Schiavina, G., Squeri, A., Barbieri, A., Di Tano, G., Pirelli, S., Fucili, A., Passero, T., Musio, S., Di Biase, M., Correale, M., Salvemini, G., Brognoli, S., Zanelli, E., Giordano, A., Agostoni, P., Italiano, G., Salvioni, E., Copelli, S., Modena, M.G., Reggianini, L., Valenti, C., Olaru, A., Bandino, S., Deidda, M., Mercuro, G., Cadeddu Dessalvi, C., Marino, P.N., Di Ruocco, M.V., Sartori, C., Piccinino, C., Parrinello, G., Licata, G., Torres, D., Giambanco, S., Busalacchi, S., Arrotti, S., Novo, S., Inciardi, R.M., Pieri, P., Chirco, P.R., Ausilia Galifi, M., Teresi, G., Buccheri, D., Minacapelli, A., Veniani, M., Frisinghelli, A., Priori, S.G., Cattaneo, S., Opasich, C., Gualco, A., Pagliaro, M., Mancone, M., Fedele, F., Cinque, A., Vellini, M., Scarfo, I., Romeo, F., Ferraiuolo, F., Sergi, D., Anselmi, M., Melandri, F., Leci, E., Iori, E., Bovolo, V., Pidello, S., Frea, S., Bergerone, S., Botta, M., Canavosio, F.G., Gaita, F., Merlo, M., Cinquetti, M., Sinagra, G., Ramani, F., Fabris, E., Stolfo, D., Artico, J., Miani, D., Fresco, C., Daneluzzi, C., Proclemer, A., Cicoira, M., Zanolla, L., Marchese, G., Torelli, F., Vassanelli, C., Voronina, N., Tamakauskas, V., Smalinskas, V., Karaliute, R., Petraskiene, I., Kazakauskaite, E., Rumbinaite, E., Vysniauskas, V., Brazyte-Ramanauskiene, R., Petraskiene, D., Stankala, S., Switala, P., Juszczyk, Z., Sinkiewicz, W., Gilewski, W., Pietrzak, J., Orzel, T., Kasztelowicz, P., Kardaszewicz, P., Lazorko-Piega, M., Gabryel, J., Mosakowska, K., Bellwon, J., Rynkiewicz, A., Raczak, G., Lewicka, E., Dabrowska-Kugacka, A., Bartkowiak, R., Sosnowska-Pasiarska, B., Wozakowska-Kaplon, B., Krzeminski, A., Zabojszcz, M., Mirek-Bryniarska, E., Grzegorzko, A., Bury, K., Nessler, J., Zalewski, J., Furman, A., Broncel, M., Poliwczak, A., Bala, A., Zycinski, P., Rudzinska, M., Jankowski, L., Kasprzak, J.D., Michalak, L., Wojtczak Soska, K., Huziuk, I., Retwinski, A., Flis, P., Weglarz, J., Bodys, A., Grajek, S., Kaluzna-Oleksy, M., Straburzynska-Migaj, E., Dankowski, R., Szymanowska, K., Grabia, J., Szyszka, A., Nowicka, A., Samcik, M., Wolniewicz, L., Baczynska, K., Komorowska, K., Poprawa, I., Komorowska, E., Sajnaga, D., Zolbach, A., Dudzik-Plocica, A., Abdulkarim, A.-F., Lauko-Rachocka, A., Kaminski, L., Kostka, A., Cichy, A., Ruszkowski, P., Splawski, M., Fitas, G., Szymczyk, A., Serwicka, A., Fiega, A., Zysko, D., Krysiak, W., Szabowski, S., Skorek, E., Pruszczyk, P., Bienias, P., Ciurzynski, M., Welnicki, M., Mamcarz, A., Folga, A., Zielinski, T., Rywik, T., Leszek, P., Sobieszczanska-Malek, M., Piotrowska, M., Kozar-Kaminska, K., Komuda, K., Wisniewska, J., Tarnowska, A., Balsam, P., Marchel, M., Opolski, G., Kaplon-Cieslicka, A., Gil, R.J., Mozenska, O., Byczkowska, K., Gil, K., Pawlak, A., Michalek, A., Krzesinski, P., Piotrowicz, K., Uzieblo-Zyczkowska, B., Stanczyk, A., Skrobowski, A., Ponikowski, P., Jankowska, E., Rozentryt, P., Polonski, L., Gadula-Gacek, E., Nowalany-Kozielska, E., Kuczaj, A., Kalarus, Z., Szulik, M., Przybylska, K., Klys, J., Prokop-Lewicka, G., Kleinrok, A., Tavares Aguiar, C., Ventosa, A., Pereira, S., Faria, R., Chin, J., De Jesus, I., Santos, R., Silva, P., Moreno, N., Queirós, C., Lourenço, C., Pereira, A., Castro, A., Andrade, A., Oliveira Guimaraes, T., Martins, S., Placido, R., Lima, G., Brito, D., Francisco, A.R., Cardiga, R., Proenca, M., Araujo, I., Marques, F., Moura, B., Leite, S., Campelo, M., Silva-Cardoso, J., Rodrigues, J., Rangel, I., Martins, E., Sofia Correia, A., Peres, M., Marta, L., Ferreira da Silva, G., Severino, D., Durao, D., Leao, S., Magalhaes, P., Moreira, I., Filipa Cordeiro, A., Ferreira, C., Araujo, C., Ferreira, A., Baptista, A., Radoi, M., Bicescu, G., Vinereanu, D., Sinescu, C.-J., Macarie, C., Popescu, R., Daha, I., Dan, G.-A., Stanescu, C., Dan, A., Craiu, E., Nechita, E., Aursulesei, V., Christodorescu, R., Otasevic, P., Simeunovic, D., Ristic, A.D., Celic, V., Pavlovic-Kleut, M., Suzic Lazic, J., Stojcevski, B., Pencic, B., Stevanovic, A., Andric, A., Iric-Cupic, V., Jovic, M., Davidovic, G., Milanov, S., Mitic, V., Atanaskovic, V., Antic, S., Pavlovic, M., Stanojevic, D., Stoickov, V., Ilic, S., Deljanin Ilic, M., Petrovic, D., Stojsic, S., Kecojevic, S., Dodic, S., Cemerlic Adic, N., Cankovic, M., Stojiljkovic, J., Mihajlovic, B., Radin, A., Radovanovic, S., Krotin, M., Klabnik, A., Pernicky, M., Murin, J., Kovar, F., Kmec, J., Semjanova, H., Strasek, M., Savnik Iskra, M., Ravnikar, T., Cernic Suligoj, N., Komel, J., Fras, Z., Jug, B., Glavic, T., Losic, R., Bombek, M., Krajnc, I., Krunic, B., Horvat, S., Kovac, D., Rajtman, D., Cencic, V., Letonja, M., Winkler, R., Valentincic, M., Melihen-Bartolic, C., Bartolic, A., Pusnik Vrckovnik, M., Kladnik, M., Slemenik Pusnik, C., Marolt, A., Klen, J., Drnovsek, B., Leskovar, B., Fernandez Anguita, M.J., Gallego Page, J.C., Salmeron Martinez, F.M., Andres, J., Genis, A.B., Mirabet, S., Mendez, A., Garcia-Cosio, L., Roig, E., Leon, V., Gonzalez-Costello, J., Muntane, G., Garay, A., Alcade-Martinez, V., Lopez Fernandez, S., Rivera-Lopez, R., Puga-Martinez, M., Fernandez-Alvarez, M., Serrano-Martinez, J.L., Grille-Cancela, Z., Marzoa-Rivas, R., Blanco-Canosa, P., Paniagua-Martin, M.J., Barge-Caballero, E., Laynez Cerdena, I., Famara Hernandez Baldomero, I., Lara Padron, A., Ofelia Rosillo, S., Dalmau Gonzalez-Gallarza, R., Salvador Montanes, O., Iniesta Manjavacas, A.M., Castro Conde, A., Araujo, A., Soria, T., Garcia-Pavia, P., Gomez-Bueno, M., Cobo-Marcos, M., Alonso-Pulpon, L., Segovia Cubero, J., Sayago, I., Gonzalez-Segovia, A., Briceno, A., Escribano Subias, P., Vicente Hernandez, M., Ruiz Cano, M.J., Gomez Sanchez, M.A., Barrios Garrido-Lestache, E., Garcia Pinilla, J.M., Garcia de la Villa, B., Sahuquillo, A., Bravo Marques, R., Torres Calvo, F., Perez-Martinez, M.T., Gracia-Rodenas, M.R., Garrido-Bravo, I.P., Pastor-Perez, F., Pascual-Figal, D.A., Diaz Molina, B., Orus, J., Epelde Gonzalo, F., Bertomeu, V., Valero, R., Martinez-Abellan, R., Quiles, J., Rodrigez-Ortega, J.A., Mateo, I., ElAmrani, A., Fernandez-Vivancos, C., Bierge Valero, D., Almenar-Bonet, L., Sanchez-Lazaro, I.J., Marques-Sule, E., Facila-Rubio, L., Perez-Silvestre, J., Garcia-Gonzalez, P., Ridocci-Soriano, F., Garcia-Escriva, D., Pellicer-Cabo, A., de la Fuente Galan, L., Lopez Diaz, J., Recio Platero, A., Arias, J.C., Blasco-Peiro, T., Sanz Julve, M., Sanchez-Insa, E., Aured-Guallar, C., Portoles-Ocampo, A., Melin, M., Hägglund, E., Stenberg, A., Lindahl, I.-M., Asserlund, B., Olsson, L., Afzelius, M., Karlström, P., Tengvall, L., Wiklund, P.-A., Olsson, B., Kalayci, S., Cavusoglu, Y., Gencer, E., Yilmaz, M.B., Gunes, H., Canepa, Marco, Fonseca, Candida, Chioncel, Ovidiu, Laroche, Cécile, Crespo-Leiro, Maria G., Coats, Andrew J.S., Mebazaa, Alexandre, Piepoli, Massimo F., Tavazzi, Luigi, and Maggioni, Aldo P.

Published
2018
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10. Manejo de la hipertensión pulmonar tromboembólica crónica. Experiencia de un centro de referencia nacional

Author

María Jesús López Gude, Alejandro Santos-Lozano, Enrique Pérez de la Sota, Jorge Centeno Rodríguez, Jose Luís Pérez Vela, María Teresa Velázquez, Juan Delgado Jiménez, Rafael Morales, José María Aguado García, Pilar Escribano Subías, and José María Cortina Romero

Subjects
Medicine, Surgery, RD1-811
Abstract

Resumen: Introducción: La tromboendarterectomía pulmonar es el tratamiento de elección y potencialmente curativo de la hipertensión pulmonar tromboembólica crónica; el tratamiento médico quedaría limitado a los pacientes considerados inoperables. Este estudio evalúa los resultados a largo plazo con ambos tratamientos en una Unidad de Referencia Nacional. Métodos: Desde febrero de 1996 hasta diciembre del 2015 se ha valorado a 292 pacientes, intervenido 142 (grupo 1) y considerado inoperables 116 (grupo 2). El seguimiento finalizó en diciembre del 2016. Resultados: La supervivencia a los 3 años fue del 92% en el grupo 1 y del 86% en el grupo 2, a los 5 años el 91 y el 78%, respectivamente (p = 0,001). En el análisis multivariante la supervivencia en ambos grupos estuvo relacionada con la tromboendarterectomía (HR 0,37; IC del 95% 0,19-0,72; p = 0,003), el antecedente de embolia aguda de pulmón (HR 0,50; IC del 95% 0,27-0,92; p = 0,026), el test de la marcha de 6 min (HR 0,79; IC del 95% 0,73-0,85; p = 0,001), la historia de cáncer (HR 2,57; IC del 95% 1,22-5,43; p = 0,013) y las resistencias vasculares pulmonares (HR 1,19; IC del 95% 1,02-1,39; p = 0,024). En el grupo 1 la supervivencia se relacionó con el test de 6 min (HR 0,84; IC del 95% 0,79-0,96; p = 0,009) y el gasto cardiaco (HR 0,61; IC del 95% 0,39-0,96; p = 0,033); en el grupo 2 únicamente con el test de 6 min (HR 0,81; IC del 95% 0,71-0,92; p = 0,001). Conclusiones: Los pacientes con hipertensión pulmonar tromboembólica crónica intervenidos con tromboendarterectomía tienen una supervivencia excelente y significativamente superior a la de los pacientes tratados médicamente. En la presente serie la tromboendarterectomía es el factor predictor de supervivencia más importante. Abstract: Introduction: Although medical treatment can clinically improve inoperable patients, pulmonary endarterectomy surgery is the treatment of choice for patients with chronic thromboembolic pulmonary hypertension, and is potentially curative. An evaluation was made of the long-term outcomes in medical and surgical populations at a national Unit of Reference. Methods: A total of 292 patients were evaluated from February 1996 until December 2015, of whom 142 were had surgery (group 1) and 116 were considered inoperable (group 2). The follow-up ended in December 2016. Results: Survival rate after 3 3 years was 92% in group 1 and 86% in group 2, and after 5 5 years it was 91% and 78%, respectively (P=.001). The multivariate analysis results showed that survival in patients with chronic thromboembolic pulmonary hypertension was related to surgery (HR 0.37; 95% CI; 0.19-0.72; P=.003), history of acute pulmonary embolism (HR 0.50; 95% CI; 0.27-0.92; P=.026), distance walked in the six-minute walk test (HR 0.79; 95% CI; 0.73-0.85; P=.001), history of cancer (HR 2.57; 95% CI; 1.22-5.43; P=.013), and pulmonary vascular resistance (HR 1.19; 95% CI; 1.02-1.39; P=.024). In group 1, the variables associated with survival were the six-minute walk test (HR 0.84; 95% CI; 0.79-0.96; P=.009), and the cardiac output (HR 0.61; 95% CI; 0.39-0.96; P=.033), and in group 2, the variable associated was the six-minute walk test (HR 0.81; 95% CI; 0.71-0.92; P=.001). Conclusion: Patients with chronic thromboembolic pulmonary hypertension who received pulmonary endarterectomy surgery have an excellent survival rate, and significantly superior to that of patients treated medically. In the present series, pulmonary endarterectomy surgery was the most important predictive survival factor. Palabras clave: Hipertensión pulmonar, Embolia pulmonar, Tromboendarterectomía pulmonar, Keywords: Pulmonary hypertension, Pulmonary embolism, Pulmonary thromboendarterectomy

Published
2018
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12. Effects of an 8-month exercise intervention on physical capacity, NT-proBNP, physical activity levels and quality of life data in patients with pulmonary arterial hypertension by NYHA class

Author

Alejandro Santos-Lozano, Paz Sanz-Ayan, Laura González-Saiz, Carlos A. Quezada-Loaiza, Carmen Fiuza-Luces, Angela Flox-Camacho, Diego Munguía-Izquierdo, Alfredo Santalla, María Morán, Pilar Escribano-Subías, and Alejandro Lucia

Subjects
Pulmonary arterial hypertension, Physical activity levels, Quality of life, Physical intervention, Computer applications to medicine. Medical informatics, R858-859.7, Science (General), Q1-390
Abstract

This article provides descriptive detailed (pre and post) values of physical capacity variables, NT-proBNP, physical activity levels and quality of life in patients with pulmonary arterial hypertension (PH) (both, intervention and control group) by New York Heart Association (NYHA) class before and after an 8-month exercise intervention. The data are supplemental to our original Randomized Controlled Trial (RCT) entitled “Benefits of skeletal-muscle exercise training in pulmonary arterial hypertension: The WHOLEi+12 trial” (L. González-Saiz, C. Fiuza-Luces, F. Sanchis-Gomar, A. Santos-Lozano, C.A. Quezada-Loaiza, A. Flox-Camacho, D. Munguía-Izquierdo, I. Ara, A. Santalla, M. Morán, P. Sanz-Ayan, P. Escribano-Subías, A. Lucia A, 2017) [1].

Published
2017
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13. Radiological Findings in Multidetector Computed Tomography (MDCT) of Hereditary and Sporadic Pulmonary Veno-Occlusive Disease: Certainties and Uncertainties

Author

Marta Pérez Núñez, Sergio Alonso Charterina, Carmen Pérez-Olivares, Yolanda Revilla Ostolaza, Rafael Morales Ruiz, Ana Belén Enguita Valls, Jair Antonio Tenorio, Natalia Gallego Zazo, Alicia De Pablo Gafas, Pablo Lapunzina, Adriana Rodríguez Chaverri, and Pilar Escribano Subías

Subjects
pulmonary veno-occlusive disease, pulmonary hypertension, multidetector computed tomography (MDCT), Medicine (General), R5-920
Abstract

Pulmonary veno-occlusive disease (PVOD) is a very infrequent form of pulmonary arterial hypertension with an aggressive clinical course, poor response to specific vasodilator treatment, and low survival. Confirming a definitive diagnosis is essential to guide treatment and assess lung transplantation. However, in the absence of histological or genetic confirmation, the diagnosis is complex, requiring a clinical suspicion. Multidetector computed tomography (MDCT) is an essential part of the non-invasive diagnostic tools of PVOD. We retrospectively reviewed the MDCT findings from a consecutive series of 25 patients diagnosed with PVOD, 9 with the sporadic form and 16 with the hereditary form of the disease. The presence and extent of typical findings of the diagnostic triad were assessed in all patients (ground glass parenchymal involvement, septal lines, and lymphadenopathy). In our series, 92% of patients showed at least two of the radiological findings described as typical of the disease. All patients presented at least one typical radiological characteristic. The incidence of radiological findings considered typical is very high, however was not associated with greater hemodynamic severity nor to the development of acute lung edema. No significant differences were found between the two groups. A poorly expressive MDCT does not exclude the disease.

Published
2021
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14. Impacto del cateterismo derecho de ejercicio en el diagnóstico diferencial de la enfermedad tromboembólica pulmonar crónica

Author

Huertas Nieto, Sergio, Velázquez Martín, Maite, Sarnago Cebada, Fernando, Jiménez López-Guarch, Carmen, Maneiro Melón, Nicolás, Flox Camacho, Ángela, Segura de la Cal, Teresa, Cruz Utrilla, Alejandro, Aguilar Colindres, Ricardo, López Gude, María Jesús, Quezada Loaiza, Carlos Andrés, Revilla Ostolaza, Yolanda, Alonso Charterina, Sergio, Gómez Cuervo, Covadonga, Arribas Ynsaurriaga, Fernando, and Escribano Subías, Pilar

Abstract

La enfermedad tromboembólica crónica se define como la trombosis crónica de la vasculatura pulmonar y disnea, sin hipertensión pulmonar (HP) en reposo tras una embolia de pulmón. El estudio evaluó la hemodinámica al esfuerzo en estos pacientes y su relación con la ergoespirometría.

Published
2024
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15. First Genotype-Phenotype Study in TBX4 Syndrome Gain-of-Function Mutations Causative for Lung Disease

Author

Prapa, M., Lago-Docampo, M., Swietlik, E.M., Montani, D., Eyries, M., Humbert, M., Welch, C.C.L., Chung, W., Berger, R.M.F., Bogaard, H.J., Danhaive, O., Escribano-Subías, P., Gall, H., Girerd, B., Hernandez-Gonzalez, I., Holden, S., Hunt, D., Jansen, S.M.A., Kerstjens-Frederikse, W., Kiely, D., Lapunzina, P., McDermott, J., Moledina, S., Pepke-Zaba, J., Polwarth, G.J., Schotte, G., Tenorio-Castaño, J., Thompson, A.A.R., Wharton, J., Wort, S.J., Megy, K., Mapeta, R., Treacy, C.M., Martin, J.M., Li, W., Swift, A.J., Upton, P.D., Morrell, N.W., Gräf, S., Valverde, D., Cardiovascular Centre (CVC), Pulmonary medicine, and ACS - Pulmonary hypertension & thrombosis

Subjects
interstitial lung disease, Pulmonary and Respiratory Medicine, gain-of-function, pulmonary arterial hypertension, TBX4, Critical Care and Intensive Care Medicine, lung developmental disease
Abstract

Rationale: Despite the increased recognition of TBX4 (T-BOX transcription factor 4)-associated pulmonary arterial hypertension (PAH), genotype-phenotype associations are lacking and may provide important insights.Objectives: To compile and functionally characterize all TBX4 variants reported to date and undertake a comprehensive genotype-phenotype analysis.Methods: We assembled a multicenter cohort of 137 patients harboring monoallelic TBX4 variants and assessed the pathogenicity of missense variation (n = 42) using a novel luciferase reporter assay containing T-BOX binding motifs. We sought genotype-phenotype correlations and undertook a comparative analysis with patients with PAH with BMPR2 (Bone Morphogenetic Protein Receptor type 2) causal variants (n = 162) or no identified variants in PAH-associated genes (n = 741) genotyped via the National Institute for Health Research BioResource-Rare Diseases.Measurements and Main Results: Functional assessment of TBX4 missense variants led to the novel finding of gain-of-functio effects associated with older age at diagnosis of lung disease compared with loss-of-function effects (P = 0.038). Variants located in the T-BOX and nuclear localization domains were associated with earlier presentation (P = 0.005) and increased incidence of interstitial lung disease (P = 0.003). Event-free survival (death or transplantation) was shorter in the T-BOX group (P = 0.022), although age had a significant effect in the hazard model (P = 0.0461). Carriers of TBX4 variants were diagnosed at a younger age (P, 0.001) and had worse baseline lung function (FEV1, FVC) (P = 0.009) than the BMPR2 and no identified causal variant groups.Conclusions: We demonstrated that TBX4 syndrome is not strictly the result of haploinsufficiency but can also be caused by gain of function. The pleiotropic effects of TBX4 in lung disease may be in part explained by the differential effect of pathogenic mutations located in critical protein domains.

Published
2022

16. Effect of Coronavirus Disease 2019 in Pulmonary Circulation. The Particular Scenario of Precapillary Pulmonary Hypertension

Author

Jorge Nuche, Teresa Segura de la Cal, Carmen Jiménez López Guarch, Francisco López-Medrano, Carmen Pérez-Olivares Delgado, Fernando Arribas Ynsaurriaga, Juan F. Delgado, Borja Ibáñez, Eduardo Oliver, and Pilar Escribano Subías

Subjects
pulmonary hypertension, COVID-19, pulmonary circulation, endothelial dysfunction, Medicine (General), R5-920
Abstract

The Coronavirus Disease of 2019 (COVID-19) has supposed a global health emergency affecting millions of people, with particular severity in the elderly and patients with previous comorbidities, especially those with cardiovascular disease. Patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) could represent an especially vulnerable population because of the high mortality rates reported for respiratory infections. However, the number of COVID-19 cases reported among PAH and CTEPH patients is surprisingly low. Furthermore, the clinical picture that has been described in these patients is far from the severity that experts would expect. Endothelial dysfunction is a common feature between patients with PAH/CTEPH and COVID-19, leading to ventilation/perfusion mismatch, vasoconstriction, thrombosis and inflammation. In this picture, the angiotensin-converting enzyme 2 plays an essential role, being directly involved in the pathophysiology of both clinical entities. Some of these common characteristics could explain the good adaptation of PAH and CTEPH patients to COVID-19, who could also have obtained a benefit from the disease’s specific treatments (anticoagulant and pulmonary vasodilators), probably due to its protective effect on the endothelium. Additionally, these common features could also lead to PAH/CTEPH as a potential sequelae of COVID-19. Throughout this comprehensive review, we describe the similarities and differences between both conditions and the possible pathophysiological and therapeutic-based mechanisms leading to the low incidence and severity of COVID-19 reported in PAH/CTEPH patients to date. Nevertheless, international registries should look carefully into this population for better understanding and management.

Published
2020
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18. From Health-Related Quality of Life (HRQoL) of Patients with Pulmonary Hypertension to Patient Experience with the Care Received: Should We Be More Aware of Current Patient Needs?

Author

Martínez Meñaca A, Blanco Vich I, López Meseguer M, López Reyes R, Bedate Díaz P, Elías Hernández T, Fernández-Cuesta Peñafiel I, Fernández G, Álvez Liste A, and Escribano Subías P

Subjects
CAMPHOR, Chronic thromboembolic pulmonary hypertension, EQ-5D-5L, Empowerment, Health-related quality of life, IEXPAC, Information and communications technology (ICT), Patient engagement, Pulmonary arterial hypertension, Pulmonary hypertension, humanities
Abstract

Self-perceived health-related quality of life (HRQoL) of patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) and their experience with the care received are important for improving their management. We conducted a study to assess both patient-reported outcomes (PROs) and how they interrelate.

Published
2021

19. Riociguat treatment in patients with chronic thromboembolic pulmonary hypertension: Final safety data from the EXPERT registry

Author

Ghofrani, H.A., Gomez Sanchez, M.-A. (Miguel-Angel), Humbert, M., Pittrow, D. (David), Simonneau, G. (Gérald), Gall, H. (Henning), Grünig, E. (Ekkehard), Klose, H. (Hans), Halank, M. (Michael), Langleben, D. (David), Snijder, R., Escribano Subías, P. (Pilar), Mielniczuk, L.M. (Lisa M.), Lange, T.J. (Tobias J.), Vachiéry, J.-L. (Jean-Luc), Wirtz, H. (Hubert), Helmersen, D.S. (Douglas S.), Tsangaris, I. (Iraklis), Barberá, J.A. (Joan A.), Pepke-Zaba, J. (Joanna), Boonstra, A. (Anco), Rosenkranz, S. (Stephan), Ulrich, S. (Silvia), Steringer-Mascherbauer, R. (Regina), Delcroix, M. (Marion), Jansa, P. (Pavel), Šimková, I. (Iveta), Giannakoulas, G. (George), Klotsche, J. (Jens), Williams, E. (Evgenia), Meier, C. (Christian), Hoeper, M.M. (Marius M.), Caneva, J. (Jorge), Tuhay, G. (Graciela), Diez, M. (Mirta), Talavera, M.L. (Maria Lujan), Acosta, A. (Adriana), Vulcano, N. (Norberto), Bosio, M. (Martin), Maldonado, L. (Lorena), Deleo, S. (Sabino), Melatini, L. (Luciano), Keogh, A. (Anne), Kotlyar, E. (Eugene), Feenstra, J. (John), Dwyer, N. (Nathan), Adams, H. (Heath), Stevens, W. (Wendy), Steele, P. (Peter), Proudman, S. (Susanna), Minson, R. (Robert), Reeves, G. (Glenn), Lavender, M. (Melanie), Ng, B. (Benjamin), Mackenzie, M. (Michele), Barry, L. (Lisa), Gruenberger, M. (Margarethe), Huber, C. (Charlotte), Lang, I. (Irene), Tilea, I. (Ioana), Sadushi-Kolici, R. (Roela), Löffler-Ragg, J. (Judith), Feistmantl, L.-T. (Lisa-Theresa), Evrard, P. (Patrick), Louis, R. (Renaud), Guiot, J. (Julien), Naldi, M. (Marco), De Pauw, M. (Michel), Mehta, S. (Sanjay), Camacho, R.C. (Rafael Conde), Tovar, P.P. (Patricia Parada), Londoño, A. (Alejandro), Campo, F. (Felipe), Garcia, P. (Paula), Lema, C. (Camila), Orozco-Levi, M. (Mauricio), Martinez, W. (William), Gomez, J.E. (Juan Esteban), Nielsen-Kudsk, J.E. (Jens Erik), Mellemkjaer, S. (Soren), Anton, L. (Ly), Altraja, A. (Alan), Vihinen, T. (Tapani), Vasankari, T. (Tuija), Sitbon, O. (Olivier), Cottin, V. (Vincent), Têtu, L. (Laurent), Noël-Savina, E. (Elise), Shearman, N. (Nicole), Tayler, S. (Susanne), Olzik, I. (Ilona), Kulka, C. (Christine), Grimminger, J. (Jan), Simon, M. (Marcel), Nolde, A. (Anna), Oqueka, T. (Tim), Harbaum, L. (Lars), Egenlauf, B. (Benjamin), Ewert, R. (Ralf), Schulz, C. (Christian), Regotta, S. (Sabine), Kramer, T. (Tilmann), Knoop-Busch, S. (Susanne), Gerhardt, F. (Felix), Konstantinides, S. (Stavros), Pitsiou, G. (Georgia), Stanopoulos, I. (Ioannis), Sourla, E. (Evdokia), Mouratoglou, S. (Sofia), Karvounis, H.I., Pappas, A. (Athanasios), Georgopoulos, D. (Dimitrios), Fanaridis, M. (Michail), Mitrouska, I. (Ioanna), Michalis, L.K. (Lampros), Pappas, K. (Konstantinos), Kotsia, A. (Anna), Gaine, S. (Sean), Vizza, C.D. (Carmine Dario), Manzi, G. (Giovanna), Poscia, R. (Roberto), Badagliacca, R. (Roberto), Agostoni, P. (Piergiuseppe), Bruno, N. (Noemi), Farina, S. (Stefania), D'Alto, M. (Michele), Argiento, P. (Paola), Correra, A. (Anna), Di Marco, G.M. (Giovanni Maria), Cresci, C. (Chiara), Vannucchi, V. (Vieri), Torricelli, E. (Elena), Garcea, A. (Alessio), Pesci, A. (Alberto), Sardella, L. (Luca), Paciocco, G. (Giuseppe), Pane, F. (Federico), D'Armini, A.M. (Andrea Maria), Pin, M. (Maurizio), Grazioli, V. (Valentina), Massola, G. (Giulia), Sciortino, A. (Antonio), Prediletto, R. (Renato), Bauleo, C. (Carolina), Airò, E. (Edoardo), Ndreu, R. (Rudina), Pavlickova, I. (Ivana), Lunardi, C. (Claudio), Mulè, M. (Massimiliano), Farruggio, S. (Silvia), Costa, S. (Serena), Galgano, G. (Giuseppe), Petruzzi, M. (Mario), De Luca, A. (Anna), Lombardi, F. (Francesco), Roncon, L. (Loris), Conte, L. (Luca), Picariello, C. (Claudio), Wirtz, G. (Gil), Alexandre, M. (Myriam), Vonk Noordegraaf, A. (Anton), Boogaard, H. (H.), Mager, J. (J.), Reesink, H.J. (Herre), Toorn, L.M. (Leon) van den, Boomars, K.A.T. (Karin), Andreassen, A.K. (Arne K.), Castro, G. (Graça), Tania, G. (Gonçalves), Baptista, R. (Rui), Marinho, A. (António), Shiang, T. (Teresa), Oliveira, A. (Ana), Coutinho, D. (Daniel), Sousa, J. (Joana), Loureiro, M.J. (Maria José), Repolho, D. (Débora), Martins Jesus, S.M. (Susana Maria), Capinha, M. (Marta), Agostinho, J. (João), Cardoso, T. (Tania), Rocha, A. (Andreia), Espinha, M. (Mafalda), Ivanov, K.I. (Kyundyul Ivanovich), Alexeeva, D.E. (Dalyana Eduardovna), Batalina, M.V. (Marina Vadimovna), Hegya, D.V. (Daria Viktorovna), Zvereva, T.N. (Tatyana Nikolaevna), Avdeev, S.N. (Sergey Nikolaevich), Tsareva, N.A. (Natalia Anatolievna), Galyavich, A.S. (Albert Sarvatovich), Nikolaevich, B.A. (Bykov Aleksander), Filippov, E.V. (Evgeny Vladimirovich), Yakovleva, O.E. (Olga Eduardovna), Pavlova, O.B. (Olga Borisovna), Skripkina, E.S. (Elena Sergeevna), Martynyuk, T.V. (Tamila Vitalievna), Bukatova, I.F. (Irina Fedorovna), Tregubova, A.V. (Anna Viktorovna), Platonov, D.Y. (Dmitry Yurievich), Kolomeytseva, T.M. (Tatyana Mikhaylovna), Al Dalaan, A. (Abdullah), Abdelsayed, A.A. (Abeer Abeer), Weheba, I. (Ihab), Saleemi, S. (Sarferaz), Sakkijha, H. (Hussam), Bohacekova, M. (Marcela), Valkovicova, T. (Tatiana), Farkasova, I. (Iveta), Quezada, C.A. (Carlos Andres), Piccari, L. (Lucilla), Blanco, I. (Isabel), Sebastian, L. (Laura), Roman, A. (Antonio), Lopez, M. (Manuel), Otero, R. (Remedios), Elias, T. (Teresa), Jara, L. (Luis), Asencio, I. (Isabel), Arjona, J.J. (Josefa Jiménez), Almagro, R.M. (Raúl Menor), Cárdenas, S.L. (Salvador López), García, S.A. (Salvador Alcaraz), Rodríguez, P.V. (Patricia Villanueva), Lopez, R. (Raquel), Garcia, A. (Alberto), Avilés, F.F. (Francisco Fernandez), De La Pava, S. (Sebastian), Yotti, R. (Raquel), Peñate, G.P. (Gregorio Pérez), Marrero, F.L. (Fernando León), Cifrián Martínez, J.M. (José Manuel), Martinez-Meñaca, A. (Amaya), Alonso, L.P. (Lecue Pilar), Rozas, S.F. (Sonia Fernandez), Fernandez, D.I. (David Iturbe), Cuesta, V.M. (Victor Mora), Söderberg, S. (Stefan), Bartfay, S.-E. (Sven-Erik), Rundqvist, B. (Bengt), Alfetlawi, M. (Monthir), Wodlin, P. (Peter), Schwarz, E.I. (Esther Irene), Speich, R. (Rudolf), Lador, F. (Frédéric), Rochat, T. (Thierry), Gasche-Soccal, P. (Paola), Hsu, C.-H. (Chih-Hsin), Lin, T.-H. (Tsung-Hsien), Su, H.-M. (Ho-Ming), Lai, W.-T. (Wen-Ter), Chu, C.Y. (Chun Yuan), Hsu, P.-C. (Po-Chao), Voon, W.-C. (Wen-Chol), Yen, H.-W. (Hsueh-Wei), Yih-Jer Wu, J. (Jacob), Wu, S.-H. (Shu-Hao), Huang, W.-P. (Wen-Pin), Fong, M.-C. (Man-Cai), Huang, C.-L. (Chien-Lung), Kuo, P.-H. (Ping-Hung), Lin, Y.-H. (Yen-Hung), Lin, J.-L. (Jiunn-Lee), Hung, C.-S. (Chi-Sheng), Wu, C.-K. (Cho-Kai), Sung, S.-H. (Shih-Hsien), Huang, W.-C. (Wei-Chun), Cheng, C.-C. (Chin-Chang), Kuo, S.-H. (Shu-Hung), Wang, W.-H. (Wen-Hwa), Ho, W.-J. (Wan-Jing), Hsu, T.-S. (Tsu-Shiu), Mutlu, B. (Bülent), Atas, H. (Halil), Ongen, G. (Gul), Un, Z. (Zeynep), Okumus, G. (Gulfer), Hanta, I. (Ismail), Corris, P. (Paul), Peacock, A. (Andrew), Church, C. (Colin), Toshner, M. (Mark), Newnham, M. (Michael), Ghofrani, H.A., Gomez Sanchez, M.-A. (Miguel-Angel), Humbert, M., Pittrow, D. (David), Simonneau, G. (Gérald), Gall, H. (Henning), Grünig, E. (Ekkehard), Klose, H. (Hans), Halank, M. (Michael), Langleben, D. (David), Snijder, R., Escribano Subías, P. (Pilar), Mielniczuk, L.M. (Lisa M.), Lange, T.J. (Tobias J.), Vachiéry, J.-L. (Jean-Luc), Wirtz, H. (Hubert), Helmersen, D.S. (Douglas S.), Tsangaris, I. (Iraklis), Barberá, J.A. (Joan A.), Pepke-Zaba, J. (Joanna), Boonstra, A. (Anco), Rosenkranz, S. (Stephan), Ulrich, S. (Silvia), Steringer-Mascherbauer, R. (Regina), Delcroix, M. (Marion), Jansa, P. (Pavel), Šimková, I. (Iveta), Giannakoulas, G. (George), Klotsche, J. (Jens), Williams, E. (Evgenia), Meier, C. (Christian), Hoeper, M.M. (Marius M.), Caneva, J. (Jorge), Tuhay, G. (Graciela), Diez, M. (Mirta), Talavera, M.L. (Maria Lujan), Acosta, A. (Adriana), Vulcano, N. (Norberto), Bosio, M. (Martin), Maldonado, L. (Lorena), Deleo, S. (Sabino), Melatini, L. (Luciano), Keogh, A. (Anne), Kotlyar, E. (Eugene), Feenstra, J. (John), Dwyer, N. (Nathan), Adams, H. (Heath), Stevens, W. (Wendy), Steele, P. (Peter), Proudman, S. (Susanna), Minson, R. (Robert), Reeves, G. (Glenn), Lavender, M. (Melanie), Ng, B. (Benjamin), Mackenzie, M. (Michele), Barry, L. (Lisa), Gruenberger, M. (Margarethe), Huber, C. (Charlotte), Lang, I. (Irene), Tilea, I. (Ioana), Sadushi-Kolici, R. (Roela), Löffler-Ragg, J. (Judith), Feistmantl, L.-T. (Lisa-Theresa), Evrard, P. (Patrick), Louis, R. (Renaud), Guiot, J. (Julien), Naldi, M. (Marco), De Pauw, M. (Michel), Mehta, S. (Sanjay), Camacho, R.C. (Rafael Conde), Tovar, P.P. (Patricia Parada), Londoño, A. (Alejandro), Campo, F. (Felipe), Garcia, P. (Paula), Lema, C. (Camila), Orozco-Levi, M. (Mauricio), Martinez, W. (William), Gomez, J.E. (Juan Esteban), Nielsen-Kudsk, J.E. (Jens Erik), Mellemkjaer, S. (Soren), Anton, L. (Ly), Altraja, A. (Alan), Vihinen, T. (Tapani), Vasankari, T. (Tuija), Sitbon, O. (Olivier), Cottin, V. (Vincent), Têtu, L. (Laurent), Noël-Savina, E. (Elise), Shearman, N. (Nicole), Tayler, S. (Susanne), Olzik, I. (Ilona), Kulka, C. (Christine), Grimminger, J. (Jan), Simon, M. (Marcel), Nolde, A. (Anna), Oqueka, T. (Tim), Harbaum, L. (Lars), Egenlauf, B. (Benjamin), Ewert, R. (Ralf), Schulz, C. (Christian), Regotta, S. (Sabine), Kramer, T. (Tilmann), Knoop-Busch, S. (Susanne), Gerhardt, F. (Felix), Konstantinides, S. (Stavros), Pitsiou, G. (Georgia), Stanopoulos, I. (Ioannis), Sourla, E. (Evdokia), Mouratoglou, S. (Sofia), Karvounis, H.I., Pappas, A. (Athanasios), Georgopoulos, D. (Dimitrios), Fanaridis, M. (Michail), Mitrouska, I. (Ioanna), Michalis, L.K. (Lampros), Pappas, K. (Konstantinos), Kotsia, A. (Anna), Gaine, S. (Sean), Vizza, C.D. (Carmine Dario), Manzi, G. (Giovanna), Poscia, R. (Roberto), Badagliacca, R. (Roberto), Agostoni, P. (Piergiuseppe), Bruno, N. (Noemi), Farina, S. (Stefania), D'Alto, M. (Michele), Argiento, P. (Paola), Correra, A. (Anna), Di Marco, G.M. (Giovanni Maria), Cresci, C. (Chiara), Vannucchi, V. (Vieri), Torricelli, E. (Elena), Garcea, A. (Alessio), Pesci, A. (Alberto), Sardella, L. (Luca), Paciocco, G. (Giuseppe), Pane, F. (Federico), D'Armini, A.M. (Andrea Maria), Pin, M. (Maurizio), Grazioli, V. (Valentina), Massola, G. (Giulia), Sciortino, A. (Antonio), Prediletto, R. (Renato), Bauleo, C. (Carolina), Airò, E. (Edoardo), Ndreu, R. (Rudina), Pavlickova, I. (Ivana), Lunardi, C. (Claudio), Mulè, M. (Massimiliano), Farruggio, S. (Silvia), Costa, S. (Serena), Galgano, G. (Giuseppe), Petruzzi, M. (Mario), De Luca, A. (Anna), Lombardi, F. (Francesco), Roncon, L. (Loris), Conte, L. (Luca), Picariello, C. (Claudio), Wirtz, G. (Gil), Alexandre, M. (Myriam), Vonk Noordegraaf, A. (Anton), Boogaard, H. (H.), Mager, J. (J.), Reesink, H.J. (Herre), Toorn, L.M. (Leon) van den, Boomars, K.A.T. (Karin), Andreassen, A.K. (Arne K.), Castro, G. (Graça), Tania, G. (Gonçalves), Baptista, R. (Rui), Marinho, A. (António), Shiang, T. (Teresa), Oliveira, A. (Ana), Coutinho, D. (Daniel), Sousa, J. (Joana), Loureiro, M.J. (Maria José), Repolho, D. (Débora), Martins Jesus, S.M. (Susana Maria), Capinha, M. (Marta), Agostinho, J. (João), Cardoso, T. (Tania), Rocha, A. (Andreia), Espinha, M. (Mafalda), Ivanov, K.I. (Kyundyul Ivanovich), Alexeeva, D.E. (Dalyana Eduardovna), Batalina, M.V. (Marina Vadimovna), Hegya, D.V. (Daria Viktorovna), Zvereva, T.N. (Tatyana Nikolaevna), Avdeev, S.N. (Sergey Nikolaevich), Tsareva, N.A. (Natalia Anatolievna), Galyavich, A.S. (Albert Sarvatovich), Nikolaevich, B.A. (Bykov Aleksander), Filippov, E.V. (Evgeny Vladimirovich), Yakovleva, O.E. (Olga Eduardovna), Pavlova, O.B. (Olga Borisovna), Skripkina, E.S. (Elena Sergeevna), Martynyuk, T.V. (Tamila Vitalievna), Bukatova, I.F. (Irina Fedorovna), Tregubova, A.V. (Anna Viktorovna), Platonov, D.Y. (Dmitry Yurievich), Kolomeytseva, T.M. (Tatyana Mikhaylovna), Al Dalaan, A. (Abdullah), Abdelsayed, A.A. (Abeer Abeer), Weheba, I. (Ihab), Saleemi, S. (Sarferaz), Sakkijha, H. (Hussam), Bohacekova, M. (Marcela), Valkovicova, T. (Tatiana), Farkasova, I. (Iveta), Quezada, C.A. (Carlos Andres), Piccari, L. (Lucilla), Blanco, I. (Isabel), Sebastian, L. (Laura), Roman, A. (Antonio), Lopez, M. (Manuel), Otero, R. (Remedios), Elias, T. (Teresa), Jara, L. (Luis), Asencio, I. (Isabel), Arjona, J.J. (Josefa Jiménez), Almagro, R.M. (Raúl Menor), Cárdenas, S.L. (Salvador López), García, S.A. (Salvador Alcaraz), Rodríguez, P.V. (Patricia Villanueva), Lopez, R. (Raquel), Garcia, A. (Alberto), Avilés, F.F. (Francisco Fernandez), De La Pava, S. (Sebastian), Yotti, R. (Raquel), Peñate, G.P. (Gregorio Pérez), Marrero, F.L. (Fernando León), Cifrián Martínez, J.M. (José Manuel), Martinez-Meñaca, A. (Amaya), Alonso, L.P. (Lecue Pilar), Rozas, S.F. (Sonia Fernandez), Fernandez, D.I. (David Iturbe), Cuesta, V.M. (Victor Mora), Söderberg, S. (Stefan), Bartfay, S.-E. (Sven-Erik), Rundqvist, B. (Bengt), Alfetlawi, M. (Monthir), Wodlin, P. (Peter), Schwarz, E.I. (Esther Irene), Speich, R. (Rudolf), Lador, F. (Frédéric), Rochat, T. (Thierry), Gasche-Soccal, P. (Paola), Hsu, C.-H. (Chih-Hsin), Lin, T.-H. (Tsung-Hsien), Su, H.-M. (Ho-Ming), Lai, W.-T. (Wen-Ter), Chu, C.Y. (Chun Yuan), Hsu, P.-C. (Po-Chao), Voon, W.-C. (Wen-Chol), Yen, H.-W. (Hsueh-Wei), Yih-Jer Wu, J. (Jacob), Wu, S.-H. (Shu-Hao), Huang, W.-P. (Wen-Pin), Fong, M.-C. (Man-Cai), Huang, C.-L. (Chien-Lung), Kuo, P.-H. (Ping-Hung), Lin, Y.-H. (Yen-Hung), Lin, J.-L. (Jiunn-Lee), Hung, C.-S. (Chi-Sheng), Wu, C.-K. (Cho-Kai), Sung, S.-H. (Shih-Hsien), Huang, W.-C. (Wei-Chun), Cheng, C.-C. (Chin-Chang), Kuo, S.-H. (Shu-Hung), Wang, W.-H. (Wen-Hwa), Ho, W.-J. (Wan-Jing), Hsu, T.-S. (Tsu-Shiu), Mutlu, B. (Bülent), Atas, H. (Halil), Ongen, G. (Gul), Un, Z. (Zeynep), Okumus, G. (Gulfer), Hanta, I. (Ismail), Corris, P. (Paul), Peacock, A. (Andrew), Church, C. (Colin), Toshner, M. (Mark), and Newnham, M. (Michael)

Abstract

Objective: The soluble guanylate cyclase stimulator riociguat is approved for the treatment of adult patients with pulmonary arterial hypertension (PAH) and inoperable or persistent/recurrent chronic thromboembolic pulmonary hypertension (CTEPH) following Phase 3

Published
2021
Full Text
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20. Portopulmonary hypertension: prognosis and management in the current treatment era. Results from the REHAP Registry

Author

Lázaro Salvador M, Quezada Loaiza CA, Rodríguez Padial L, Barberá JA, López-Meseguer M, López-Reyes R, Sala Llinas E, Alcolea S, Blanco I, and Escribano Subías P

Subjects
PAH-targeted therapy, Portal hypertension, Portopulmonary hypertension, Pulmonary arterial hypertension, survival
Abstract

Portopulmonary hypertension (PoPH) is a rare condition with poorer survival compared to idiopatic/familial pulmonary arterial hypertension (IPAH/FPAH).

Published
2020

22. Influence of socioeconomic factors on pregnancy outcome in women with structural heart disease

Author

Van Hagen, Iris M., Baart, Sara, Fong Soe Khioe, Rebekah, Sliwa-Hahnle, Karen, Taha, Nasser, Lelonek, Malgorzata, Tavazzi, Luigi, Maggioni, Aldo Pietro, Johnson, Mark R., Maniadakis, Nikolaos, Fordham, Richard, Hall, Roger, Roos-Hesselink, Jolien W., Ferrari, Roberto, Marelli, Ariane, Webb, Gary, Kaemmerer, Harald, Popelova, Jana, Sliwa, Karen, Parsonage, William Anthony, Stein, Joerg, Elkayam, Uri, Thilen, Ulf, Budts, Werner, Ruys, Titia, Vardas, Panos, Komajda, Michel, Pinto, Fausto, Alonso, Angeles, Wood, David, Ferreira, Thierry, Gracia, Gérard, Laroche, Cécile, Missiamenou, Viviane, Taylor, Charles, Konte, Marème, Andarala, Maryna, Fiorucci, Emanuela, Lefrancq, Elin Folkesson, Glémot, Myriam, McNeill, Patti Ann, Pommier, Caroline, Lafay, Myriam, Aquieri, A., Vega, H. Ruda, Blanco, M. Vázquez, Lust, K., Fagermo, N., Gabriel, H., Donhauser, E., Gasimov, Z., Jahangirov, T., Hasanova, I., De Backer, J., Demulier, L., de Hosson, M., Beckx, M., Moissens, M., Kovacevic-Preradovic, T., Kozic, M., Lovric, M., Freire, C. Vilas, Chilingirova, N., Kratunkov, P., Montesclaros, A. R., Beaubien, E., Gordon, E., Walter, L., Lindsay, C., Wahab, N., Vavera, Z., El Nagar, A., Ebaid, H. H., El Sayed Makled, W. A., Dardier, A., Shabaan, M., Elrakshy, Y., Eltamawey, K., Abd-El Aziz, M. Gamal, Saad, A., Aboleineen, W., Ashour, Z., Sorour, K., Mahdy, M. A.Meguid, Iserin, L., Ladouceur, M., Cohen, S., Iung, B., Maisuradze, D., Mebus, S., Gembruch, U., Hammerstingl, C., Merz, W. M., Wald, C., Baumgartner, H., Orwat, S., Schmidt, R., Motz, R., Olsson, A., Berger, F., Nagdyman, N., Frogoudaki, A., Anastasiou-Nana, M., Temesvari, A., Kohalmi, D., Balint, H., Merkely, B., Liptai, C., Bowen, M., Cullen, M., Thornton, P., Husarova, V., Blatt, A., Elbaz-Greener, G., Moravsky, G., Vered, Z., Fuhrmann, A. Vazan, Shotan, A., Goland, S., Festa, P., Ali, L. Ait, Sinagra, G., Puggia, I., Mottolese, B. D.Agata, Carmina, M. G., Romeo, C., Crepaz, R., Fesslova, V., Azzarelli, A., Baldi, D., Bovenzi, F., Donvito, V., Vasario, E., Todros, T., Niwa, K., Mussagaliyeva, A., Mekebekova, D., Sharipova, S., Zaliunas, R., Jonkaitiene, R., Petrauskaite, J., Gumbiene, L., Jovanova, S., Cassar, A., Caruana, M., Karamermer, Y., Cornette, J. M.J., van Dijk, A., Bellersen, L., Duijnhouwer, T., De Groot, C., Pieper, E. P.G., van Oppen, C., Polak, P., Wajon, E., Wagenaar, L., Estensen, M., Lesniak-Sobelga, A., Podolec, P., Wisniowska-Smialek, S., Trybuch, A., Hoffman, P., Cichocka-Radwan, A., Sobczak, S., Faflik, U., Tomaszuk-Kazberuk, A., Przepiesc, J., Gil, M., Plaskota, K., Trojnarska, O., Guerra, N., de Sousa, L., Petrescu, V., Ginghina, C., Jurcut, R., Coman, I. Mircea, Gaisin, I. Ravilevich, Shilina, L. Valeryevna, Sharashkina, N., Tkacheva, O., Ivanov, D., Irtyuga, O., Jovovic, L., Prokselj, K., Kozelj, M., Elliott, C., Galian-Gay, L., Pijuan-Domenech, A., Subirana-Domenech, M. T., Tornos, P., Murga, N., Oliver, J. M., Escribano-Subías, P., Ruiz-Cano, M. J., Delgado-Jiménez, J., Furenas, E., Dellborg, M., Thilén, U., Schwerzmann, M., Bouchardy, J., Rutz, T., Tobler, D., Sarac, L., Esen, O. Batukan, Enar, S. Catirli, Al Mulla, A., Bazargani, N., Al Hatou, E., Farook, F., Almahmeed, W., Salih, B., Clifford, P., Bowers, N., Veldtman, G., Kerr, J., Tellett, L., Hudsmith, L., Thompson, P., Thorne, S., Bowater, S., Nihoyannopoulos, P., Curry, R., Freeman, L., Schroeder, F., Wendler, R., Hammond, S., Talluto, C., Murphy, D., Perlroth, M. G., Chintala, K., Gupta, P., Pare, E., Khatri, N., Scott, N., De Faria-Yeh, D., Bhatt, A. B., Tsiaras, S., Gurvitz, M., Otto, C., Botti, J., Ting, J., Davidson, W. R., Van Hagen, Iris M., Baart, Sara, Fong Soe Khioe, Rebekah, Sliwa-Hahnle, Karen, Taha, Nasser, Lelonek, Malgorzata, Tavazzi, Luigi, Maggioni, Aldo Pietro, Johnson, Mark R., Maniadakis, Nikolaos, Fordham, Richard, Hall, Roger, Roos-Hesselink, Jolien W., Ferrari, Roberto, Marelli, Ariane, Webb, Gary, Kaemmerer, Harald, Popelova, Jana, Sliwa, Karen, Parsonage, William Anthony, Stein, Joerg, Elkayam, Uri, Thilen, Ulf, Budts, Werner, Ruys, Titia, Vardas, Panos, Komajda, Michel, Pinto, Fausto, Alonso, Angeles, Wood, David, Ferreira, Thierry, Gracia, Gérard, Laroche, Cécile, Missiamenou, Viviane, Taylor, Charles, Konte, Marème, Andarala, Maryna, Fiorucci, Emanuela, Lefrancq, Elin Folkesson, Glémot, Myriam, McNeill, Patti Ann, Pommier, Caroline, Lafay, Myriam, Aquieri, A., Vega, H. Ruda, Blanco, M. Vázquez, Lust, K., Fagermo, N., Gabriel, H., Donhauser, E., Gasimov, Z., Jahangirov, T., Hasanova, I., De Backer, J., Demulier, L., de Hosson, M., Beckx, M., Moissens, M., Kovacevic-Preradovic, T., Kozic, M., Lovric, M., Freire, C. Vilas, Chilingirova, N., Kratunkov, P., Montesclaros, A. R., Beaubien, E., Gordon, E., Walter, L., Lindsay, C., Wahab, N., Vavera, Z., El Nagar, A., Ebaid, H. H., El Sayed Makled, W. A., Dardier, A., Shabaan, M., Elrakshy, Y., Eltamawey, K., Abd-El Aziz, M. Gamal, Saad, A., Aboleineen, W., Ashour, Z., Sorour, K., Mahdy, M. A.Meguid, Iserin, L., Ladouceur, M., Cohen, S., Iung, B., Maisuradze, D., Mebus, S., Gembruch, U., Hammerstingl, C., Merz, W. M., Wald, C., Baumgartner, H., Orwat, S., Schmidt, R., Motz, R., Olsson, A., Berger, F., Nagdyman, N., Frogoudaki, A., Anastasiou-Nana, M., Temesvari, A., Kohalmi, D., Balint, H., Merkely, B., Liptai, C., Bowen, M., Cullen, M., Thornton, P., Husarova, V., Blatt, A., Elbaz-Greener, G., Moravsky, G., Vered, Z., Fuhrmann, A. Vazan, Shotan, A., Goland, S., Festa, P., Ali, L. Ait, Sinagra, G., Puggia, I., Mottolese, B. D.Agata, Carmina, M. G., Romeo, C., Crepaz, R., Fesslova, V., Azzarelli, A., Baldi, D., Bovenzi, F., Donvito, V., Vasario, E., Todros, T., Niwa, K., Mussagaliyeva, A., Mekebekova, D., Sharipova, S., Zaliunas, R., Jonkaitiene, R., Petrauskaite, J., Gumbiene, L., Jovanova, S., Cassar, A., Caruana, M., Karamermer, Y., Cornette, J. M.J., van Dijk, A., Bellersen, L., Duijnhouwer, T., De Groot, C., Pieper, E. P.G., van Oppen, C., Polak, P., Wajon, E., Wagenaar, L., Estensen, M., Lesniak-Sobelga, A., Podolec, P., Wisniowska-Smialek, S., Trybuch, A., Hoffman, P., Cichocka-Radwan, A., Sobczak, S., Faflik, U., Tomaszuk-Kazberuk, A., Przepiesc, J., Gil, M., Plaskota, K., Trojnarska, O., Guerra, N., de Sousa, L., Petrescu, V., Ginghina, C., Jurcut, R., Coman, I. Mircea, Gaisin, I. Ravilevich, Shilina, L. Valeryevna, Sharashkina, N., Tkacheva, O., Ivanov, D., Irtyuga, O., Jovovic, L., Prokselj, K., Kozelj, M., Elliott, C., Galian-Gay, L., Pijuan-Domenech, A., Subirana-Domenech, M. T., Tornos, P., Murga, N., Oliver, J. M., Escribano-Subías, P., Ruiz-Cano, M. J., Delgado-Jiménez, J., Furenas, E., Dellborg, M., Thilén, U., Schwerzmann, M., Bouchardy, J., Rutz, T., Tobler, D., Sarac, L., Esen, O. Batukan, Enar, S. Catirli, Al Mulla, A., Bazargani, N., Al Hatou, E., Farook, F., Almahmeed, W., Salih, B., Clifford, P., Bowers, N., Veldtman, G., Kerr, J., Tellett, L., Hudsmith, L., Thompson, P., Thorne, S., Bowater, S., Nihoyannopoulos, P., Curry, R., Freeman, L., Schroeder, F., Wendler, R., Hammond, S., Talluto, C., Murphy, D., Perlroth, M. G., Chintala, K., Gupta, P., Pare, E., Khatri, N., Scott, N., De Faria-Yeh, D., Bhatt, A. B., Tsiaras, S., Gurvitz, M., Otto, C., Botti, J., Ting, J., and Davidson, W. R.

Abstract

Objective Cardiac disease is the leading cause of indirect maternal mortality. The aim of this study was to analyse to what extent socioeconomic factors influence the outcome of pregnancy in women with heart disease. Methods The Registry of Pregnancy and Cardiac disease is a global prospective registry. For this analysis, countries that enrolled ≥10 patients were included. A combined cardiac endpoint included maternal cardiac death, arrhythmia requiring treatment, heart failure, thromboembolic event, aortic dissection, endocarditis, acute coronary syndrome, hospitalisation for cardiac reason or intervention. Associations between patient characteristics, country characteristics (income inequality expressed as Gini coefficient, health expenditure, schooling, gross domestic product, birth rate and hospital beds) and cardiac endpoints were checked in a three-level model (patient-centre-country). Results A total of 30 countries enrolled 2924 patients from 89 centres. At least one endpoint occurred in 645 women (22.1%). Maternal age, New York Heart Association classification and modified WHO risk classification were associated with the combined endpoint and explained 37% of variance in outcome. Gini coefficient and country-specific birth rate explained an additional 4%. There were large differences between the individual countries, but the need for multilevel modelling to account for these differences disappeared after adjustment for patient characteristics, Gini and country-specific birth rate. Conclusion While there are definite interregional differences in pregnancy outcome in women with cardiac disease, these differences seem to be mainly driven by individual patient characteristics. Adjustment for country characteristics refined the results to a limited extent, but maternal condition seems to be the main determinant of outcome.

Published
2018

23. Hipertensión pulmonar tromboembólica crónica en España: una década de cambio

Author

Martínez-Santos, Paula, Velázquez-Martín, María Teresa, Barberá, Joan Albert, Fernández Pérez, Cristina, López-Meseguer, Manuel, López-Reyes, Raquel, Martínez-Meñaca, Amaya, Lara-Padrón, Antonio, Domingo-Morera, Juan Antonio, Blanco, Isabel, and Escribano-Subías, Pilar

Abstract

El tratamiento de la hipertensión pulmonar tromboembólica crónica (HTPTEC) ha evolucionado en la última década. Sin embargo, apenas se dispone de información sobre el impacto de estos logros en la población general a escala nacional. Este estudio se diseñó para describir las características de los pacientes con HTPTEC en España en la última década.

Published
2021
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24. Tromboendarterectomía pulmonar en pacientes con hipertensión pulmonar tromboembólica crónica y afectación distal

Author

Gotor-Pérez, Consuelo A., López-Gude, María J., Benito-Arnaiz, Victoria, Pérez de la Sota, Enrique, Centeno-Rodríguez, Jorge E., Eixerés-Esteve, Andrea, Aguilar-Blanco, Eva M., Barajas-Díaz, Carolina, Velázquez-Martín, María T., Pérez-Núñez, Marta, Pérez-Vela, José L., Escribano-Subías, Pilar, and Cortina-Romero, José M.

Abstract

La hipertensión pulmonar tromboembólica crónica es una rara consecuencia de la embolia pulmonar aguda. La cirugía de tromboendarterectomía pulmonar es el tratamiento de elección. Cuando la afectación es distal (nivel iiianatomoquirúrgico o segmentario) supone un mayor desafío quirúrgico y presenta mayor riesgo de morbimortalidad perioperatoria. Analizamos los resultados de la tromboendarterectomía pulmonar en pacientes con hipertensión pulmonar tromboembólica crónica nivel i-iiy nivel iiien nuestro centro.

Published
2020
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26. Tratamiento multimodal en la hipertensión pulmonar residual tras tromboendarterectomía pulmonar

Author

López-Gude, María Jesús, Coronel, Lorena, Velazquez-Martín, Teresa, Pérez-de la Sota, Enrique, Centeno-Rodríguez, Jorge E., Eixerés-Esteve, Andrea, Aguilar-Blanco, Eva, Meca-Aguirrezabalaga, Juan, Escribano-Subías, Pilar, and Muñoz-Guijosa, Christian

Abstract

La cirugía de tromboendarterectomía pulmonar es el tratamiento de elección en la hipertensión pulmonar tromboembólica crónica. El tratamiento médico o el intervencionismo (angioplastia pulmonar) deben contemplarse en pacientes inoperables o con hipertensión pulmonar residual tras cirugía.

Published
2024
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27. Actualización de la guía de práctica clínica española sobre infección del tracto urinario en la población pediátrica. Síntesis de las recomendaciones sobre diagnóstico, tratamiento y seguimiento

Author

González Rodríguez, Juan David, Fraga Rodríguez, Gloria María, García Vera, César Joaquín, Gómez Fraile, Andrés, Martín Sánchez, Juan Ignacio, Mengual Gil, José María, Ochoa Sangrador, Carlos, Valenciano Fuentes, Blanca, and Escribano Subías, Joaquín

Abstract

En las últimas décadas, se han producido profundos cambios en el manejo de la infección urinaria en pediatría, a partir de estudios que cuestionaban la eficacia de las estrategias para prevenir el daño renal y la evolución posterior a enfermedad renal crónica, muy poco probable en la mayoría de los casos. No obstante, todavía existe una gran variabilidad clínica en el manejo e incertidumbre en cuestiones relativas al diagnóstico, la indicación de una exploración radiológica, el tratamiento o el seguimiento de estos niños. La actualización de la guía de práctica clínica (GPC) española ha examinado en la literatura publicada desde el año 2009 y de modo riguroso aspectos de la práctica actual, considerando la evidencia de los beneficios de cada intervención, junto con los riesgos asociados y desventajas, para intentar delimitar las pautas de actuación más precisas.

Published
2024
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28. Pulmonary hypertension and pregnancy outcomes: Data from the Registry of Pregnancy and Cardiac Disease (ROPAC) of the European Society of Cardiology

Author

Sliwa, Karen, van Hagen, Iris M., Budts, Werner, Swan, Lorna, Sinagra, Gianfranco, Caruana, Maryanne, Blanco, Manuel Vazquez, Wagenaar, Lodewijk J., Johnson, Mark R., Webb, Gary, Hall, Roger, Roos-Hesselink, Jolien W., Ferrari, Roberto, Maggioni, Aldo P., Marelli, Ariane, Kaemmerer, Harald, Popelova, Jana, Tavazzi, Luigi, Parsonage, William Anthony, Stein, Joerg, Elkayam, Uri, Thilen, Ulf, Ruys, Titia, Vardas, Panos, Komajda, Michel, Pinto, Fausto, Alonso, Angeles, Wood, David, Maniadakis, Nikolaos, Ferreira, Thierry, Gracia, Gérard, Laroche, Cécile, Missiamenou, Viviane, Taylor, Charles, Konte, Marème, Andarala, Maryna, Fiorucci, Emanuela, Lefrancq, Elin Folkesson, Glémot, Myriam, McNeill, Patti Ann, Pommier, Caroline, Lafay, Myriam, Aquieri, A., Vega, H. Ruda, Lust, K., Fagermo, N., Gabriel, H., Donhauser, E., Gasimov, Z., Jahangirov, T., Hasanova, I., De Backer, J., Demulier, L., de Hosson, M., Beckx, M., Moissens, M., Kovacevic-Preradovic, T., Kozic, M., Lovric, M., Freire, C. Vilas, Chilingirova, N., Kratunkov, P., Montesclaros, A. R., Beaubien, E., Gordon, E., Walter, L., Lindsay, C., Wahab, N., Vavera, Z., El Nagar, A., Ebaid, H. H., El Sayed Makled, W. A., Taha, N., Dardier, A., Shabaan, M., Elrakshy, Y., Eltamawey, K., Abd-El Aziz, M. Gamal, Saad, Aly, Aboleineen, W., Ashour, Z., Sorour, K., Mahdy, M. A.Meguid, Iserin, L., Ladouceur, M., Cohen, S., Iung, B., Maisuradze, D., Mebus, S., Gembruch, U., Hammerstingl, C., Merz, W. M., Wald, C., Baumgartner, H., Orwat, S., Schmidt, R., Motz, R., Olsson, A., Berger, F., Nagdyman, N., Frogoudaki, A., Anastasiou-Nana, M., Temesvari, A., Kohalmi, D., Balint, H., Merkely, B., Liptai, C., Bowen, M., Cullen, M., Thornton, P., Husarova, V., Blatt, A., Elbaz-Greener, G., Moravsky, G., Vered, Z., Fuhrmann, A. Vazan, Shotan, A., Goland, S., Festa, P., Ali, L. Ait, Puggia, I., Mottolese, B. D.Agata, Carmina, M. G., Romeo, C., Crepaz, R., Fesslova, V., Azzarelli, A., Baldi, D., Bovenzi, F., Donvito, V., Vasario, E., Todros, T., Niwa, K., Mussagaliyeva, A., Mekebekova, D., Sharipova, S., Zaliunas, R., Jonkaitiene, R., Petrauskaite, J., Gumbiene, L., Jovanova, S., Cassar, A., Karamermer, Y., Cornette, J. M.J., van Dijk, A., Bellersen, L., Duijnhouwer, T., De Groot, C., Pieper, E. P.G., van Oppen, C., Polak, P., Wajon, E., Estensen, M., Lesniak-Sobelga, A., Podolec, P., Wisniowska-Smialek, S., Trybuch, A., Hoffman, P., Cichocka-Radwan, A., Lelonek, M., Sobczak, S., Faflik, U., Tomaszuk-Kazberuk, A., Przepiesc, J., Gil, M., Plaskota, K., Trojnarska, O., Guerra, N., de Sousa, L., Petrescu, V., Ginghina, C., Jurcut, R., Coman, I. Mircea, Gaisin, I. Ravilevich, Shilina, L. Valeryevna, Sharashkina, N., Tkacheva, O., Ivanov, D., Irtyuga, O., Jovovic, L., Prokselj, K., Kozelj, M., Elliott, C., Galian-Gay, L., Pijuan-Domenech, A., Subirana-Domenech, M. T., Tornos, P., Murga, N., Oliver, J. M., Escribano-Subías, P., Ruiz-Cano, M. J., Delgado-Jiménez, J., Furenas, E., Dellborg, M., Thilén, U., Schwerzmann, M., Bouchardy, J., Rutz, T., Tobler, D., Sarac, L., Esen, O. Batukan, Enar, S. Catirli, Al Mulla, A., Bazargani, N., Al Hatou, E., Farook, F., Almahmeed, W., Salih, B., Clifford, P., Bowers, N., Veldtman, G., Kerr, J., Tellett, L., Hudsmith, L., Thompson, P., Thorne, S., Bowater, S., Nihoyannopoulos, P., Curry, R., Freeman, L., Schroeder, F., Wendler, R., Hammond, S., Talluto, C., Murphy, D., Perlroth, M. G., Chintala, K., Gupta, P., Pare, E., Khatri, N., Scott, N., De Faria-Yeh, D., Bhatt, A. B., Tsiaras, S., Gurvitz, M., Otto, C., Botti, J., Ting, J., Davidson, W. R., Sliwa, Karen, van Hagen, Iris M., Budts, Werner, Swan, Lorna, Sinagra, Gianfranco, Caruana, Maryanne, Blanco, Manuel Vazquez, Wagenaar, Lodewijk J., Johnson, Mark R., Webb, Gary, Hall, Roger, Roos-Hesselink, Jolien W., Ferrari, Roberto, Maggioni, Aldo P., Marelli, Ariane, Kaemmerer, Harald, Popelova, Jana, Tavazzi, Luigi, Parsonage, William Anthony, Stein, Joerg, Elkayam, Uri, Thilen, Ulf, Ruys, Titia, Vardas, Panos, Komajda, Michel, Pinto, Fausto, Alonso, Angeles, Wood, David, Maniadakis, Nikolaos, Ferreira, Thierry, Gracia, Gérard, Laroche, Cécile, Missiamenou, Viviane, Taylor, Charles, Konte, Marème, Andarala, Maryna, Fiorucci, Emanuela, Lefrancq, Elin Folkesson, Glémot, Myriam, McNeill, Patti Ann, Pommier, Caroline, Lafay, Myriam, Aquieri, A., Vega, H. Ruda, Lust, K., Fagermo, N., Gabriel, H., Donhauser, E., Gasimov, Z., Jahangirov, T., Hasanova, I., De Backer, J., Demulier, L., de Hosson, M., Beckx, M., Moissens, M., Kovacevic-Preradovic, T., Kozic, M., Lovric, M., Freire, C. Vilas, Chilingirova, N., Kratunkov, P., Montesclaros, A. R., Beaubien, E., Gordon, E., Walter, L., Lindsay, C., Wahab, N., Vavera, Z., El Nagar, A., Ebaid, H. H., El Sayed Makled, W. A., Taha, N., Dardier, A., Shabaan, M., Elrakshy, Y., Eltamawey, K., Abd-El Aziz, M. Gamal, Saad, Aly, Aboleineen, W., Ashour, Z., Sorour, K., Mahdy, M. A.Meguid, Iserin, L., Ladouceur, M., Cohen, S., Iung, B., Maisuradze, D., Mebus, S., Gembruch, U., Hammerstingl, C., Merz, W. M., Wald, C., Baumgartner, H., Orwat, S., Schmidt, R., Motz, R., Olsson, A., Berger, F., Nagdyman, N., Frogoudaki, A., Anastasiou-Nana, M., Temesvari, A., Kohalmi, D., Balint, H., Merkely, B., Liptai, C., Bowen, M., Cullen, M., Thornton, P., Husarova, V., Blatt, A., Elbaz-Greener, G., Moravsky, G., Vered, Z., Fuhrmann, A. Vazan, Shotan, A., Goland, S., Festa, P., Ali, L. Ait, Puggia, I., Mottolese, B. D.Agata, Carmina, M. G., Romeo, C., Crepaz, R., Fesslova, V., Azzarelli, A., Baldi, D., Bovenzi, F., Donvito, V., Vasario, E., Todros, T., Niwa, K., Mussagaliyeva, A., Mekebekova, D., Sharipova, S., Zaliunas, R., Jonkaitiene, R., Petrauskaite, J., Gumbiene, L., Jovanova, S., Cassar, A., Karamermer, Y., Cornette, J. M.J., van Dijk, A., Bellersen, L., Duijnhouwer, T., De Groot, C., Pieper, E. P.G., van Oppen, C., Polak, P., Wajon, E., Estensen, M., Lesniak-Sobelga, A., Podolec, P., Wisniowska-Smialek, S., Trybuch, A., Hoffman, P., Cichocka-Radwan, A., Lelonek, M., Sobczak, S., Faflik, U., Tomaszuk-Kazberuk, A., Przepiesc, J., Gil, M., Plaskota, K., Trojnarska, O., Guerra, N., de Sousa, L., Petrescu, V., Ginghina, C., Jurcut, R., Coman, I. Mircea, Gaisin, I. Ravilevich, Shilina, L. Valeryevna, Sharashkina, N., Tkacheva, O., Ivanov, D., Irtyuga, O., Jovovic, L., Prokselj, K., Kozelj, M., Elliott, C., Galian-Gay, L., Pijuan-Domenech, A., Subirana-Domenech, M. T., Tornos, P., Murga, N., Oliver, J. M., Escribano-Subías, P., Ruiz-Cano, M. J., Delgado-Jiménez, J., Furenas, E., Dellborg, M., Thilén, U., Schwerzmann, M., Bouchardy, J., Rutz, T., Tobler, D., Sarac, L., Esen, O. Batukan, Enar, S. Catirli, Al Mulla, A., Bazargani, N., Al Hatou, E., Farook, F., Almahmeed, W., Salih, B., Clifford, P., Bowers, N., Veldtman, G., Kerr, J., Tellett, L., Hudsmith, L., Thompson, P., Thorne, S., Bowater, S., Nihoyannopoulos, P., Curry, R., Freeman, L., Schroeder, F., Wendler, R., Hammond, S., Talluto, C., Murphy, D., Perlroth, M. G., Chintala, K., Gupta, P., Pare, E., Khatri, N., Scott, N., De Faria-Yeh, D., Bhatt, A. B., Tsiaras, S., Gurvitz, M., Otto, C., Botti, J., Ting, J., and Davidson, W. R.

Abstract

Aims: To describe the outcomes of pregnancy in women with pulmonary hypertension. Methods and results: In 2007 the European Registry on Pregnancy and Heart Disease was initiated by the European Society of Cardiology. Consecutive patients with all forms of cardiovascular disease, presenting with pregnancy, were enrolled with the aim of investigating the pregnancy outcomes. This subgroup of the cohort included 151 women with pulmonary hypertension (PH) either diagnosed by right heart catheterization or diagnosed as possible PH by echocardiographic signs, with 26% having pulmonary arterial hypertension (PAH), in three subgroups: idiopathic (iPAH), associated with congenital heart disease (CHD-PAH), or associated with other disease (oPAH), and 74% having PH caused by left heart disease (LHD-PH, n = 112). Maternal mean age was 29.2 ± 5.6 years and 37% were nulliparous. Right ventricular systolic pressure was <50 mmHg in 59.6% of patients, 50–70 mmHg in 28.5% and >70 mmHg in 11.9%. In more than 75% of patients, the diagnosis of PH had been made before pregnancy. Maternal death up to 1 week after delivery occurred in five patients (3.3%), with another two out of 78 patients who presented for follow-up (2.6%), dying within 6 months after delivery. The highest mortality was found in iPAH (3/7, 43%). During pregnancy, heart failure occurred in 27%. Caesarean section was performed in 63.4% (23.9% as emergency). Therapeutic abortion was performed in 4.0%. Complications included miscarriage (5.6%), fetal mortality (2%), premature delivery (21.7%), low birth weight (19.0%), and neonatal mortality (0.7%). Conclusion: Mortality in this group of patients with various forms of PH was lower than previously reported as specialized care during pregnancy and delivery was available. However, maternal and fetal mortality remains prohibitively high in women with iPAH, although this conclusion is restricted by limited numbers. Early advice on c

Published
2016

29. Risk of pregnancy in moderate and severe aortic stenosis: From the multinational ROPAC registry

Author

Orwat, Stefan, Diller, Gerhard Paul, van Hagen, Iris M., Schmidt, Renate, Tobler, Daniel, Greutmann, Matthias, Jonkaitiene, Regina, Elnagar, Amro, Johnson, Mark R., Hall, Roger, Roos-Hesselink, Jolien W., Baumgartner, Helmut, Ferrari, Roberto, Maggioni, Aldo P., Marelli, Ariane, Webb, Gary, Kaemmerer, Harald, Popelova, Jana, Sliwa, Karen, Tavazzi, Luigi, Parsonage, William Anthony, Stein, Joerg, Elkayam, Uri, Thilen, Ulf, Budts, Werner, Ruys, Titia, Vardas, Panos, Komajda, Michel, Pinto, Fausto, Alonso, Angeles, Wood, David, Maniadakis, Nikolaos, Ferreira, Thierry, Gracia, Gérard, Laroche, Cécile, Missiamenou, Viviane, Taylor, Charles, Konte, Marème, Andarala, Maryna, Fiorucci, Emanuela, Lefrancq, Elin Folkesson, Glémot, Myriam, McNeill, Patti Ann, Pommier, Caroline, Lafay, Myriam, Aquieri, A., Vega, H. Ruda, Blanco, M. Vázquez, Lust, K., Fagermo, N., Gabriel, H., Donhauser, E., Gasimov, Z., Jahangirov, T., Hasanova, I., De Backer, J., Demulier, L., de Hosson, M., Beckx, M., Moissens, M., Kovacevic-Preradovic, T., Kozic, M., Lovric, M., Freire, C. Vilas, Chilingirova, N., Kratunkov, P., Montesclaros, A. R., Beaubien, E., Gordon, E., Walter, L., Lindsay, C., Wahab, N., Vavera, Z., El Nagar, A., Ebaid, H. H., El Sayed Makled, W. A., Taha, N., Dardier, A., Shabaan, M., Elrakshy, Y., Eltamawey, K., Abd-El Aziz, M. Gamal, Saad, A., Aboleineen, W., Ashour, Z., Sorour, K., Mahdy, M. A.Meguid, Iserin, L., Ladouceur, M., Cohen, S., Iung, B., Maisuradze, D., Mebus, S., Gembruch, U., Hammerstingl, C., Merz, W. M., Wald, C., Motz, R., Olsson, A., Berger, F., Nagdyman, N., Frogoudaki, A., Anastasiou-Nana, M., Temesvari, A., Kohalmi, D., Balint, H., Merkely, B., Liptai, C., Bowen, M., Cullen, M., Thornton, P., Husarova, V., Blatt, A., Elbaz-Greener, G., Moravsky, G., Vered, Z., Fuhrmann, A. Vazan, Shotan, A., Goland, S., Festa, P., Ali, L. Ait, Sinagra, G., Puggia, I., Mottolese, B. D.Agata, Carmina, M. G., Romeo, C., Crepaz, R., Fesslova, V., Azzarelli, A., Baldi, D., Bovenzi, F., Donvito, V., Vasario, E., Todros, T., Niwa, K., Mussagaliyeva, A., Mekebekova, D., Sharipova, S., Zaliunas, R., Petrauskaite, J., Gumbiene, L., Jovanova, S., Cassar, A., Caruana, M., Karamermer, Y., Cornette, J. M.J., van Dijk, A., Bellersen, L., Duijnhouwer, T., De Groot, C., Pieper, E. P.G., van Oppen, C., Polak, P., Wajon, E., Wagenaar, L., Estensen, M., Lesniak-Sobelga, A., Podolec, P., Wisniowska-Smialek, S., Trybuch, A., Hoffman, P., Cichocka-Radwan, A., Lelonek, M., Sobczak, S., Faflik, U., Tomaszuk-Kazberuk, A., Przepiesc, J., Gil, M., Plaskota, K., Trojnarska, O., Guerra, N., de Sousa, L., Petrescu, V., Ginghina, C., Jurcut, R., Coman, I. Mircea, Gaisin, I. Ravilevich, Shilina, L. Valeryevna, Sharashkina, N., Tkacheva, O., Ivanov, D., Irtyuga, O., Jovovic, L., Prokselj, K., Kozelj, M., Elliott, C., Galian-Gay, L., Pijuan-Domenech, A., Subirana-Domenech, M. T., Tornos, P., Murga, N., Oliver, J. M., Escribano-Subías, P., Ruiz-Cano, M. J., Delgado-Jiménez, J., Furenas, E., Dellborg, M., Thilén, U., Schwerzmann, M., Bouchardy, J., Rutz, T., Sarac, L., Esen, O. Batukan, Enar, S. Catirli, Al Mulla, A., Bazargani, N., Al Hatou, E., Farook, F., Almahmeed, W., Salih, B., Clifford, P., Bowers, N., Veldtman, G., Kerr, J., Tellett, L., Hudsmith, L., Thompson, P., Thorne, S., Bowater, S., Nihoyannopoulos, P., Curry, R., Freeman, L., Schroeder, F., Wendler, R., Hammond, S., Talluto, C., Murphy, D., Perlroth, M. G., Chintala, K., Gupta, P., Pare, E., Khatri, N., Scott, N., De Faria-Yeh, D., Bhatt, A. B., Tsiaras, S., Gurvitz, M., Otto, C., Botti, J., Ting, J., Davidson, W. R., Orwat, Stefan, Diller, Gerhard Paul, van Hagen, Iris M., Schmidt, Renate, Tobler, Daniel, Greutmann, Matthias, Jonkaitiene, Regina, Elnagar, Amro, Johnson, Mark R., Hall, Roger, Roos-Hesselink, Jolien W., Baumgartner, Helmut, Ferrari, Roberto, Maggioni, Aldo P., Marelli, Ariane, Webb, Gary, Kaemmerer, Harald, Popelova, Jana, Sliwa, Karen, Tavazzi, Luigi, Parsonage, William Anthony, Stein, Joerg, Elkayam, Uri, Thilen, Ulf, Budts, Werner, Ruys, Titia, Vardas, Panos, Komajda, Michel, Pinto, Fausto, Alonso, Angeles, Wood, David, Maniadakis, Nikolaos, Ferreira, Thierry, Gracia, Gérard, Laroche, Cécile, Missiamenou, Viviane, Taylor, Charles, Konte, Marème, Andarala, Maryna, Fiorucci, Emanuela, Lefrancq, Elin Folkesson, Glémot, Myriam, McNeill, Patti Ann, Pommier, Caroline, Lafay, Myriam, Aquieri, A., Vega, H. Ruda, Blanco, M. Vázquez, Lust, K., Fagermo, N., Gabriel, H., Donhauser, E., Gasimov, Z., Jahangirov, T., Hasanova, I., De Backer, J., Demulier, L., de Hosson, M., Beckx, M., Moissens, M., Kovacevic-Preradovic, T., Kozic, M., Lovric, M., Freire, C. Vilas, Chilingirova, N., Kratunkov, P., Montesclaros, A. R., Beaubien, E., Gordon, E., Walter, L., Lindsay, C., Wahab, N., Vavera, Z., El Nagar, A., Ebaid, H. H., El Sayed Makled, W. A., Taha, N., Dardier, A., Shabaan, M., Elrakshy, Y., Eltamawey, K., Abd-El Aziz, M. Gamal, Saad, A., Aboleineen, W., Ashour, Z., Sorour, K., Mahdy, M. A.Meguid, Iserin, L., Ladouceur, M., Cohen, S., Iung, B., Maisuradze, D., Mebus, S., Gembruch, U., Hammerstingl, C., Merz, W. M., Wald, C., Motz, R., Olsson, A., Berger, F., Nagdyman, N., Frogoudaki, A., Anastasiou-Nana, M., Temesvari, A., Kohalmi, D., Balint, H., Merkely, B., Liptai, C., Bowen, M., Cullen, M., Thornton, P., Husarova, V., Blatt, A., Elbaz-Greener, G., Moravsky, G., Vered, Z., Fuhrmann, A. Vazan, Shotan, A., Goland, S., Festa, P., Ali, L. Ait, Sinagra, G., Puggia, I., Mottolese, B. D.Agata, Carmina, M. G., Romeo, C., Crepaz, R., Fesslova, V., Azzarelli, A., Baldi, D., Bovenzi, F., Donvito, V., Vasario, E., Todros, T., Niwa, K., Mussagaliyeva, A., Mekebekova, D., Sharipova, S., Zaliunas, R., Petrauskaite, J., Gumbiene, L., Jovanova, S., Cassar, A., Caruana, M., Karamermer, Y., Cornette, J. M.J., van Dijk, A., Bellersen, L., Duijnhouwer, T., De Groot, C., Pieper, E. P.G., van Oppen, C., Polak, P., Wajon, E., Wagenaar, L., Estensen, M., Lesniak-Sobelga, A., Podolec, P., Wisniowska-Smialek, S., Trybuch, A., Hoffman, P., Cichocka-Radwan, A., Lelonek, M., Sobczak, S., Faflik, U., Tomaszuk-Kazberuk, A., Przepiesc, J., Gil, M., Plaskota, K., Trojnarska, O., Guerra, N., de Sousa, L., Petrescu, V., Ginghina, C., Jurcut, R., Coman, I. Mircea, Gaisin, I. Ravilevich, Shilina, L. Valeryevna, Sharashkina, N., Tkacheva, O., Ivanov, D., Irtyuga, O., Jovovic, L., Prokselj, K., Kozelj, M., Elliott, C., Galian-Gay, L., Pijuan-Domenech, A., Subirana-Domenech, M. T., Tornos, P., Murga, N., Oliver, J. M., Escribano-Subías, P., Ruiz-Cano, M. J., Delgado-Jiménez, J., Furenas, E., Dellborg, M., Thilén, U., Schwerzmann, M., Bouchardy, J., Rutz, T., Sarac, L., Esen, O. Batukan, Enar, S. Catirli, Al Mulla, A., Bazargani, N., Al Hatou, E., Farook, F., Almahmeed, W., Salih, B., Clifford, P., Bowers, N., Veldtman, G., Kerr, J., Tellett, L., Hudsmith, L., Thompson, P., Thorne, S., Bowater, S., Nihoyannopoulos, P., Curry, R., Freeman, L., Schroeder, F., Wendler, R., Hammond, S., Talluto, C., Murphy, D., Perlroth, M. G., Chintala, K., Gupta, P., Pare, E., Khatri, N., Scott, N., De Faria-Yeh, D., Bhatt, A. B., Tsiaras, S., Gurvitz, M., Otto, C., Botti, J., Ting, J., and Davidson, W. R.

Abstract

Background Controversial results on maternal risk and fetal outcome have been reported in women with aortic stenosis (AS). Objectives The authors sought to investigate maternal and fetal outcomes in patients with AS in a large cohort. Methods The Registry on Pregnancy and Cardiac Disease (ROPAC) is a global, prospective observational registry of women with structural heart disease, providing a uniquely large study population. Data of women with moderate (peak gradient 36 to 63 mm Hg) and severe AS (peak gradient ≥64 mm Hg) were analyzed. Results Of 2,966 pregnancies in ROPAC, the authors identified 96 women who had at least moderate AS (34 with severe AS). No deaths were observed during pregnancy and in the first week after delivery. However, 20.8% of women were hospitalized for cardiac reasons during pregnancy. This was significantly more common in severe AS compared with moderate AS (35.3% vs. 12.9%; p = 0.02), and reached the highest rate (42.1%) in severe, symptomatic AS. Pregnancy was complicated by heart failure in 6.7% of asymptomatic and 26.3% of symptomatic patients, but could be managed medically, except for 1 patient who was symptomatic before pregnancy and underwent balloon valvotomy. Children of patients with severe AS had a significantly higher percentage of low birth weight (35.0% vs. 6.0%; p = 0.006). Conclusions Mortality in pregnant women with AS, including those with severe AS, appears to be close to zero in the current era. Symptomatic and severe AS does, however, carry a substantial risk of heart failure and is associated with high rates of hospitalization for cardiac reasons, although heart failure can nearly always be managed medically. The results highlight the importance of appropriate pre-conceptional patient evaluation and counseling.

Published
2016

30. Management and outcomes in chronic thromboembolic pulmonary hypertension: From expert centers to a nationwide perspective

Author

Universitat Rovira i Virgili, Escribano-Subías P, Del Pozo R, Román-Broto A, Domingo Morera JA, Lara-Padrón A, Elías Hernández T, Molina-Ferragut L, Blanco I, Cortina J, Barberà JA, REHAP Investigators, Universitat Rovira i Virgili, and Escribano-Subías P, Del Pozo R, Román-Broto A, Domingo Morera JA, Lara-Padrón A, Elías Hernández T, Molina-Ferragut L, Blanco I, Cortina J, Barberà JA, REHAP Investigators

Abstract

The Spanish "Registry of Pulmonary Arterial Hypertension" (REHAP), started in 2007, includes chronic thromboembolic hypertension (CTEPH) patients. Based on data provided by this registry and retrospective data from patients diagnosed during 2006 (? 12 months since the registry was created), clinical management and long-term outcomes of CTEPH patients are analyzed nationwide for the first time in a scenario of a decentralized organization model of CTEPH management.A total of 391 patients (median [Q1:Q3] age 63.7 [48.0;73.3] years, 58% females) with CTEPH included during the period January 1, 2006-December 31, 2013 in the REHAP registry were analyzed. Rate of pulmonary endarterectomy (PEA) was 31.2%, and highly asymmetric among centers: rate was 47.9% at two centers designated as CTEPH expert centers, while it was 4.6% in other centers. Among patients not undergoing PEA, 82% were treated with therapies licensed for pulmonary arterial hypertension (PAH). Five-year survival rate was 86.3% for PEA patients, and 64.9% for non-PEA patients. Among non-PEA patients, presenting proximal lesions (42% of non-referred patients) was associated with a 3-fold increase in mortality. PEA patients achieved significantly better hemodynamic and clinical outcomes at one-year follow-up compared to non-PEA patients. Patients not being referred for PEA assessment were older and had a worse functional capacity. Older age was the most deterrent factor for non-operability.Despite the increase in diagnosis and expertise in PEA-specialized centers, an important percentage of patients do not benefit of PEA in a decentralized organization model of CTEPH management.Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

Published
2016

31. Pregnancy in women with a mechanical heart valve: Data of the European Society of Cardiology Registry of Pregnancy and Cardiac Disease (ROPAC)

Author

Van Hagen, Iris M., Roos-Hesselink, Jolien W., Ruys, Titia P.E., Merz, Waltraut M., Goland, Sorel, Gabriel, Harald, Lelonek, Malgorzata, Trojnarska, Olga, Al Mahmeed, Wael Abdulrahman, Balint, Hajnalka Olga, Ashour, Zeinab, Baumgartner, Helmut, Boersma, Eric, Johnson, Mark R., Hall, Roger, Ferrari, Roberto, Maggioni, Aldo P., Marelli, Ariane, Webb, Gary, Kaemmerer, Harald, Popelova, Jana, Sliwa, Karen, Tavazzi, Luigi, Parsonage, William Anthony, Stein, Joerg, Elkayam, Uri, Thilen, Ulf, Budts, Werner, Vardas, Panos, Komajda, Michel, Pinto, Fausto, Alonso, Angeles, Wood, David, Maniadakis, Nikolaos, Ferreira, Thierry, Gracia, Gérard, Laroche, Cécile, Missiamenou, Viviane, Taylor, Charles, Konte, Marème, Andarala, Maryna, Fiorucci, Emanuela, Lefrancq, Elin Folkesson, Glémot, Myriam, McNeill, Patti Ann, Pommier, Caroline, Lafay, Myriam, Aquieri, A., Vega, H. Ruda, Blanco, M. Vázquez, Lust, K., Fagermo, N., Donhauser, E., Gasimov, Z., Jahangirov, T., Hasanova, I., De Backer, J., Demulier, L., de Hosson, M., Beckx, M., Moissens, M., Kovacevic-Preradovic, T., Kozic, M., Lovric, M., Freire, C. Vilas, Chilingirova, N., Kratunkov, P., Montesclaros, A. R., Beaubien, E., Gordon, E., Walter, L., Lindsay, C., Wahab, N., Vavera, Z., El Nagar, A. E., Ebaid, H. H., El Sayed Makled, W. A., Taha, N., Dardier, A., Shabaan, M., Elrakshy, Y., Eltamawey, K., Abd-El Aziz, M. Gamal, Saad, A., Aboleineen, W., Sorour, K., Mahdy, M. A.Meguid, Iserin, L., Ladouceur, M., Cohen, S., Iung, B., Maisuradze, D., Mebus, S., Gembruch, U., Hammerstingl, C., Wald, C., Orwat, S., Schmidt, R., Motz, R., Olsson, A., Berger, F., Nagdyman, N., Frogoudaki, A., Anastasiou-Nana, M., Temesvari, A., Kohalmi, D., Merkely, B., Liptai, C., Bowen, M., Cullen, M., Thornton, P., Husarova, V., Blatt, A., Elbaz-Greener, G., Moravsky, G., Vered, Z., Fuhrmann, A. Vazan, Shotan, A., Festa, P., Ali, L. Ait, Sinagra, G., Puggia, I., Mottolese, B. D.Agata, Carmina, M. G., Romeo, C., Crepaz, R., Fesslova, V., Azzarelli, A., Baldi, D., Bovenzi, F., Donvito, V., Vasario, E., Todros, T., Niwa, K., Mussagaliyeva, A., Mekebekova, D., Sharipova, S., Zaliunas, R., Jonkaitiene, R., Petrauskaite, J., Gumbiene, L., Jovanova, S., Cassar, A., Caruana, M., Karamermer, Y., Cornette, J. M.J., van Dijk, A., Bellersen, L., Duijnhouwer, T., De Groot, C., Pieper, E. P.G., van Oppen, C., Polak, P., Wajon, E., Wagenaar, L., Estensen, M., Lesniak-Sobelga, A., Podolec, P., Wisniowska-Smialek, S., Trybuch, A., Hoffman, P., Cichocka-Radwan, A., Sobczak, S., Faflik, U., Tomaszuk-Kazberuk, A., Przepiesc, J., Gil, M., Plaskota, K., Guerra, N., de Sousa, L., Petrescu, V., Ginghina, C., Jurcut, R., Coman, I. Mircea, Gaisin, I. Ravilevich, Shilina, L. Valeryevna, Sharashkina, N., Tkacheva, O., Ivanov, D., Irtyuga, O., Jovovic, L., Prokselj, K., Kozelj, M., Elliott, C., Galian-Gay, L., Pijuan-Domenech, A., Subirana-Domenech, M. T., Tornos, P., Murga, N., Oliver, J. M., Escribano-Subías, P., Ruiz-Cano, M. J., Delgado-Jiménez, J., Furenas, E., Dellborg, M., Thilén, U., Schwerzmann, M., Bouchardy, J., Rutz, T., Tobler, D., Sarac, L., Esen, O. B., Enar, S. C., Al Mulla, A., Bazargani, N., Al Hatou, E., Farook, F., Salih, B., Clifford, P., Bowers, N., Veldtman, G., Kerr, J., Tellett, L., Hudsmith, L., Thompson, P., Thorne, S., Bowater, S., Nihoyannopoulos, P., Curry, R., Freeman, L., Schroeder, F., Wendler, R., Hammond, S., Talluto, C., Murphy, D., Perlroth, M. G., Chintala, K., Gupta, P., Pare, E., Khatri, N., Scott, N., De Faria-Yeh, D., Bhatt, A. B., Tsiaras, S., Gurvitz, M., Otto, C., Botti, J., Ting, J., Davidson, W. R., Van Hagen, Iris M., Roos-Hesselink, Jolien W., Ruys, Titia P.E., Merz, Waltraut M., Goland, Sorel, Gabriel, Harald, Lelonek, Malgorzata, Trojnarska, Olga, Al Mahmeed, Wael Abdulrahman, Balint, Hajnalka Olga, Ashour, Zeinab, Baumgartner, Helmut, Boersma, Eric, Johnson, Mark R., Hall, Roger, Ferrari, Roberto, Maggioni, Aldo P., Marelli, Ariane, Webb, Gary, Kaemmerer, Harald, Popelova, Jana, Sliwa, Karen, Tavazzi, Luigi, Parsonage, William Anthony, Stein, Joerg, Elkayam, Uri, Thilen, Ulf, Budts, Werner, Vardas, Panos, Komajda, Michel, Pinto, Fausto, Alonso, Angeles, Wood, David, Maniadakis, Nikolaos, Ferreira, Thierry, Gracia, Gérard, Laroche, Cécile, Missiamenou, Viviane, Taylor, Charles, Konte, Marème, Andarala, Maryna, Fiorucci, Emanuela, Lefrancq, Elin Folkesson, Glémot, Myriam, McNeill, Patti Ann, Pommier, Caroline, Lafay, Myriam, Aquieri, A., Vega, H. Ruda, Blanco, M. Vázquez, Lust, K., Fagermo, N., Donhauser, E., Gasimov, Z., Jahangirov, T., Hasanova, I., De Backer, J., Demulier, L., de Hosson, M., Beckx, M., Moissens, M., Kovacevic-Preradovic, T., Kozic, M., Lovric, M., Freire, C. Vilas, Chilingirova, N., Kratunkov, P., Montesclaros, A. R., Beaubien, E., Gordon, E., Walter, L., Lindsay, C., Wahab, N., Vavera, Z., El Nagar, A. E., Ebaid, H. H., El Sayed Makled, W. A., Taha, N., Dardier, A., Shabaan, M., Elrakshy, Y., Eltamawey, K., Abd-El Aziz, M. Gamal, Saad, A., Aboleineen, W., Sorour, K., Mahdy, M. A.Meguid, Iserin, L., Ladouceur, M., Cohen, S., Iung, B., Maisuradze, D., Mebus, S., Gembruch, U., Hammerstingl, C., Wald, C., Orwat, S., Schmidt, R., Motz, R., Olsson, A., Berger, F., Nagdyman, N., Frogoudaki, A., Anastasiou-Nana, M., Temesvari, A., Kohalmi, D., Merkely, B., Liptai, C., Bowen, M., Cullen, M., Thornton, P., Husarova, V., Blatt, A., Elbaz-Greener, G., Moravsky, G., Vered, Z., Fuhrmann, A. Vazan, Shotan, A., Festa, P., Ali, L. Ait, Sinagra, G., Puggia, I., Mottolese, B. D.Agata, Carmina, M. G., Romeo, C., Crepaz, R., Fesslova, V., Azzarelli, A., Baldi, D., Bovenzi, F., Donvito, V., Vasario, E., Todros, T., Niwa, K., Mussagaliyeva, A., Mekebekova, D., Sharipova, S., Zaliunas, R., Jonkaitiene, R., Petrauskaite, J., Gumbiene, L., Jovanova, S., Cassar, A., Caruana, M., Karamermer, Y., Cornette, J. M.J., van Dijk, A., Bellersen, L., Duijnhouwer, T., De Groot, C., Pieper, E. P.G., van Oppen, C., Polak, P., Wajon, E., Wagenaar, L., Estensen, M., Lesniak-Sobelga, A., Podolec, P., Wisniowska-Smialek, S., Trybuch, A., Hoffman, P., Cichocka-Radwan, A., Sobczak, S., Faflik, U., Tomaszuk-Kazberuk, A., Przepiesc, J., Gil, M., Plaskota, K., Guerra, N., de Sousa, L., Petrescu, V., Ginghina, C., Jurcut, R., Coman, I. Mircea, Gaisin, I. Ravilevich, Shilina, L. Valeryevna, Sharashkina, N., Tkacheva, O., Ivanov, D., Irtyuga, O., Jovovic, L., Prokselj, K., Kozelj, M., Elliott, C., Galian-Gay, L., Pijuan-Domenech, A., Subirana-Domenech, M. T., Tornos, P., Murga, N., Oliver, J. M., Escribano-Subías, P., Ruiz-Cano, M. J., Delgado-Jiménez, J., Furenas, E., Dellborg, M., Thilén, U., Schwerzmann, M., Bouchardy, J., Rutz, T., Tobler, D., Sarac, L., Esen, O. B., Enar, S. C., Al Mulla, A., Bazargani, N., Al Hatou, E., Farook, F., Salih, B., Clifford, P., Bowers, N., Veldtman, G., Kerr, J., Tellett, L., Hudsmith, L., Thompson, P., Thorne, S., Bowater, S., Nihoyannopoulos, P., Curry, R., Freeman, L., Schroeder, F., Wendler, R., Hammond, S., Talluto, C., Murphy, D., Perlroth, M. G., Chintala, K., Gupta, P., Pare, E., Khatri, N., Scott, N., De Faria-Yeh, D., Bhatt, A. B., Tsiaras, S., Gurvitz, M., Otto, C., Botti, J., Ting, J., and Davidson, W. R.

Abstract

Background - Pregnant women with a mechanical heart valve (MHV) are at a heightened risk of a thrombotic event, and their absolute need for adequate anticoagulation puts them at considerable risk of bleeding and, with some anticoagulants, fetotoxicity. Methods and Results - Within the prospective, observational, contemporary, worldwide Registry of Pregnancy and Cardiac disease (ROPAC), we describe the pregnancy outcome of 212 patients with an MHV. We compare them with 134 patients with a tissue heart valve and 2620 other patients without a prosthetic valve. Maternal mortality occurred in 1.4% of the patients with an MHV, in 1.5% of patients with a tissue heart valve (P=1.000), and in 0.2% of patients without a prosthetic valve (P=0.025). Mechanical valve thrombosis complicated pregnancy in 10 patients with an MHV (4.7%). In 5 of these patients, the valve thrombosis occurred in the first trimester, and all 5 patients had been switched to some form of heparin. Hemorrhagic events occurred in 23.1% of patients with an MHV, in 5.1% of patients with a tissue heart valve (P<0.001), and in 4.9% of patients without a prosthetic valve (P<0.001). Only 58% of the patients with an MHV had a pregnancy free of serious adverse events compared with 79% of patients with a tissue heart valve (P<0.001) and 78% of patients without a prosthetic valve (P<0.001). Vitamin K antagonist use in the first trimester compared with heparin was associated with a higher rate of miscarriage (28.6% versus 9.2%; P<0.001) and late fetal death (7.1% versus 0.7%; P=0.016). Conclusions - Women with an MHV have only a 58% chance of experiencing an uncomplicated pregnancy with a live birth. The markedly increased mortality and morbidity warrant extensive prepregnancy counseling and centralization of care.

Published
2015

32. Defining the clinical validity of genes reported to cause pulmonary arterial hypertension

Author

Welch, Carrie L., Aldred, Micheala A., Balachandar, Srimmitha, Dooijes, Dennis, Eichstaedt, Christina A., Gräf, Stefan, Houweling, Arjan C., Machado, Rajiv D., Pandya, Divya, Prapa, Matina, Shaukat, Memoona, Southgate, Laura, Tenorio-Castano, Jair, Callejo, Emily P., Day, Kristina M., Macaya, Daniela, Maldonado-Velez, Gabriel, Chung, Wendy K., Archer, Stephen L., Auckland, Kathryn, Austin, Eric D., Badagliacca, Roberto, Barberà, Joan-Albert, Belge, Catharina, Bogaard, Harm Jan, Bonnet, Sébastien, Boomars, Karin A., Boucherat, Olivier, Chakinala, Murali M., Condliffe, Robin, Damico, Rachel Lynn, Delcroix, Marion, Desai, Ankit A., Doboszynska, Anna, Elliott, C. Greg, Eyries, Melanie, Escribano Subías, Maria Pilar, Gall, Henning, Ghio, Stefano, Ghofrani, Ardeschir-Hossein, Grünig, Ekkehard, Hamid, Rizwan, Harbaum, Lars, Hassoun, Paul M., Hemnes, Anna R., Hinderhofer, Katrin, Howard, Luke S., Humbert, Marc, Kiely, David G., Langleben, David, Lawrie, Allan, Loyd, Jim E., Moledina, Shahin, Montani, David, Morrell, Nichols W., Nichols, William C., Olschewski, Andrea, Olschewski, Horst, Papa, Silvia, Pauciulo, Mike W., Provencher, Steve, Quarck, Rozenn, Rhodes, Christopher J., Scelsi, Laura, Seeger, Werner, Stewart, Duncan J., Sweatt, Andrew, Swietlik, Emilia M., Treacy, Carmen, Trembath, Richard C., Tura-Ceide, Olga, Vizza, Carmine Dario, Noordegraaf, Anton Vonk, Wilkins, Martin R., Zamanian, Roham T., and Zateyshchikov, Dmitry

Abstract

Pulmonary arterial hypertension (PAH) is a rare, progressive vasculopathy with significant cardiopulmonary morbidity and mortality. Genetic testing is currently recommended for adults diagnosed with heritable, idiopathic, anorexigen-, hereditary hemorrhagic telangiectasia–, and congenital heart disease–associated PAH, PAH with overt features of venous/capillary involvement, and all children diagnosed with PAH. Variants in at least 27 genes have putative evidence for PAH causality. Rigorous assessment of the evidence is needed to inform genetic testing.

Published
2023
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33. A founderEIF2AK4mutation causes an aggressive form of pulmonary arterial hypertension in Iberian Gypsies

Author

Tenorio, J., primary, Navas, P., additional, Barrios, E., additional, Fernández, L., additional, Nevado, J., additional, Quezada, C.A., additional, López-Meseguer, M., additional, Arias, P., additional, Mena, R., additional, Lobo, J.L., additional, Alvarez, C., additional, Heath, K., additional, Escribano-Subías, P., additional, and Lapunzina, P., additional

Published
2015
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34. Manejo de la hipertensión pulmonar tromboembólica crónica. Experiencia de un centro de referencia nacional

Author

López Gude, María Jesús, Santos-Lozano, Alejandro, Pérez de la Sota, Enrique, Centeno Rodríguez, Jorge, Pérez Vela, Jose Luís, Velázquez, María Teresa, Delgado Jiménez, Juan, Morales, Rafael, Aguado García, José María, Escribano Subías, Pilar, and Cortina Romero, José María

Abstract

La tromboendarterectomía pulmonar es el tratamiento de elección y potencialmente curativo de la hipertensión pulmonar tromboembólica crónica; el tratamiento médico quedaría limitado a los pacientes considerados inoperables. Este estudio evalúa los resultados a largo plazo con ambos tratamientos en una Unidad de Referencia Nacional.

Published
2018
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35. Expresividad variable de una mutación fundadora en el gen EIF2AK4en pacientes con enfermedad venooclusiva pulmonar hereditaria. Impacto en la supervivencia

Author

Navas Tejedor, Paula, Palomino Doza, Julián, Tenorio Castaño, Jair Antonio, Enguita Valls, Ana Belén, Rodríguez Reguero, José Julián, Martínez Meñaca, Amaya, Hernández González, Ignacio, Bueno Zamora, Héctor, Lapunzina Badía, Pablo Daniel, and Escribano Subías, Pilar

Abstract

La enfermedad venooclusiva pulmonar (EVOP) hereditaria se relaciona con mutaciones bialélicas en EIF2AK4y se ha descrito una mutación fundadora en pacientes ibéricos de etnia gitana con EVOP familiar. Los objetivos son la caracterización fenotípica y el análisis de supervivencia de pacientes ibéricos de etnia gitana con EVOP familiar portadores de la mutación fundadora p.Pro1115Leu en EIF2AK4, según su tolerancia clínica a vasodilatadores pulmonares (VDP). Estudio genético familiar y análisis de factores socioculturales de la etnia con potencial impacto en la propagación de la enfermedad.

Published
2018
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36. La evolución de la hipertensión arterial pulmonar a lo largo de 30 años: experiencia de un centro de referencia

Author

Quezada Loaiza, Carlos Andrés, Velázquez Martín, María Teresa, Jiménez López-Guarch, Carmen, Ruiz Cano, María José, Navas Tejedor, Paula, Carreira, Patricia Esmeralda, Flox Camacho, Ángela, de Pablo Gafas, Alicia, Delgado Jiménez, Juan Francisco, Gómez Sánchez, Miguel Ángel, and Escribano Subías, Pilar

Abstract

La hipertensión arterial pulmonar (HAP) se caracteriza por aumento de resistencias vasculares pulmonares, disfunción progresiva del ventrículo derecho y muerte. A pesar de los avances, sigue asociada a alta morbimortalidad. El objetivo del estudio es describir el tratamiento de esta enfermedad y determinar factores pronósticos de pacientes con HAP tratados en un centro de referencia nacional a lo largo de 30 años.

Published
2017
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38. Evolución y pronóstico de la insuficiencia tricuspídea moderada-grave tras tromboendarterectomía pulmonar o angioplastia de arterias pulmonares

Author

Hinojosa, Williams, Cruz-Utrilla, Alejandro, Jiménez López-Guarch, Carmen, Velázquez-Martín, Maite, Segura de la Cal, Teresa, Gómez-Burgueño, Lorena, Otero, Macarena, López-Gude, María Jesús, Morales, Rafael, Cortina-Romero, José María, Solís, Jorge, Arribas Ynsurriaga, Fernando, and Escribano-Subías, Pilar

Abstract

No está definido el abordaje de la insuficiencia tricuspídea (IT) funcional moderada-grave en los pacientes con hipertensión pulmonar tromboembólica crónica tras la tromboendarterectomía pulmonar (TEA) o angioplastia con balón de las arterias pulmonares (ABAP). El objetivo de este estudio es analizar la evolución y los predictores de IT residual tras el procedimiento, así como su impacto pronóstico.

Published
2023
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39. Análisis de los genes BMPR2, TBX4y KCNK3y correlación genotipo-fenotipo en pacientes y familias españolas con hipertensión arterial pulmonar

Author

Navas, Paula, Tenorio, Jair, Quezada, Carlos Andrés, Barrios, Elvira, Gordo, Gema, Arias, Pedro, López Meseguer, Manuel, Santos-Lozano, Alejandro, Palomino Doza, Julian, Lapunzina, Pablo, and Escribano Subías, Pilar

Abstract

Avances recientes en genética han permitido el descubrimiento de nuevos genes relacionados con la hipertensión arterial pulmonar, como TBX4y KCNK3. El fenotipo y el pronóstico asociado a ellos se han detallado escasamente y se desconoce su papel en la población española. El objetivo de este estudio es caracterizar genotípicamente una cohorte española de pacientes con hipertensión arterial pulmonar idiopática y hereditaria, describiendo el fenotipo y los factores pronósticos asociados a BMPR2y a los nuevos genes (KCNK3y TBX4).

Published
2016
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41. Transición de prostaciclina intravenosa a subcutánea en la hipertensión pulmonar

Author

Escribano Subías, Pilar, Cea-Calvo, Luis, Tello de Menesses, Rocío, Gómez Sánchez, Miguel A., Delgado Jiménez, Juan F., and Sáenz de la Calzada, Carlos

Abstract

El tratamiento de la hipertensión arterial pulmonar con epoprostenol (prostaciclina intravenosa) mejora la supervivencia y la calidad de vida de los pacientes. Sin embargo, la necesidad de implantar un catéter intravenoso central implica frecuentes complicaciones que en algunos casos (infecciones, desplazamientos) son graves y requieren su reemplazo. Treprostinil es un análogo de la prostaciclina que puede administrarse en infusión continua por vía subcutánea. Describimos la transición de tratamiento con epeprostenol intravenoso a treprostinil subcutáneo en 4 pacientes con hipertensión pulmonar severa que presentaron complicaciones graves asociadas al sistema de infusión de epoprostenol.

Published
2003
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42. Tratamiento de la hipertensión pulmonar asociada a la infección por VIH con treprostinil

Author

Cea-Calvo, Luis, Escribano Subías, Pilar, Tello de Menesses, Rocío, Lázaro Salvador, María, Gómez Sánchez, Miguel A., Delgado Jiménez, Juan F., Pindado Rodríguez, Carlos, Ruiz Cano, María J., and Sáenz de la Calzada, Carlos

Abstract

El tratamiento de la hipertensión pulmonar asociada a infección por virus de la inmunodeficiencia humana no está bien definido. El treprostinil es un análogo de la prostaciclina que recientemente ha demostrado eficacia en el tratamiento de la hipertensión pulmonar primaria, secundaria a cardiopatías congénitas y asociada a colagenosis, en un estudio doble ciego de 12 semanas de duración. Describimos el seguimiento al año de los 3 pacientes con hipertensión pulmonar asociada a infección por virus de la inmunodeficiencia humana tratados con treprostinil en nuestro centro.

Published
2003
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43. Experiencia de 10 años en el tratamiento con epoprostenol en perfusión intravenosa continua en hipertensión pulmonar arterial grave

Author

Pombo Jiménez, Marta, Escribano Subías, Pilar, Tello de Meneses, Rocío, Ángel Gómez-Sánchez, Miguel, Delgado Jiménez, Juan, Dalmau González-Gallarza, Regina, Lázaro Salvador, María, Hernández Rodríguez, Isidro, Tascón Pérez, Juan, and Sáenz de la Calzada, Carlos

Abstract

La hipertensión pulmonar (HTP) primaria y sus formas asociadas tienen un curso habitualmente progresivo y fatal, modificado en la última década por el tratamiento con prostaciclina.

Published
2003
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44. Guías de práctica clínica de la Sociedad Española de Cardiología en tromboembolismo e hipertensión pulmonar

Author

Sáenz de la Calzada, Carlos, Sánchez Sánchez, Violeta, Teresa Velázquez Martín, M., Tello de Meneses, Rocío, Gómez Sánchez, Miguel A., Delgado Jiménez, Juan, Escribano Subías, Pilar, García Gallego, Francisco, Ortigosa Aso, Javier, Melero Pita, Antonio, Lázaro Salvador, María, Gascueña Rubia, Raúl, and Hernández Simón, Pedro

Abstract

La hipertensión pulmonar primaria es una enfermedad de carácter progresivo, más frecuente en mujeres jóvenes y de mediana edad. Su etiología se desconoce, aunque existe una predisposición familiar hasta en un 6% de los casos. Las teorías patogénicas actuales se centran en la existencia de disfunción endotelial y fallos en los canales iónicos de las fibras musculares lisas del vaso. Las pruebas diagnósticas se dirigen a descartar las causas secundarias y a evaluar la gravedad de la enfermedad. El test vasodilatador agudo es imprescindible para la elección del tratamiento más adecuado. La anticoagulación oral está indicada en todos los pacientes. El trasplante de pulmón queda reservado a aquellos casos en los que fracasa el tratamiento médico. La septostomía auricular es un procedimiento paliativo útil en casos seleccionados. La hipertensión pulmonar tromboembólica crónica es una forma especial de hipertensión pulmonar secundaria; aunque indistinguible clínicamente de la hipertensión pulmonar primaria, su diagnóstico resulta crucial, ya que es posible su curación mediante la realización de tromboendarterectomía pulmonar.

Published
2001
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45. Disfunción diastólica en la infección por el virus de la inmunodeficiencia humana

Author

Hernández Hernández, Felipe, Gascueña Rubia, Raúl, Escribano Subías, Pilar, Velázquez Martín, María T., Lombera Romero, Federico, de la Calzada, Carlos Sáenz, Rubio García, Rafael, Pulido Ortegaa, Federico, and Costa Pérez-Herrero, J. Ramón

Abstract

Determinar la prevalencia y características de las alteraciones ecocardiográficas (disfunción sistólica y/o diastólica, derrame pericárdico) en pacientes infectadospor el virus de la inmunodeficiencia humana sin síntomas ni antecedentes de enfermedad cardíaca, y su comparación con un grupo control sano

Published
2001
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48. Tromboendarterectomía pulmonar en pacientes sintomáticos con enfermedad tromboembólica crónica sin hipertensión arterial pulmonar

Author

Gotor-Pérez, Consuelo A., López-Gude, María J., Benito-Arnaiz, Victoria, Pérez-Vela, Jose L., Escribano-Subías, Pilar, and Cortina-Romero, Jose M.

Abstract

La hipertensión pulmonar tromboembólica crónica es una rara consecuencia del embolismo pulmonar agudo. La cirugía de tromboendarterectomía pulmonar es el tratamiento de elección. El tratamiento quirúrgico está empezando a considerarse en pacientes con oclusión vascular crónica significativa e intolerancia al ejercicio, pero sin hipertensión pulmonar en reposo (enfermedad tromboembólica crónica). Desde febrero de 1996, hemos realizado 307 tromboendarterectomías pulmonares consecutivas, 10 (3,2%) presentaban la enfermedad sin hipertensión pulmonar en reposo.

Published
2021
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49. Transición de prostaciclina a bosentán en 5 pacientes con hipertensión pulmonar severa: el cambio es posible

Author

Flox Camacho, Ángela, Escribano Subías, Pilar, Tello de Meneses, Rocío, Delgado Jiménez, Juan, Gómez Sánchez, Miguel A., and Sáenz de la Calzada, Carlos

Abstract

La prostaciclina mejora los síntomas, la capacidad de ejercicio y la supervivencia en los pacientes con hipertensión arterial pulmonar. Sin embargo, sus complejas vías de administración (intravenosa, inhalada, subcutánea) ocasionan frecuentes efectos adversos que disminuyen la calidad de vida y pueden ser graves. Bosentán, un antagonista oral de los receptores de la endotelina, mejora la clase funcional y la capacidad de ejercicio en estos pacientes. Describimos la transición de prostaciclina a bosentán en 5 pacientes con hipertensión arterial pulmonary severa e importantes complicaciones secundarias al tratamiento con prostaciclina.

Published
2006
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50. Aneurisma micótico por Aspergillusen la línea de sutura aórtica tras el trasplante cardíaco

Author

Escribano Subías, Pilar, López Ríos, Fernando, Delgado Jiménez, Juan F., Sotelo Rodríguez, Teresa, Aguado, José M., and Rodríguez Hernández, Enrique

Abstract

:Se describe el primer caso de aneurisma micótico de aorta por Aspergillus en un paciente con trasplante cardíaco, en el que la infección se produjo por contaminación quirúrgica directa de la sutura aórtica. El período de latencia, hasta producir las manifestaciones clínicas, fue de 8 meses. Se comentan la excepcionalidad del caso y sus dificultades diagnósticas.

Published
2000
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