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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.

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.

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.

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

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.

Morbidity and mortality in pulmonary arterial hypertension (PAH) remain high. Activation of platelet-derived growth factor receptor, colony stimulating factor 1 receptor, and mast or stem cell growth factor receptor kinases stimulates inflammatory, proliferative, and fibrotic pathways driving pulmonary vascular remodelling in PAH. Seralutinib, an inhaled kinase inhibitor, targets these pathways. We aimed to evaluate the efficacy and safety of seralutinib in patients with PAH receiving standard background therapy. The TORREY trial was a phase 2, randomised, multicentre, multinational, double-blind, placebo-controlled study. Patients with PAH from 40 hospital and community sites were randomly assigned 1:1 via interactive response technologies to receive seralutinib (60 mg twice daily for 2 weeks, then increased to 90 mg twice daily as tolerated) or placebo by dry powder inhaler twice daily for 24 weeks. Randomisation was stratified by baseline pulmonary vascular resistance (PVR; <800 dyne·s/cm5 and ≥800 dyne·s/cm5). Patients were eligible if classified as WHO Group 1 PH (PAH), WHO Functional Class II or III, with a PVR of 400 dyne·s/cm5 or more, and a 6 min walk distance of between 150 m and 550 m. The primary endpoint was change in PVR from baseline to 24 weeks. Analyses for efficacy endpoints were conducted in randomly assigned patients (intention-to-treat population). Safety analyses included all patients who received the study drug. TORREY was registered with ClinicalTrials.gov (NCT04456998) and EudraCT (2019-002669-37) and is completed. From Nov 12, 2020, to April 20, 2022, 151 patients were screened for eligibility, and following exclusions, 86 adults receiving PAH background therapy were randomly assigned to seralutinib (n=44; four male, 40 female) or placebo (n=42; four male, 38 female), and comprised the intention-to-treat population. At baseline, treatment groups were balanced except for a higher representation of WHO Functional Class II patients in the seralutinib group. The least squares mean change from baseline to week 24 in PVR was 21·2 dyne·s/cm5 (95% CI −37·4 to 79·8) for the placebo group and −74·9 dyne·s/cm5 (−139·7 to −10·2) for the seralutinib group. The least squares mean difference between the seralutinib and placebo groups for change in PVR was −96·1 dyne·s/cm5 (95% CI −183·5 to −8·8; p=0·03). The most common treatment-emergent adverse event in both treatment groups was cough: 16 (38%) of 42 patients in the placebo group; 19 (43%) of 44 patients in the seralutinib group. Treatment with inhaled seralutinib significantly decreased PVR, meeting the primary endpoint of the study among patients receiving background therapy for PAH. Gossamer Bio. [ABSTRACT FROM AUTHOR]

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.

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

Copyright of Revista Española de Cardiología (18855857) is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

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].

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.

Copyright of Revista Española de Cardiología (18855857) is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

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.

Copyright of Revista Española de Cardiología (18855857) is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)