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2. Congenital heart disease in the ESC EORP Registry of Pregnancy and Cardiac disease (ROPAC)

Author

Hall, Roger, Roos-Hesselink, Jolien, Stein, Joerg, Parsonage, William Anthony, Budts, Werner, De Backer, Julie, Grewal, Jasmin, Marelli, Ariane, Kaemmerer, Harald, Jondeau, Guillaume, Johnson, Mark, Maggioni, Aldo P., Tavazzi, Luigi, Thilen, Ulf, Elkayam, Uri, Otto, Catherine, Sliwa, Karen, Aquieri, A., Saad, A., Vega, H. Ruda, Hojman, J., Caparros, J.M., Blanco, M. Vazquez, Arstall, M., Chung, C.M., Mahadavan, G., Aldridge, E., Wittwer, M., Chow, Y.Y., Parsonage, W.A., Lust, K., Collins, N., Warner, G., Hatton, R., Gordon, A., Nyman, E., Stein, J., Donhauser, E., Gabriel, H., Bahshaliyev, A., Guliyev, F., Hasanova, I., Jahangirov, T., Gasimov, Z., Salim, A., Ahmed, C.M., Begum, F., Hoque, M.H., Mahmood, M., Islam, M.N., Haque, P.P., Banerjee, S.K., Parveen, T., Morissens, M., De Backer, J., Demulier, L., de Hosson, M., Budts, W., Beckx, M., Kozic, M., Lovric, M., Kovacevic-Preradovic, T., Chilingirova, N., Kratunkov, P., Wahab, N., McLean, S., Gordon, E., Walter, L., Marelli, A., Montesclaros, A.R., Monsalve, G., Rodriguez, C., Balthazar, F., Quintero, V., Palacio, W., Cadavid, L.A. Mejía, Ortiz, E. Munoz, Hoyos, F. Fortich, Guerrero, E. Arevalo, Ricardo, J. Gandara, Penagos, J. Velasquez, Vavera, Z., Prague, Popelova, J., Vejlstrup, N., Grønbeck, L., Johansen, M., Ersboll, A., Elrakshy, Y., Eltamawy, K., Abd-El Aziz, M. Gamal, El Nagar, A., Ebaid, H., Elenin, H. Abo, Saed, M., Farag, S., Makled, W., Sorour, K., Ashour, Z., El-Sayed, G., Meguid Mahdy, M. Abdel, Taha, N., Dardeer, A., Shabaan, M., Ali, M., Moceri, P., Duthoit, G., Gouton, M., Nizard, J., Baris, L., Cohen, S., Ladouceur, M., Khimoud, D., Iung, B., Berger, F., Olsson, A., Gembruch, U., Merz, W.M., Reinert, E., Clade, S., Kliesch, Y., Wald, C., Sinning, C., Kozlik-Feldmann, R., Blankenberg, S., Zengin-Sahm, E., Mueller, G., Hillebrand, M., Hauck, P., von Kodolitsch, Y., Zarniko, N., Baumgartner, Muenster H., Schmidt, R., Hellige, A., Tutarel, O., Kaemmerer, H., Kuschel, B., Nagdyman, N., Motz, R., Maisuradze, D., Frogoudaki, A., Iliodromitis, E., Anastasiou-Nana, M., Marousi, Triantafyllis, D., Bekiaris, G., Karvounis, H., Giannakoulas, G., Ntiloudi, D., Mouratoglou, S.A., Temesvari, A., Balint, H., Kohalmi, D., Merkely, B., Liptai, C., Nemes, A., Forster, T., Kalapos, A., Berek, K., Havasi, K., Ambrus, N., Shelke, A., Kawade, R., Patil, S., Martanto, E., Aprami, T.M., Purnomowati, A., Cool, C.J., Hasan, M., Akbar, R., Hidayat, S., Dewi, T.I., Permadi, W., Soedarsono, D.A., Ansari-Ramandi, M.M., Samiei, N., Tabib, A., Kashfi, F., Ansari-Ramandi, S., Rezaei, S., Farhan, H. Ali, Al-Hussein, A., Al-Saedi, G., Mahmood, G., Yaseen, I.F., Al-Yousuf, L., AlBayati, M., Mahmood, S., Raheem, S., AlHaidari, T., Dakhil, Z., Thornton, P., Donnelly, J., Bowen, M., Blatt, A., Elbaz-Greener, G., Shotan, A., Yalonetsky, S., Goland, S., Biener, M., Assenza, G. Egidy, Bonvicini, M., Donti, A., Bulgarelli, A., Prandstraller, D., Romeo, C., Crepaz, R., Sciatti, E., Metra, M., Orabona, R., Ali, L. Ait, Festa, P., Fesslova, V., Bonanomi, C., Calcagnino, M., Lombardi, F., Colli, A.M., Ossola, M.W., Gobbi, C., Gherbesi, E., Tondi, L., Schiavone, M., Squillace, M., Carmina, M.G., Maina, A., Macchi, C., Gollo, E., Comoglio, F.M., Montali, N., Re, P., Bordese, R., Todros, T., Donvito, V., Marra, W. Grosso, Sinagra, G., D'Agata Mottolese, B., Bobbo, M., Gesuete, V., Rakar, S., Ramani, F., Niwa, K., Mekebekova, D., Mussagaliyeva, A., Lee, T., Mirrakhimov, E., Abilova, S., Bektasheva, E., Neronova, K., Lunegova, O., Žaliūnas, R., Jonkaitienė, R., Petrauskaitė, J., Laucevicius, A., Jancauskaite, D., Lauciuviene, L., Gumbiene, L., Lankutiene, L., Glaveckaite, S., Laukyte, M., Solovjova, S., Rudiene, V., Chee, K.H., Yim, C.C.-W., Ang, H.L., Kuppusamy, R., Watson, T., Caruana, M., Estensen, M.-E., Kayani, M.G.A. Mahmood, Munir, R., Tomaszuk-Kazberuk, A., Sobkowicz, B., Przepiesc, J., Lesniak-Sobelga, A., Tomkiewicz-Pajak, L., Komar, M., Olszowska, M., Podolec, P., Wisniowska-Smialek, S., Lelonek, M., Faflik, U., Cichocka-Radwan, A., Plaskota, K., Trojnarska, O., Guerra, N., de Sousa, L., Cruz, C., Ribeiro, V., Jovanova, S., Petrescu, V., Jurcut, R., Ginghina, C., Coman, I. Mircea, Musteata, M., Osipova, O., Golivets, T., Khamnagadaev, I., Golovchenko, O., Nagibina, A., Ropatko, I., Gaisin, I.R., Shilina, L. Valeryevna, Sharashkina, N., Shlyakhto, E., Irtyuga, O., Moiseeva, O., Karelkina, E., Zazerskaya, I., Kozlenok, A., Sukhova, I., Jovovic, L., Prokšelj, K., Koželj, M., Askar, A.O., Abdilaahi, A.A., Mohamed, M.H., Dirir, A.M., Sliwa, K., Manga, P., Pijuan-Domenech, A., Galian-Gay, L., Tornos, P., Subirana, M.T., T, M., Subirana, Oliver, J.M., Garcia-Aranda Dominguez, B., Gonzalez, I. Hernandez, Jimenez, J.F. Delgado, Subias, P. Escribano, Murga, N., Elbushi, A., Suliman, A., Jazzar, K., Murtada, M., Ahamed, N., Dellborg, M., Furenas, E., Jinesjo, M., Skoglund, K., Eriksson, P., Gilljam, T., Thilen, U., Tobler, D., Wustmann, K., Schwitz, F., Schwerzmann, M., Rutz, T., Bouchardy, J., Greutmann, M., Lopes, B.M. Santos, Meier, L., Arrigo, M., de Boer, K., Konings, T., Wajon, E., Wagenaar, L.J., Polak, P., Pieper, E.P.G., Roos-Hesselink, J., van Hagen, I., Duvekot, H., Cornette, J.M.J., De Groot, C., van Oppen, C., Sarac, L., Esen, O. Batukan, Enar, S. Catirli, Mondo, C., Ingabire, P., Nalwanga, B., Semu, T., Salih, B.T., Almahmeed, W.A.R., Wani, S., Farook, F.S. Mohamed, Ain, Al, Gerges, F., Komaranchath, A.M., Al bakshi, F., Al Mulla, A., Yusufali, A.H., Al Hatou, E.I., Bazargani, N., Hussain, F., Hudsmith, L., Thompson, P., Thorne, S., Bowater, S., Money-Kyrle, A., Clifford, P., Ramrakha, P., Firoozan, S., Chaplin, J., Bowers, N., Adamson, D., Schroeder, F., Wendler, R., Hammond, S., Nihoyannopoulos, P., Norfolk, Norwich, Hall, R., Freeman, L., Veldtman, G., Kerr, J., Tellett, L., Scott, N., Bhatt, A.B., DeFaria Yeh, D., Youniss, M.A., Wood, M., Sarma, A.A., Tsiaras, S., Stefanescu, A., Duran, J.M., Stone, L., Majdalany, D.S., Chapa, J., Chintala, K., Gupta, P., Botti, J., Ting, J., Davidson, W.R., Wells, G., Sparks, D., Paruchuri, V., Marzo, K., Patel, D., Wagner, W., Ahanya, S.N., Colicchia, L., Jentink, T., Han, K., Loichinger, M., Parker, M., Longtin, C., Yetman, A., Erickson, K., Cramer, J., Tsai, S., Fletcher, B., Warta, S., Cohen, C., Lindblade, C., Puntel, R., Nagaran, K., Croft, N., Gurvitz, M., Otto, C., Talluto, C., Murphy, D., Perlroth, M.G., Ramlakhan, Karishma P., Johnson, Mark R., Lelonek, Malgorzata, Saad, Aly, Gasimov, Zaur, Sharashkina, Natalia V., Thornton, Patrick, Arstall, Margaret, and Roos-Hesselink, Jolien W.

Published
2021
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4. Effect of Migalastat on cArdiac Involvement in FabRry Disease: MAIORA study

Author

Camporeale, A, Bandera, F, Pieroni, M, Pieruzzi, F, Spada, M, Bersano, A, Econimo, L, Lanzillo, C, Rubino, M, Mignani, R, Motta, I, Olivotto, I, Tanini, I, Valaperta, R, Chow, K, Baroni, I, Boveri, S, Graziani, F, Pica, S, Tondi, L, Guazzi, M, Lombardi, M, Camporeale A., Bandera F., Pieroni M., Pieruzzi F., Spada M., Bersano A., Econimo L., Lanzillo C., Rubino M., Mignani R., Motta I., Olivotto I., Tanini I., Valaperta R., Chow K., Baroni I., Boveri S., Graziani F., Pica S., Tondi L., Guazzi M., Lombardi M., Camporeale, A, Bandera, F, Pieroni, M, Pieruzzi, F, Spada, M, Bersano, A, Econimo, L, Lanzillo, C, Rubino, M, Mignani, R, Motta, I, Olivotto, I, Tanini, I, Valaperta, R, Chow, K, Baroni, I, Boveri, S, Graziani, F, Pica, S, Tondi, L, Guazzi, M, Lombardi, M, Camporeale A., Bandera F., Pieroni M., Pieruzzi F., Spada M., Bersano A., Econimo L., Lanzillo C., Rubino M., Mignani R., Motta I., Olivotto I., Tanini I., Valaperta R., Chow K., Baroni I., Boveri S., Graziani F., Pica S., Tondi L., Guazzi M., and Lombardi M.

Abstract

Background: A small but significant reduction in left ventricular (LV) mass after 18 months of migalastat treatment has been reported in Fabry disease (FD). This study aimed to assess the effect of migalastat on FD cardiac involvement, combining LV morphology and tissue characterisation by cardiac magnetic resonance (CMR) with cardiopulmonary exercise testing (CPET). Methods: Sixteen treatment-naïve patients with FD (4 women, 46.4±16.2 years) with cardiac involvement (reduced T1 values on CMR and/or LV hypertrophy) underwent ECG, echocardiogram, troponin T and NT-proBNP (N-Terminal prohormone of Brain Natriuretic Peptide) assay, CMR with T1 mapping, and CPET before and after 18 months of migalastat. Results: No change in LV mass was detected at 18 months compared to baseline (95.2 g/m2 (66.0-184.0) vs 99.0 g/m2 (69.0-121.0), p=0.55). Overall, there was an increase in septal T1 of borderline significance (870.0 ms (848-882) vs 860.0 ms (833.0-875.0), p=0.056). Functional capacity showed an increase in oxygen consumption (VO2) at anaerobic threshold (15.50 mL/kg/min (13.70-21.50) vs 14.50 mL/kg/min (11.70-18.95), p=0.02), and a trend towards an increase in percent predicted peak VO2 (72.0 (63.0-80.0) vs 69.0 (53.0-77.0), p=0.056) was observed. The subset of patients who showed an increase in T1 value and a reduction in LV mass (n=7, 1 female, age 40.5 (28.6-76.0)) was younger and at an earlier disease stage compared to the others, and also exhibited greater improvement in exercise tolerance. Conclusion: In treatment-naïve FD patients with cardiac involvement, 18-month treatment with migalastat stabilised LV mass and was associated with a trend towards an improvement in exercise tolerance. A tendency to T1 increase was detected by CMR. The subset of patients who had significant benefits from the treatment showed an earlier cardiac disease compared to the others. Trial registration number: NCT03838237.

Published
2023

5. Effect of Migalastat on cArdiac Involvement in FabRry Disease: MAIORA study

Author

Camporeale, Antonia, Bandera, F., Pieroni, M., Pieruzzi, F., Spada, Marina, Bersano, A., Econimo, L., Lanzillo, C., Rubino, M., Mignani, R., Motta, I., Olivotto, I., Tanini, I., Valaperta, R., Chow, K., Baroni, I., Boveri, S., Graziani, Francesca, Pica, S., Tondi, L., Guazzi, M., Lombardi, M., Camporeale A., Spada M., Graziani F. (ORCID:0000-0002-4520-5689), Camporeale, Antonia, Bandera, F., Pieroni, M., Pieruzzi, F., Spada, Marina, Bersano, A., Econimo, L., Lanzillo, C., Rubino, M., Mignani, R., Motta, I., Olivotto, I., Tanini, I., Valaperta, R., Chow, K., Baroni, I., Boveri, S., Graziani, Francesca, Pica, S., Tondi, L., Guazzi, M., Lombardi, M., Camporeale A., Spada M., and Graziani F. (ORCID:0000-0002-4520-5689)

Abstract

Background: A small but significant reduction in left ventricular (LV) mass after 18 months of migalastat treatment has been reported in Fabry disease (FD). This study aimed to assess the effect of migalastat on FD cardiac involvement, combining LV morphology and tissue characterisation by cardiac magnetic resonance (CMR) with cardiopulmonary exercise testing (CPET). Methods: Sixteen treatment-naïve patients with FD (4 women, 46.4±16.2 years) with cardiac involvement (reduced T1 values on CMR and/or LV hypertrophy) underwent ECG, echocardiogram, troponin T and NT-proBNP (N-Terminal prohormone of Brain Natriuretic Peptide) assay, CMR with T1 mapping, and CPET before and after 18 months of migalastat. Results: No change in LV mass was detected at 18 months compared to baseline (95.2 g/m2 (66.0-184.0) vs 99.0 g/m2 (69.0-121.0), p=0.55). Overall, there was an increase in septal T1 of borderline significance (870.0 ms (848-882) vs 860.0 ms (833.0-875.0), p=0.056). Functional capacity showed an increase in oxygen consumption (VO2) at anaerobic threshold (15.50 mL/kg/min (13.70-21.50) vs 14.50 mL/kg/min (11.70-18.95), p=0.02), and a trend towards an increase in percent predicted peak VO2 (72.0 (63.0-80.0) vs 69.0 (53.0-77.0), p=0.056) was observed. The subset of patients who showed an increase in T1 value and a reduction in LV mass (n=7, 1 female, age 40.5 (28.6-76.0)) was younger and at an earlier disease stage compared to the others, and also exhibited greater improvement in exercise tolerance. Conclusion: In treatment-naïve FD patients with cardiac involvement, 18-month treatment with migalastat stabilised LV mass and was associated with a trend towards an improvement in exercise tolerance. A tendency to T1 increase was detected by CMR. The subset of patients who had significant benefits from the treatment showed an earlier cardiac disease compared to the others. Trial registration number: NCT03838237.

Published
2023

6. Myocardial Fibrosis at Cardiac MRI Helps Predict Adverse Clinical Outcome in Patients with Mitral Valve Prolapse

Author

Figliozzi, S, Georgiopoulos, G, Lopes, P, Bauer, K, Moura-Ferreira, S, Tondi, L, Mushtaq, S, Censi, S, Pavon, A, Bassi, I, Servato, M, Teske, A, Biondi, F, Filomena, D, Pica, S, Torlasco, C, Muraru, D, Monney, P, Quattrocchi, G, Maestrini, V, Agati, L, Monti, L, Pedrotti, P, Vandenberk, B, Squeri, A, Lombardi, M, Ferreira, A, Schwitter, J, Aquaro, G, Chiribiri, A, Rodríguez Palomares, J, Yilmaz, A, Andreini, D, Florian, A, Leiner, T, Abecasis, J, Badano, L, Bogaert, J, Masci, P, Figliozzi, Stefano, Georgiopoulos, Georgios, Lopes, Pedro M, Bauer, Klemens B, Moura-Ferreira, Sara, Tondi, Lara, Mushtaq, Saima, Censi, Stefano, Pavon, Anna Giulia, Bassi, Ilaria, Servato, Maria Luz, Teske, Arco J, Biondi, Federico, Filomena, Domenico, Pica, Silvia, Torlasco, Camilla, Muraru, Denisa, Monney, Pierre, Quattrocchi, Giuseppina, Maestrini, Viviana, Agati, Luciano, Monti, Lorenzo, Pedrotti, Patrizia, Vandenberk, Bert, Squeri, Angelo, Lombardi, Massimo, Ferreira, António M, Schwitter, Juerg, Aquaro, Giovanni Donato, Chiribiri, Amedeo, Rodríguez Palomares, José F, Yilmaz, Ali, Andreini, Daniele, Florian, Anca, Leiner, Tim, Abecasis, João, Badano, Luigi, Bogaert, Jan, Masci, Pier-Giorgio, Figliozzi, S, Georgiopoulos, G, Lopes, P, Bauer, K, Moura-Ferreira, S, Tondi, L, Mushtaq, S, Censi, S, Pavon, A, Bassi, I, Servato, M, Teske, A, Biondi, F, Filomena, D, Pica, S, Torlasco, C, Muraru, D, Monney, P, Quattrocchi, G, Maestrini, V, Agati, L, Monti, L, Pedrotti, P, Vandenberk, B, Squeri, A, Lombardi, M, Ferreira, A, Schwitter, J, Aquaro, G, Chiribiri, A, Rodríguez Palomares, J, Yilmaz, A, Andreini, D, Florian, A, Leiner, T, Abecasis, J, Badano, L, Bogaert, J, Masci, P, Figliozzi, Stefano, Georgiopoulos, Georgios, Lopes, Pedro M, Bauer, Klemens B, Moura-Ferreira, Sara, Tondi, Lara, Mushtaq, Saima, Censi, Stefano, Pavon, Anna Giulia, Bassi, Ilaria, Servato, Maria Luz, Teske, Arco J, Biondi, Federico, Filomena, Domenico, Pica, Silvia, Torlasco, Camilla, Muraru, Denisa, Monney, Pierre, Quattrocchi, Giuseppina, Maestrini, Viviana, Agati, Luciano, Monti, Lorenzo, Pedrotti, Patrizia, Vandenberk, Bert, Squeri, Angelo, Lombardi, Massimo, Ferreira, António M, Schwitter, Juerg, Aquaro, Giovanni Donato, Chiribiri, Amedeo, Rodríguez Palomares, José F, Yilmaz, Ali, Andreini, Daniele, Florian, Anca, Leiner, Tim, Abecasis, João, Badano, Luigi, Bogaert, Jan, and Masci, Pier-Giorgio

Abstract

Background: Patients with mitral valve prolapse (MVP) may develop adverse outcomes even in the absence of mitral regurgitation or left ventricular (LV) dysfunction. Purpose: To investigate the prognostic value of mitral annulus disjunction (MAD) and myocardial fibrosis at late gadolinium enhancement (LGE) cardiac MRI in patients with MVP without moderate-to-severe mitral regurgitation or LV dysfunction. Materials and Methods: In this longitudinal retrospective study, 118 144 cardiac MRI studies were evaluated between October 2007 and June 2020 at 15 European tertiary medical centers. Follow-up was from the date of cardiac MRI examination to June 2020; the minimum and maximum follow-up intervals were 6 months and 156 months, respectively. Patients were excluded if at least one of the following conditions was present: cardiomyopathy, LV ejection fraction less than 40%, ischemic heart disease, congenital heart disease, inflammatory heart disease, moderate or worse mitral regurgitation, participation in competitive sport, or electrocardiogram suggestive of channelopathies. In the remainder, cardiac MRI studies were reanalyzed, and patients were included if they were aged 18 years or older, MVP was diagnosed at cardiac MRI, and clinical information and electrocardiogram monitoring were available within 3 months from cardiac MRI examination. The end point was a composite of adverse outcomes: sustained ventricular tachycardia (VT), sudden cardiac death (SCD), or unexplained syncope. Multivariable Cox regression analysis was performed. Results: A total of 474 patients (mean age, 47 years ± 16 [SD]; 244 women) were included. Over a median follow-up of 3.3 years, 18 patients (4%) reached the study end point. LGE presence (hazard ratio, 4.2 [95% CI: 1.5, 11.9]; P = .006) and extent (hazard ratio, 1.2 per 1% increase [95% CI: 1.1, 1.4]; P = .006), but not MAD presence (P = .89), were associated with clinical outcome. LGE presence had incremental prognostic value over MVP severi

Published
2023

8. ECG-based score estimates the probability to detect Fabry Disease cardiac involvement

Author

Figliozzi, S, Camporeale, A, Boveri, S, Pieruzzi, F, Pieroni, M, Lusardi, P, Spada, M, Mignani, R, Burlina, A, Graziani, F, Pica, S, Tondi, L, Bernardini, A, Chow, K, Namdar, M, Lombardi, M, Figliozzi S., Camporeale A., Boveri S., Pieruzzi F., Pieroni M., Lusardi P., Spada M., Mignani R., Burlina A., Graziani F., Pica S., Tondi L., Bernardini A., Chow K., Namdar M., Lombardi M., Figliozzi, S, Camporeale, A, Boveri, S, Pieruzzi, F, Pieroni, M, Lusardi, P, Spada, M, Mignani, R, Burlina, A, Graziani, F, Pica, S, Tondi, L, Bernardini, A, Chow, K, Namdar, M, Lombardi, M, Figliozzi S., Camporeale A., Boveri S., Pieruzzi F., Pieroni M., Lusardi P., Spada M., Mignani R., Burlina A., Graziani F., Pica S., Tondi L., Bernardini A., Chow K., Namdar M., and Lombardi M.

Abstract

Objectives: To elaborate an ECG-based nomogram estimating the probability to detect cardiac involvement by cardiac magnetic resonance (CMR) in Fabry Disease (FD). Methods: 119 FD patients and 26 healthy controls underwent ECG and CMR. Test (n = 88, 60%) and validation cohorts (n = 57, 40%) were randomly derived. Cardiac involvement was defined as the presence of low myocardial T1 value, a CMR-surrogate of myocardial glycosphingolipid storage. ECG changes associated with low T1 value were identified in the test cohort, included in the nomogram and then tested in the validation cohort. Results: Sokolow-Lyon index (AUC = 0.769), ratio between P-wave and PR-segment durations (Pwave/PRsegment) (AUC = 0.778), QRS duration (AUC = 0.703), QT (AUC = 0.769) duration were independently associated with the presence of low T1 on CMR at multivariate analysis. An ECG-based nomogram including these four parameters was accurate in identifying patients with CMR evidence of glycosphingolipid storage (c-index of the derived-nomogram = 0.90 in the test group; 0.81 in the validation group). Conclusion: We propose a practical ECG-based nomogram accurately estimating the probability to detect low T1 values by CMR in FD patients. The application of this tool in clinical practice could improve early detection of FD cardiac involvement.

Published
2021

10. Atrial Dysfunction Assessed by Cardiac Magnetic Resonance as an Early Marker of Fabry Cardiomyopathy

Author

Bernardini, A, Camporeale, A, Pieroni, M, Pieruzzi, F, Figliozzi, S, Lusardi, P, Spada, M, Mignani, R, Burlina, A, Carubbi, F, Battaglia, Y, Graziani, F, Pica, S, Tondi, L, Chow, K, Boveri, S, Olivotto, I, Lombardi, M, Bernardini A., Camporeale A., Pieroni M., Pieruzzi F., Figliozzi S., Lusardi P., Spada M., Mignani R., Burlina A., Carubbi F., Battaglia Y., Graziani F., Pica S., Tondi L., Chow K., Boveri S., Olivotto I., Lombardi M., Bernardini, A, Camporeale, A, Pieroni, M, Pieruzzi, F, Figliozzi, S, Lusardi, P, Spada, M, Mignani, R, Burlina, A, Carubbi, F, Battaglia, Y, Graziani, F, Pica, S, Tondi, L, Chow, K, Boveri, S, Olivotto, I, Lombardi, M, Bernardini A., Camporeale A., Pieroni M., Pieruzzi F., Figliozzi S., Lusardi P., Spada M., Mignani R., Burlina A., Carubbi F., Battaglia Y., Graziani F., Pica S., Tondi L., Chow K., Boveri S., Olivotto I., and Lombardi M.

Published
2020

11. Cardiac Magnetic Resonance Features of Fabry Disease: From Early Diagnosis to Prognostic Stratification

Author

Camporeale, Antonia, Diano, A., Tondi, L., Pica, S., Pasqualin, G., Ciabatti, M., Graziani, Francesca, Pieroni, M., Lombardi, M., Camporeale A., Graziani F. (ORCID:0000-0002-4520-5689), Camporeale, Antonia, Diano, A., Tondi, L., Pica, S., Pasqualin, G., Ciabatti, M., Graziani, Francesca, Pieroni, M., Lombardi, M., Camporeale A., and Graziani F. (ORCID:0000-0002-4520-5689)

Abstract

In the past few years, the wide application of cardiac magnetic resonance (CMR) significantly changed the approach to the study of cardiac involvement in Fabry Disease (FD). The possibility to perform non-invasive tissue characterization, including new sequences such as T1/T2 mapping, offered a powerful tool for differential diagnosis with other forms of left ventricular hypertrophy. In patients with confirmed diagnosis of FD, CMR is the most sensitive non-invasive technique for early detection of cardiac involvement and it provides new insight into the evolution of cardiac damage, including gender-specific features. Finally, CMR multiparametric detection of subtle changes in cardiac morphology, function and tissue composition is potentially useful for monitoring the efficacy of specific treatment over time. This paper aims to provide a comprehensive review of current knowledge regarding the application of CMR in FD cardiac involvement and its clinical implication.

Published
2022

16. Predictors of adverse prognosis in COVID-19: A systematic review and meta-analysis

Author

Figliozzi, S. Masci, P.G. Ahmadi, N. Tondi, L. Koutli, E. Aimo, A. Stamatelopoulos, K. Dimopoulos, M.-A. Caforio, A.L.P. Georgiopoulos, G.

Abstract

Background: Identification of reliable outcome predictors in coronavirus disease 2019 (COVID-19) is of paramount importance for improving patient's management. Methods: A systematic review of literature was conducted until 24 April 2020. From 6843 articles, 49 studies were selected for a pooled assessment; cumulative statistics for age and sex were retrieved in 587 790 and 602 234 cases. Two endpoints were defined: (a) a composite outcome including death, severe presentation, hospitalization in the intensive care unit (ICU) and/or mechanical ventilation; and (b) in-hospital mortality. We extracted numeric data on patients’ characteristics and cases with adverse outcomes and employed inverse variance random-effects models to derive pooled estimates. Results: We identified 18 and 12 factors associated with the composite endpoint and death, respectively. Among those, a history of CVD (odds ratio (OR) = 3.15, 95% confidence intervals (CIs) 2.26-4.41), acute cardiac (OR = 10.58, 5.00-22.40) or kidney (OR = 5.13, 1.78-14.83) injury, increased procalcitonin (OR = 4.8, 2.034-11.31) or D-dimer (OR = 3.7, 1.74-7.89), and thrombocytopenia (OR = 6.23, 1.031-37.67) conveyed the highest odds for the adverse composite endpoint. Advanced age, male sex, cardiovascular comorbidities, acute cardiac or kidney injury, lymphocytopenia and D-dimer conferred an increased risk of in-hospital death. With respect to the treatment of the acute phase, therapy with steroids was associated with the adverse composite endpoint (OR = 3.61, 95% CI 1.934-6.73), but not with mortality. Conclusions: Advanced age, comorbidities, abnormal inflammatory and organ injury circulating biomarkers captured patients with an adverse clinical outcome. Clinical history and laboratory profile may then help identify patients with a higher risk of in-hospital mortality. © 2020 Stichting European Society for Clinical Investigation Journal Foundation

Published
2020

38. Congenital heart disease in the ESC EORP Registry of Pregnancy and Cardiac disease (ROPAC)

Author

Ramlakhan, Karishma P., Johnson, Mark R., Lelonek, Malgorzata, Saad, Aly, Gasimov, Zaur, Sharashkina, Natalia V., Thornton, Patrick, Arstall, Margaret, Hall, Roger, Roos-Hesselink, Jolien W., Hall, Roger, Roos-Hesselink, Jolien, Stein, Joerg, Parsonage, William Anthony, Budts, Werner, De Backer, Julie, Grewal, Jasmin, Marelli, Ariane, Kaemmerer, Harald, Jondeau, Guillaume, Johnson, Mark, Maggioni, Aldo P., Tavazzi, Luigi, Thilen, Ulf, Elkayam, Uri, Otto, Catherine, Sliwa, Karen, Aquieri, A., Saad, A., Vega, H. Ruda, Hojman, J., Caparros, J.M., Blanco, M. Vazquez, Arstall, M., Chung, C.M., Mahadavan, G., Aldridge, E., Wittwer, M., Chow, Y.Y., Parsonage, W.A., Lust, K., Collins, N., Warner, G., Hatton, R., Gordon, A., Nyman, E., Stein, J., Donhauser, E., Gabriel, H., Bahshaliyev, A., Guliyev, F., Hasanova, I., Jahangirov, T., Gasimov, Z., Salim, A., Ahmed, C.M., Begum, F., Hoque, M.H., Mahmood, M., Islam, M.N., Haque, P.P., Banerjee, S.K., Parveen, T., Morissens, M., De Backer, J., Demulier, L., de Hosson, M., Budts, W., Beckx, M., Kozic, M., Lovric, M., Kovacevic-Preradovic, T., Chilingirova, N., Kratunkov, P., Wahab, N., McLean, S., Gordon, E., Walter, L., Marelli, A., Montesclaros, A.R., Monsalve, G., Rodriguez, C., Balthazar, F., Quintero, V., Palacio, W., Cadavid, L.A. Mejía, Ortiz, E. Munoz, Hoyos, F. Fortich, Guerrero, E. Arevalo, Ricardo, J. Gandara, Penagos, J. Velasquez, Vavera, Z., Prague, Popelova, J., Vejlstrup, N., Grønbeck, L., Johansen, M., Ersboll, A., Elrakshy, Y., Eltamawy, K., Abd-El Aziz, M. Gamal, El Nagar, A., Ebaid, H., Elenin, H. Abo, Saed, M., Farag, S., Makled, W., Sorour, K., Ashour, Z., El-Sayed, G., Meguid Mahdy, M. Abdel, Taha, N., Dardeer, A., Shabaan, M., Saad, A., Ali, M., Moceri, P., Duthoit, G., Gouton, M., Nizard, J., Baris, L., Cohen, S., Ladouceur, M., Khimoud, D., Iung, B., Berger, F., Olsson, A., Gembruch, U., Merz, W.M., Reinert, E., Clade, S., Kliesch, Y., Wald, C., Sinning, C., Kozlik-Feldmann, R., Blankenberg, S., Zengin-Sahm, E., Mueller, G., Hillebrand, M., Hauck, P., von Kodolitsch, Y., Zarniko, N., Baumgartner, Muenster H., Schmidt, R., Hellige, A., Tutarel, O., Kaemmerer, H., Kuschel, B., Nagdyman, N., Motz, R., Maisuradze, D., Frogoudaki, A., Iliodromitis, E., Anastasiou-Nana, M., Marousi, Triantafyllis, D., Bekiaris, G., Karvounis, H., Giannakoulas, G., Ntiloudi, D., Mouratoglou, S.A., Temesvari, A., Balint, H., Kohalmi, D., Merkely, B., Liptai, C., Nemes, A., Forster, T., Kalapos, A., Berek, K., Havasi, K., Ambrus, N., Shelke, A., Kawade, R., Patil, S., Martanto, E., Aprami, T.M., Purnomowati, A., Cool, C.J., Hasan, M., Akbar, R., Hidayat, S., Dewi, T.I., Permadi, W., Soedarsono, D.A., Ansari-Ramandi, M.M., Samiei, N., Tabib, A., Kashfi, F., Ansari-Ramandi, S., Rezaei, S., Farhan, H. Ali, Al-Hussein, A., Al-Saedi, G., Mahmood, G., Yaseen, I.F., Al-Yousuf, L., AlBayati, M., Mahmood, S., Raheem, S., AlHaidari, T., Dakhil, Z., Thornton, P., Donnelly, J., Bowen, M., Blatt, A., Elbaz-Greener, G., Shotan, A., Yalonetsky, S., Goland, S., Biener, M., Assenza, G. Egidy, Bonvicini, M., Donti, A., Bulgarelli, A., Prandstraller, D., Romeo, C., Crepaz, R., Sciatti, E., Metra, M., Orabona, R., Ali, L. Ait, Festa, P., Fesslova, V., Bonanomi, C., Calcagnino, M., Lombardi, F., Colli, A.M., Ossola, M.W., Gobbi, C., Gherbesi, E., Tondi, L., Schiavone, M., Squillace, M., Carmina, M.G., Maina, A., Macchi, C., Gollo, E., Comoglio, F.M., Montali, N., Re, P., Bordese, R., Todros, T., Donvito, V., Marra, W. Grosso, Sinagra, G., D'Agata Mottolese, B., Bobbo, M., Gesuete, V., Rakar, S., Ramani, F., Niwa, K., Mekebekova, D., Mussagaliyeva, A., Lee, T., Mirrakhimov, E., Abilova, S., Bektasheva, E., Neronova, K., Lunegova, O., Žaliūnas, R., Jonkaitienė, R., Petrauskaitė, J., Laucevicius, A., Jancauskaite, D., Lauciuviene, L., Gumbiene, L., Lankutiene, L., Glaveckaite, S., Laukyte, M., Solovjova, S., Rudiene, V., Chee, K.H., Yim, C.C.-W., Ang, H.L., Kuppusamy, R., Watson, T., Caruana, M., Estensen, M.-E., Kayani, M.G.A. Mahmood, Munir, R., Tomaszuk-Kazberuk, A., Sobkowicz, B., Przepiesc, J., Lesniak-Sobelga, A., Tomkiewicz-Pajak, L., Komar, M., Olszowska, M., Podolec, P., Wisniowska-Smialek, S., Lelonek, M., Faflik, U., Cichocka-Radwan, A., Plaskota, K., Trojnarska, O., Guerra, N., de Sousa, L., Cruz, C., Ribeiro, V., Jovanova, S., Petrescu, V., Jurcut, R., Ginghina, C., Coman, I. Mircea, Musteata, M., Osipova, O., Golivets, T., Khamnagadaev, I., Golovchenko, O., Nagibina, A., Ropatko, I., Gaisin, I.R., Shilina, L. Valeryevna, Sharashkina, N., Shlyakhto, E., Irtyuga, O., Moiseeva, O., Karelkina, E., Zazerskaya, I., Kozlenok, A., Sukhova, I., Jovovic, L., Prokšelj, K., Koželj, M., Askar, A.O., Abdilaahi, A.A., Mohamed, M.H., Dirir, A.M., Sliwa, K., Manga, P., Pijuan-Domenech, A., Galian-Gay, L., Tornos, P., Subirana, M.T., T, M., Subirana, Oliver, J.M., Garcia-Aranda Dominguez, B., Gonzalez, I. Hernandez, Jimenez, J.F. Delgado, Subias, P. Escribano, Murga, N., Elbushi, A., Suliman, A., Jazzar, K., Murtada, M., Ahamed, N., Dellborg, M., Furenas, E., Jinesjo, M., Skoglund, K., Eriksson, P., Gilljam, T., Thilen, U., Tobler, D., Wustmann, K., Schwitz, F., Schwerzmann, M., Rutz, T., Bouchardy, J., Greutmann, M., Lopes, B.M. Santos, Meier, L., Arrigo, M., de Boer, K., Konings, T., Wajon, E., Wagenaar, L.J., Polak, P., Pieper, E.P.G., Roos-Hesselink, J., Baris, L., van Hagen, I., Duvekot, H., Cornette, J.M.J., De Groot, C., van Oppen, C., Sarac, L., Esen, O. Batukan, Enar, S. Catirli, Mondo, C., Ingabire, P., Nalwanga, B., Semu, T., Salih, B.T., Almahmeed, W.A.R., Wani, S., Farook, F.S. Mohamed, Ain, Al, Gerges, F., Komaranchath, A.M., Al bakshi, F., Al Mulla, A., Yusufali, A.H., Al Hatou, E.I., Bazargani, N., Hussain, F., Hudsmith, L., Thompson, P., Thorne, S., Bowater, S., Money-Kyrle, A., Clifford, P., Ramrakha, P., Firoozan, S., Chaplin, J., Bowers, N., Adamson, D., Schroeder, F., Wendler, R., Hammond, S., Nihoyannopoulos, P., Norfolk, Norwich, Hall, R., Freeman, L., Veldtman, G., Kerr, J., Tellett, L., Scott, N., Bhatt, A.B., DeFaria Yeh, D., Youniss, M.A., Wood, M., Sarma, A.A., Tsiaras, S., Stefanescu, A., Duran, J.M., Stone, L., Majdalany, D.S., Chapa, J., Chintala, K., Gupta, P., Botti, J., Ting, J., Davidson, W.R., Wells, G., Sparks, D., Paruchuri, V., Marzo, K., Patel, D., Wagner, W., Ahanya, S.N., Colicchia, L., Jentink, T., Han, K., Loichinger, M., Parker, M., Wagner, W., Longtin, C., Yetman, A., Erickson, K., Cramer, J., Tsai, S., Fletcher, B., Warta, S., Cohen, C., Lindblade, C., Puntel, R., Nagaran, K., Croft, N., Gurvitz, M., Otto, C., Talluto, C., Murphy, D., and Perlroth, M.G.

Published
2021
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39. The causal relationship between crude oil price, exchange rate and rice price

Author

Pasrun Adam, Rosnawintang, Saidi, L. O., Tondi, L., and Sani, L. O. A.

Subjects
lcsh:GE1-350, food and beverages, lcsh:HD9502-9502.5, health care economics and organizations, lcsh:Environmental sciences, lcsh:Energy industries. Energy policy. Fuel trade
Abstract

This study aims to examine the causal relationship between crude oil price, IDR/EUR exchange rate, and rice price by using monthly data from January 2000 to September 2017. The result of data analysis using VAR model shows that there is no long-term relationship between crude oil price, IDR/EUR exchange rate, and the price of rice. The relationhip that happens is only in short-term one. Granger causality test result shows that the direction of relationship is from crude oil price and IDR/EUR exchange rate to rice price. The relationship between crude oil price and rice price is positive, while the relationship between IDR/EUR exchange rate and rice price is positive before the third month. However, this relationship turns into negative after the third month. Keywords: Crude oil price, exchange rate, rice price, VAR model.JEL Classifications: C58, G150, Q13, Q430

46. The use of dedicated long-axis views focused on the left atrium improves the accuracy of left atrial volumes and emptying fraction measured by cardiovascular magnetic resonance

Author

Lara Tondi, Luigi P. Badano, Stefano Figliozzi, Silvia Pica, Camilla Torlasco, Antonia Camporeale, Diana R. Florescu, Giandomenico Disabato, Gianfranco Parati, Massimo Lombardi, Denisa Muraru, Tondi, L, Badano, L, Figliozzi, S, Pica, S, Torlasco, C, Camporeale, A, Florescu, D, Disabato, G, Parati, G, Lombardi, M, and Muraru, D

Subjects
Left atrial emptying fraction, Radiological and Ultrasound Technology, Cardiac magnetic resonance, Left atrial strain, Radiology, Nuclear Medicine and imaging, Left atrial volume, Cardiology and Cardiovascular Medicine, Accuracy
Abstract

Background The use of apical views focused on the left atrium (LA) has improved the accuracy of LA volume evaluation by two-dimensional (2D) echocardiography. However, routine cardiovascular magnetic resonance (CMR) evaluation of LA volumes still uses standard 2- and 4-chamber cine images focused on the left ventricle (LV). To investigate the potential of LA-focused CMR cine images, we compared LA maximuml (LAVmax) and minimum (LAVmin) volumes, and emptying fraction (LAEF), calculated on both standard and LA-focused long-axis cine images, with LA volumes and LAEF obtained by short-axis cine stacks covering the LA. LA strain was also calculated and compared between standard and LA-focused images. Methods LA volumes and LAEF were obtained from 108 consecutive patients by applying the biplane area-length algorithm to both standard and LA-focused 2- and 4-chamber cine images. Manual segmentation of a short-axis cine stack covering the LA was used as the reference method. In addition, LA strain reservoir (εs), conduit (εe) and booster pump (εa) were calculated using CMR feature-tracking. Results Compared to the reference method, the standard approach significantly underestimated LA volumes (LAVmax: bias − 13 ml; LOA = + 11, − 37 ml; LAVmax i: bias − 7 ml/m2; LOA = + 7, − 21 ml/m2; LAVmin; bias − 10 ml, LOA: + 9, − 28 ml; LAVmin i: bias − 5 ml/m2, LOA: + 5, − 16 ml/m2), and overestimated LA-EF (bias 5%, LOA: + 23, − 14%). Conversely, LA volumes (LAVmax: bias 0 ml; LOA: + 10, − 10 ml; LAVmax i: bias 0 ml/m2; LOA: + 5, − 6 ml/m2; LAVmin: bias − 2 ml; LOA: + 7, − 10 ml; LAVmin i: bias − 1 ml/m2; LOA: + 3, − 5 ml/m2) and LAEF (bias 2%, LOA: + 11, − 7%) by LA-focused cine images were similar to those measured using the reference method. LA volumes by LA-focused images were obtained faster than using the reference method (1.2 vs 4.5 min, p Conclusion LA volumes and LAEF measured using dedicated LA-focused long-axis cine images are more accurate than using standard LV-focused cine images. Moreover, LA strain is significantly lower in LA-focused vs. standard images.

Published
2023

47. Effect of Migalastat on cArdiac Involvement in FabRry Disease: MAIORA study

Author

Antonia Camporeale, Francesco Bandera, Maurizio Pieroni, Federico Pieruzzi, Marco Spada, Anna Bersano, Laura Econimo, Chiara Lanzillo, Marta Rubino, Renzo Mignani, Irene Motta, Iacopo Olivotto, Ilaria Tanini, Rea Valaperta, Kelvin Chow, Irene Baroni, Sara Boveri, Francesca Graziani, Silvia Pica, Lara Tondi, Marco Guazzi, Massimo Lombardi, Camporeale, A, Bandera, F, Pieroni, M, Pieruzzi, F, Spada, M, Bersano, A, Econimo, L, Lanzillo, C, Rubino, M, Mignani, R, Motta, I, Olivotto, I, Tanini, I, Valaperta, R, Chow, K, Baroni, I, Boveri, S, Graziani, F, Pica, S, Tondi, L, Guazzi, M, and Lombardi, M

Subjects
Settore MED/11 - MALATTIE DELL'APPARATO CARDIOVASCOLARE, Genetics, Fabry Disease, Cardiac Magnetic Resonance, Migalastat, Genetics (clinical)
Abstract

BackgroundA small but significant reduction in left ventricular (LV) mass after 18 months of migalastat treatment has been reported in Fabry disease (FD). This study aimed to assess the effect of migalastat on FD cardiac involvement, combining LV morphology and tissue characterisation by cardiac magnetic resonance (CMR) with cardiopulmonary exercise testing (CPET).MethodsSixteen treatment-naïve patients with FD (4 women, 46.4±16.2 years) with cardiac involvement (reduced T1 values on CMR and/or LV hypertrophy) underwent ECG, echocardiogram, troponin T and NT-proBNP (N-Terminal prohormone of Brain Natriuretic Peptide) assay, CMR with T1 mapping, and CPET before and after 18 months of migalastat.ResultsNo change in LV mass was detected at 18 months compared to baseline (95.2 g/m2(66.0–184.0) vs 99.0 g/m2(69.0–121.0), p=0.55). Overall, there was an increase in septal T1 of borderline significance (870.0 ms (848–882) vs 860.0 ms (833.0–875.0), p=0.056). Functional capacity showed an increase in oxygen consumption (VO2) at anaerobic threshold (15.50 mL/kg/min (13.70–21.50) vs 14.50 mL/kg/min (11.70–18.95), p=0.02), and a trend towards an increase in percent predicted peak VO2(72.0 (63.0–80.0) vs 69.0 (53.0–77.0), p=0.056) was observed. The subset of patients who showed an increase in T1 value and a reduction in LV mass (n=7, 1 female, age 40.5 (28.6–76.0)) was younger and at an earlier disease stage compared to the others, and also exhibited greater improvement in exercise tolerance.ConclusionIn treatment-naïve FD patients with cardiac involvement, 18-month treatment with migalastat stabilised LV mass and was associated with a trend towards an improvement in exercise tolerance. A tendency to T1 increase was detected by CMR. The subset of patients who had significant benefits from the treatment showed an earlier cardiac disease compared to the others.Trial registration numberNCT03838237.

Published
2023

48. ECG-based score estimates the probability to detect Fabry Disease cardiac involvement

Author

Kelvin Chow, Massimo Lombardi, Paola Lusardi, Antonia Camporeale, Andrea Bernardini, Sara Boveri, Mehdi Namdar, Federico Pieruzzi, Alessandro P. Burlina, Marco Spada, Lara Tondi, Silvia Pica, Renzo Mignani, Maurizio Pieroni, Stefano Figliozzi, Francesca Graziani, Figliozzi, S, Camporeale, A, Boveri, S, Pieruzzi, F, Pieroni, M, Lusardi, P, Spada, M, Mignani, R, Burlina, A, Graziani, F, Pica, S, Tondi, L, Bernardini, A, Chow, K, Namdar, M, and Lombardi, M

Subjects
medicine.medical_specialty, Intraclass correlation, Magnetic Resonance Imaging, Cine, Electrocardiography, QRS complex, Predictive Value of Tests, Internal medicine, medicine, Humans, cardiovascular diseases, Probability, Fabry disease, Ejection fraction, medicine.diagnostic_test, Left bundle branch block, business.industry, Myocardium, T1 mapping, Nomogram, medicine.disease, Magnetic Resonance Imaging, Cross-Sectional Studies, Early Diagnosis, Cine, Settore MED/11 - MALATTIE DELL'APPARATO CARDIOVASCOLARE, Cohort, cardiovascular system, Cardiology, Cardiovascular magnetic resonance, Cardiology and Cardiovascular Medicine, business
Abstract

Objectives To elaborate an ECG-based nomogram estimating the probability to detect cardiac involvement by cardiac magnetic resonance (CMR) in Fabry Disease (FD). Methods 119 FD patients and 26 healthy controls underwent ECG and CMR. Test (n = 88, 60%) and validation cohorts (n = 57, 40%) were randomly derived. Cardiac involvement was defined as the presence of low myocardial T1 value, a CMR-surrogate of myocardial glycosphingolipid storage. ECG changes associated with low T1 value were identified in the test cohort, included in the nomogram and then tested in the validation cohort. Results Sokolow-Lyon index (AUC = 0.769), ratio between P-wave and PR-segment durations (Pwave/PRsegment) (AUC = 0.778), QRS duration (AUC = 0.703), QT (AUC = 0.769) duration were independently associated with the presence of low T1 on CMR at multivariate analysis. An ECG-based nomogram including these four parameters was accurate in identifying patients with CMR evidence of glycosphingolipid storage (c-index of the derived-nomogram = 0.90 in the test group; 0.81 in the validation group). Conclusion We propose a practical ECG-based nomogram accurately estimating the probability to detect low T1 values by CMR in FD patients. The application of this tool in clinical practice could improve early detection of FD cardiac involvement.

Published
2021

49. Myocardial Fibrosis at Cardiac MRI Helps Predict Adverse Clinical Outcome in Patients with Mitral Valve Prolapse

Author

Stefano Figliozzi, Georgios Georgiopoulos, Pedro M. Lopes, Klemens B. Bauer, Sara Moura-Ferreira, Lara Tondi, Saima Mushtaq, Stefano Censi, Anna Giulia Pavon, Ilaria Bassi, Maria Luz Servato, Arco J. Teske, Federico Biondi, Domenico Filomena, Silvia Pica, Camilla Torlasco, Denisa Muraru, Pierre Monney, Giuseppina Quattrocchi, Viviana Maestrini, Luciano Agati, Lorenzo Monti, Patrizia Pedrotti, Bert Vandenberk, Angelo Squeri, Massimo Lombardi, António M. Ferreira, Juerg Schwitter, Giovanni Donato Aquaro, Amedeo Chiribiri, José F. Rodríguez Palomares, Ali Yilmaz, Daniele Andreini, Anca Florian, Tim Leiner, João Abecasis, Luigi Paolo Badano, Jan Bogaert, Pier-Giorgio Masci, Figliozzi, S, Georgiopoulos, G, Lopes, P, Bauer, K, Moura-Ferreira, S, Tondi, L, Mushtaq, S, Censi, S, Pavon, A, Bassi, I, Servato, M, Teske, A, Biondi, F, Filomena, D, Pica, S, Torlasco, C, Muraru, D, Monney, P, Quattrocchi, G, Maestrini, V, Agati, L, Monti, L, Pedrotti, P, Vandenberk, B, Squeri, A, Lombardi, M, Ferreira, A, Schwitter, J, Aquaro, G, Chiribiri, A, Rodríguez Palomares, J, Yilmaz, A, Andreini, D, Florian, A, Leiner, T, Abecasis, J, Badano, L, Bogaert, J, and Masci, P

Subjects
Radiology, Nuclear Medicine and imaging, myocardial fibrosis, MED/11 - MALATTIE DELL'APPARATO CARDIOVASCOLARE, mitral valve prolapse, cardiac magnetic resonance
Abstract

Background: Patients with mitral valve prolapse (MVP) may develop adverse outcomes even in the absence of mitral regurgitation or left ventricular (LV) dysfunction. Purpose: To investigate the prognostic value of mitral annulus disjunction (MAD) and myocardial fibrosis at late gadolinium enhancement (LGE) cardiac MRI in patients with MVP without moderate-to-severe mitral regurgitation or LV dysfunction. Materials and Methods: In this longitudinal retrospective study, 118 144 cardiac MRI studies were evaluated between October 2007 and June 2020 at 15 European tertiary medical centers. Follow-up was from the date of cardiac MRI examination to June 2020; the minimum and maximum follow-up intervals were 6 months and 156 months, respectively. Patients were excluded if at least one of the following conditions was present: cardiomyopathy, LV ejection fraction less than 40%, ischemic heart disease, congenital heart disease, inflammatory heart disease, moderate or worse mitral regurgitation, participation in competitive sport, or electrocardiogram suggestive of channelopathies. In the remainder, cardiac MRI studies were reanalyzed, and patients were included if they were aged 18 years or older, MVP was diagnosed at cardiac MRI, and clinical information and electrocardiogram monitoring were available within 3 months from cardiac MRI examination. The end point was a composite of adverse outcomes: sustained ventricular tachycardia (VT), sudden cardiac death (SCD), or unexplained syncope. Multivariable Cox regression analysis was performed. Results: A total of 474 patients (mean age, 47 years ± 16 [SD]; 244 women) were included. Over a median follow-up of 3.3 years, 18 patients (4%) reached the study end point. LGE presence (hazard ratio, 4.2 [95% CI: 1.5, 11.9]; P = .006) and extent (hazard ratio, 1.2 per 1% increase [95% CI: 1.1, 1.4]; P = .006), but not MAD presence (P = .89), were associated with clinical outcome. LGE presence had incremental prognostic value over MVP severity and sustained VT and aborted SCD at baseline (area under the receiver operating characteristic curve, 0.70 vs 0.62; P = .03). Conclusion: In contrast to mitral annulus disjunction, myocardial fibrosis determined according to late gadolinium enhancement at cardiac MRI was associated with adverse outcome in patients with mitral valve prolapse without moderate-to-severe mitral regurgitation or left ventricular dysfunction.

Published
2022

50. Pregnancy Outcomes in Women After Arterial Switch Operation for Transposition of the Great Arteries: Results From ROPAC (Registry of Pregnancy and Cardiac Disease) of the European Society of Cardiology EURObservational Research Programme

Author

Oktay Tutarel, Karishma P. Ramlakhan, Lucia Baris, Maria T. Subirana, Judith Bouchardy, Attila Nemes, Niels G. Vejlstrup, Olga A. Osipova, Mark R. Johnson, Roger Hall, Jolien W. Roos‐Hesselink, Christopher Peter Gale, Branko Beleslin, Andrzej Budaj, Ovidiu Chioncel, Nikolaos Dagres, Nicolas Danchin, David Erlinge, Jonathan Emberson, Michael Glikson, Alastair Gray, Meral Kayikcioglu, Aldo Maggioni, Klaudia Vivien Nagy, Aleksandr Nedoshivin, Anna‐Sonia Petronio, Jolien Roos‐Hesselink, Lars Wallentin, Uwe Zeymer, Joerg Stein, William Anthony Parsonage, Werner Budts, Julie De Backer, Jasmin Grewal, Ariane Marelli, Harald Kaemmerer, Guillaume Jondeau, Mark Johnson, Aldo P. Maggioni, Luigi Tavazzi, Ulf Thilen, Uri Elkayam, Catherine Otto, Karen Sliwa, A. Aquieri, A. Saad, H. Ruda Vega, J. Hojman, J. M. Caparros, M. Vazquez Blanco, M. Arstall, C. M. Chung, G. Mahadavan, E. Aldridge, M. Wittwer, Y. Y. Chow, W. A. Parsonage, K. Lust, N. Collins, G. Warner, R. Hatton, A. Gordon, E. Nyman, J. Stein, E. Donhauser, H. Gabriel, A. Bahshaliyev, F. Guliyev, I. Hasanova, T. Jahangirov, Z. Gasimov, A. Salim, C. M. Ahmed, F. Begum, M. H. Hoque, M. Mahmood, M. N. Islam, P. P. Haque, S. K. Banerjee, T. Parveen, M. Morissens, J. De Backer, L. Demulier, M. de Hosson, W. Budts, M. Beckx, M. Kozic, M. Lovric, T. Kovacevic‐Preradovic, N. Chilingirova, P. Kratunkov, N. Wahab, S. McLean, E. Gordon, L. Walter, A. Marelli, A. R. Montesclaros, G. Monsalve, C. Rodriguez, F. Balthazar, V. Quintero, W. Palacio, L. A. Mejía Cadavid, E. Munoz Ortiz, F. Fortich Hoyos, E. Arevalo Guerrero, J. Gandara Ricardo, J. Velasquez Penagos, Z. Vavera, J. Popelova, N. Vejlstrup, L. Grønbeck, M. Johansen, A. Ersboll, Y. Elrakshy, K. Eltamawy, M. Gamal Abd‐El Aziz, A. El Nagar, H. Ebaid, H. Abo Elenin, M. Saed, S. Farag, W. Makled, K. Sorour, Z. Ashour, G. El‐Sayed, M. Abdel Meguid Mahdy, N. Taha, A. Dardeer, M. Shabaan, M. Ali, P. Moceri, G. Duthoit, M. Gouton, J. Nizard, L. Baris, S. Cohen, M. Ladouceur, D. Khimoud, B. Iung, F. Berger, A. Olsson, U. Gembruch, W. M. Merz, E. Reinert, S. Clade, Y. Kliesch, C. Wald, C. Sinning, R. Kozlik‐Feldmann, S. Blankenberg, E. Zengin‐Sahm, G. Mueller, M. Hillebrand, P. Hauck, Y. von Kodolitsch, N. Zarniko, H. Baumgartner, R. Schmidt, A. Hellige, O. Tutarel, H. Kaemmerer, B. Kuschel, N. Nagdyman, R. Motz, D. Maisuradze, A. Frogoudaki, E. Iliodromitis, M. Anastasiou‐Nana, D. Triantafyllis, G. Bekiaris, H. Karvounis, G. Giannakoulas, D. Ntiloudi, S. A. Mouratoglou, A. Temesvari, H. Balint, D. Kohalmi, B. Merkely, C. Liptai, A. Nemes, T. Forster, A. Kalapos, K. Berek, K. Havasi, N. Ambrus, A. Shelke, R. Kawade, S. Patil, E. Martanto, T. M. Aprami, A. Purnomowati, C. J. Cool, M. Hasan, R. Akbar, S. Hidayat, T. I. Dewi, W. Permadi, D. A. Soedarsono, M. M. Ansari‐Ramandi, N. Samiei, A. Tabib, F. Kashfi, S. Ansari‐Ramandi, S. Rezaei, H. Ali Farhan, A. Al‐Hussein, G. Al‐Saedi, G. Mahmood, I. F. Yaseen, L. Al‐Yousuf, M. AlBayati, S. Mahmood, S. Raheem, T. AlHaidari, Z. Dakhil, P. Thornton, J. Donnelly, M. Bowen, A. Blatt, G. Elbaz‐Greener, A. Shotan, S. Yalonetsky, S. Goland, M. Biener, G. Egidy Assenza, M. Bonvicini, A. Donti, A. Bulgarelli, D. Prandstraller, C. Romeo, R. Crepaz, E. Sciatti, M. Metra, R. Orabona, L. Ait Ali, P. Festa, V. Fesslova, C. Bonanomi, M. Calcagnino, F. Lombardi, null Colli, M. W. Ossola, C. Gobbi, E. Gherbesi, L. Tondi, M. Schiavone, M. Squillace, M. G. Carmina, A. Maina, C. Macchi, E. Gollo, F. M. Comoglio, N. Montali, P. Re, R. Bordese, T. Todros, V. Donvito, W. Grosso Marra, G. Sinagra, B. D'Agata Mottolese, M. Bobbo, V. Gesuete, S. Rakar, F. Ramani, K. Niwa, D. Mekebekova, A. Mussagaliyeva, T. Lee, E. Mirrakhimov, S. Abilova, E. Bektasheva, K. Neronova, O. Lunegova, R. Žaliūnas, R. Jonkaitienė, J. Petrauskaitė, A. Laucevicius, D. Jancauskaite, L. Lauciuviene, L. Gumbiene, L. Lankutiene, S. Glaveckaite, M. Laukyte, S. Solovjova, V Rudiene, K. H. Chee, C. C.‐W. Yim, H. L. Ang, R. Kuppusamy, T. Watson, M. Caruana, M.‐E. Estensen, M. G. A. Mahmood Kayani, R. Munir, A. Tomaszuk‐Kazberuk, B. Sobkowicz, J. Przepiesc, A. Lesniak‐Sobelga, L. Tomkiewicz‐Pajak, M. Komar, M. Olszowska, P. Podolec, S. Wisniowska‐Smialek, M. Lelonek, U. Faflik, A. Cichocka‐Radwan, K. Plaskota, O. Trojnarska, N. Guerra, L. de Sousa, C. Cruz, V. Ribeiro, S. Jovanova, V. Petrescu, R. Jurcut, C. Ginghina, I. Mircea Coman, M. Musteata, O. Osipova, T. Golivets, I. Khamnagadaev, O. Golovchenko, A. Nagibina, I. Ropatko, I. R. Gaisin, L. Valeryevna Shilina, N. Sharashkina, E. Shlyakhto, O. Irtyuga, O. Moiseeva, E. Karelkina, I. Zazerskaya, A. Kozlenok, I. Sukhova, L. Jovovic, K. Prokšelj, M. Koželj, A. O. Askar, A. A. Abdilaahi, M. H. Mohamed, A. M. Dirir, K. Sliwa, P. Manga, A. Pijuan‐Domenech, L. Galian‐Gay, P. Tornos, M. T. Subirana, N. Murga, J. M. Oliver, B. Garcia‐Aranda Dominguez, I. Hernandez Gonzalez, J. F. Delgado Jimenez, P. Escribano Subias, A. Elbushi, A. Suliman, K. Jazzar, M. Murtada, N. Ahamed, M. Dellborg, E. Furenas, M. Jinesjo, K. Skoglund, P. Eriksson, T. Gilljam, U. Thilen, D. Tobler, K. Wustmann, F. Schwitz, M. Schwerzmann, T. Rutz, J. Bouchardy, M. Greutmann, B. M. Santos Lopes, L. Meier, M. Arrigo, K. de Boer, T. Konings, E. Wajon, L. J. Wagenaar, P. Polak, E. P. G. Pieper, J. Roos‐Hesselink, I. van Hagen, H. Duvekot, J. M. J. Cornette, C. De Groot, C. van Oppen, L. Sarac, O. Batukan Esen, S. Catirli Enar, C. Mondo, P. Ingabire, B. Nalwanga, T. Semu, B. T. Salih, W. A. R. Almahmeed, S. Wani, F. S. Mohamed Farook, Al Ain, F. Gerges, A. M. Komaranchath, F. Al bakshi, A. Al Mulla, A. H. Yusufali, E. I. Al Hatou, N. Bazargani, F. Hussain, L. Hudsmith, P. Thompson, S. Thorne, S. Bowater, A. Money‐Kyrle, P. Clifford, P. Ramrakha, S. Firoozan, J. Chaplin, N. Bowers, D. Adamson, F. Schroeder, R. Wendler, S. Hammond, P. Nihoyannopoulos, R. Hall, L. Freeman, G. Veldtman, J. Kerr, L. Tellett, N. Scott, A. B. Bhatt, D. DeFaria Yeh, M. A. Youniss, M. Wood, A. A. Sarma, S. Tsiaras, A. Stefanescu, J. M. Duran, L. Stone, D. S. Majdalany, J. Chapa, K. Chintala, P. Gupta, J. Botti, J. Ting, W. R. Davidson, G. Wells, D. Sparks, V. Paruchuri, K. Marzo, D. Patel, W. Wagner, S. N. Ahanya, L. Colicchia, T. Jentink, K. Han, M. Loichinger, M. Parker, C. Longtin, A. Yetman, K. Erickson, J. Cramer, S. Tsai, B. Fletcher, S. Warta, C. Cohen, C. Lindblade, R. Puntel, K. Nagaran, N. Croft, M. Gurvitz, C. Otto, C. Talluto, D. Murphy, M. G. Perlroth, ROPAC (Registry of Pregnancy and Cardiac Disease) Investigators Group, Gale, C.P., Beleslin, B., Budaj, A., Chioncel, O., Dagres, N., Danchin, N., Erlinge, D., Emberson, J., Glikson, M., Gray, A., Kayikcioglu, M., Maggioni, A., Nagy, K.V., Nedoshivin, A., Petronio, A.S., Roos-Hesselink, J., Wallentin, L., Zeymer, U., Hall, R., Stein, J., Parsonage, W.A., Budts, W., De Backer, J., Grewal, J., Marelli, A., Kaemmerer, H., Jondeau, G., Johnson, M., Maggioni, A.P., Tavazzi, L., Thilen, U., Elkayam, U., Otto, C., Sliwa, K., Aquieri, A., Saad, A., Ruda Vega, H., Hojman, J., Caparros, J.M., Vazquez Blanco, M., Arstall, M., Chung, C.M., Mahadavan, G., Aldridge, E., Wittwer, M., Chow, Y.Y., Lust, K., Collins, N., Warner, G., Hatton, R., Gordon, A., Nyman, E., Donhauser, E., Gabriel, H., Bahshaliyev, A., Guliyev, F., Hasanova, I., Jahangirov, T., Gasimov, Z., Salim, A., Ahmed, C.M., Begum, F., Hoque, M.H., Mahmood, M., Islam, M.N., Haque, P.P., Banerjee, S.K., Parveen, T., Morissens, M., Demulier, L., de Hosson, M., Beckx, M., Kozic, M., Lovric, M., Kovacevic-Preradovic, T., Chilingirova, N., Kratunkov, P., Wahab, N., McLean, S., Gordon, E., Walter, L., Montesclaros, A.R., Monsalve, G., Rodriguez, C., Balthazar, F., Quintero, V., Palacio, W., Mejía Cadavid, L.A., Munoz Ortiz, E., Fortich Hoyos, F., Arevalo Guerrero, E., Gandara Ricardo, J., Velasquez Penagos, J., Vavera, Z., Popelova, J., Vejlstrup, N., Grønbeck, L., Johansen, M., Ersboll, A., Elrakshy, Y., Eltamawy, K., Gamal Abd-El Aziz, M., El Nagar, A., Ebaid, H., Abo Elenin, H., Saed, M., Farag, S., Makled, W., Sorour, K., Ashour, Z., El-Sayed, G., Abdel Meguid Mahdy, M., Taha, N., Dardeer, A., Shabaan, M., Ali, M., Moceri, P., Duthoit, G., Gouton, M., Nizard, J., Baris, L., Cohen, S., Ladouceur, M., Khimoud, D., Iung, B., Berger, F., Olsson, A., Gembruch, U., Merz, W.M., Reinert, E., Clade, S., Kliesch, Y., Wald, C., Sinning, C., Kozlik-Feldmann, R., Blankenberg, S., Zengin-Sahm, E., Mueller, G., Hillebrand, M., Hauck, P., von Kodolitsch, Y., Zarniko, N., Baumgartner, H., Schmidt, R., Hellige, A., Tutarel, O., Kuschel, B., Nagdyman, N., Motz, R., Maisuradze, D., Frogoudaki, A., Iliodromitis, E., Anastasiou-Nana, M., Triantafyllis, D., Bekiaris, G., Karvounis, H., Giannakoulas, G., Ntiloudi, D., Mouratoglou, S.A., Temesvari, A., Balint, H., Kohalmi, D., Merkely, B., Liptai, C., Nemes, A., Forster, T., Kalapos, A., Berek, K., Havasi, K., Ambrus, N., Shelke, A., Kawade, R., Patil, S., Martanto, E., Aprami, T.M., Purnomowati, A., Cool, C.J., Hasan, M., Akbar, R., Hidayat, S., Dewi, T.I., Permadi, W., Soedarsono, D.A., Ansari-Ramandi, M.M., Samiei, N., Tabib, A., Kashfi, F., Ansari-Ramandi, S., Rezaei, S., Ali Farhan, H., Al-Hussein, A., Al-Saedi, G., Mahmood, G., Yaseen, I.F., Al-Yousuf, L., AlBayati, M., Mahmood, S., Raheem, S., AlHaidari, T., Dakhil, Z., Thornton, P., Donnelly, J., Bowen, M., Blatt, A., Elbaz-Greener, G., Shotan, A., Yalonetsky, S., Goland, S., Biener, M., Egidy Assenza, G., Bonvicini, M., Donti, A., Bulgarelli, A., Prandstraller, D., Romeo, C., Crepaz, R., Sciatti, E., Metra, M., Orabona, R., Ait Ali, L., Festa, P., Fesslova, V., Bonanomi, C., Calcagnino, M., Lombardi, F., Colli, C., Ossola, M.W., Gobbi, C., Gherbesi, E., Tondi, L., Schiavone, M., Squillace, M., Carmina, M.G., Maina, A., Macchi, C., Gollo, E., Comoglio, F.M., Montali, N., Re, P., Bordese, R., Todros, T., Donvito, V., Grosso Marra, W., Sinagra, G., D'Agata Mottolese, B., Bobbo, M., Gesuete, V., Rakar, S., Ramani, F., Niwa, K., Mekebekova, D., Mussagaliyeva, A., Lee, T., Mirrakhimov, E., Abilova, S., Bektasheva, E., Neronova, K., Lunegova, O., Žaliūnas, R., Jonkaitienė, R., Petrauskaitė, J., Laucevicius, A., Jancauskaite, D., Lauciuviene, L., Gumbiene, L., Lankutiene, L., Glaveckaite, S., Laukyte, M., Solovjova, S., Rudiene, V., Chee, K.H., Yim, C.C., Ang, H.L., Kuppusamy, R., Watson, T., Caruana, M., Estensen, M.E., Mahmood Kayani, MGA, Munir, R., Tomaszuk-Kazberuk, A., Sobkowicz, B., Przepiesc, J., Lesniak-Sobelga, A., Tomkiewicz-Pajak, L., Komar, M., Olszowska, M., Podolec, P., Wisniowska-Smialek, S., Lelonek, M., Faflik, U., Cichocka-Radwan, A., Plaskota, K., Trojnarska, O., Guerra, N., de Sousa, L., Cruz, C., Ribeiro, V., Jovanova, S., Petrescu, V., Jurcut, R., Ginghina, C., Mircea Coman, I., Musteata, M., Osipova, O., Golivets, T., Khamnagadaev, I., Golovchenko, O., Nagibina, A., Ropatko, I., Gaisin, I.R., Valeryevna Shilina, L., Sharashkina, N., Shlyakhto, E., Irtyuga, O., Moiseeva, O., Karelkina, E., Zazerskaya, I., Kozlenok, A., Sukhova, I., Jovovic, L., Prokšelj, K., Koželj, M., Askar, A.O., Abdilaahi, A.A., Mohamed, M.H., Dirir, A.M., Manga, P., Pijuan-Domenech, A., Galian-Gay, L., Tornos, P., Subirana, M.T., Murga, N., Oliver, J.M., Garcia-Aranda Dominguez, B., Hernandez Gonzalez, I., Delgado Jimenez, J.F., Escribano Subias, P., Elbushi, A., Suliman, A., Jazzar, K., Murtada, M., Ahamed, N., Dellborg, M., Furenas, E., Jinesjo, M., Skoglund, K., Eriksson, P., Gilljam, T., Tobler, D., Wustmann, K., Schwitz, F., Schwerzmann, M., Rutz, T., Bouchardy, J., Greutmann, M., Santos Lopes, B.M., Meier, L., Arrigo, M., de Boer, K., Konings, T., Wajon, E., Wagenaar, L.J., Polak, P., Pieper, EPG, van Hagen, I., Duvekot, H., Cornette, JMJ, De Groot, C., van Oppen, C., Sarac, L., Batukan Esen, O., Catirli Enar, S., Mondo, C., Ingabire, P., Nalwanga, B., Semu, T., Salih, B.T., Almahmeed, WAR, Wani, S., Mohamed Farook, F.S., Ain, A., Gerges, F., Komaranchath, A.M., Al Bakshi, F., Al Mulla, A., Yusufali, A.H., Al Hatou, E.I., Bazargani, N., Hussain, F., Hudsmith, L., Thompson, P., Thorne, S., Bowater, S., Money-Kyrle, A., Clifford, P., Ramrakha, P., Firoozan, S., Chaplin, J., Bowers, N., Adamson, D., Schroeder, F., Wendler, R., Hammond, S., Nihoyannopoulos, P., Freeman, L., Veldtman, G., Kerr, J., Tellett, L., Scott, N., Bhatt, A.B., DeFaria Yeh, D., Youniss, M.A., Wood, M., Sarma, A.A., Tsiaras, S., Stefanescu, A., Duran, J.M., Stone, L., Majdalany, D.S., Chapa, J., Chintala, K., Gupta, P., Botti, J., Ting, J., Davidson, W.R., Wells, G., Sparks, D., Paruchuri, V., Marzo, K., Patel, D., Wagner, W., Ahanya, S.N., Colicchia, L., Jentink, T., Han, K., Loichinger, M., Parker, M., Longtin, C., Yetman, A., Erickson, K., Cramer, J., Tsai, S., Fletcher, B., Warta, S., Cohen, C., Lindblade, C., Puntel, R., Nagaran, K., Croft, N., Gurvitz, M., Talluto, C., Murphy, D., Perlroth, M.G., Neurosurgery, Pediatrics, Cardiology, ACS - Heart failure & arrhythmias, Obstetrics and gynaecology, Amsterdam Reproduction & Development (AR&D), Institut Català de la Salut, [Tutarel O] Department of Congenital Heart Disease and Paediatric Cardiology German Heart Centre MunichTechnical University of Munich School of MedicineTechnical University of Munich Germany. DZHK (German Centre for Cardiovascular Research), partner site Munich Heart Alliance Munich Germany. [Ramlakhan KP, Baris L] Department of Cardiology Erasmus University Medical Center Rotterdam the Netherlands. [Subirana MT] Unitat de Cardiopaties congènites de l’adult, Vall d'Hebron Hospital Universitari, Barcelona Spain. Hospital Sant Pau, Barcelona Spain. [Bouchardy J] Service of Cardiology University Hospital Lausanne and University of Lausanne Switzerland. Service of Cardiology University of Geneva Switzerland. [Nemes A] 2nd Department of Medicine and Cardiology Centre Medical Faculty Albert Szent-Györgyi Clinical Center University of Szeged Hungary, Szeged, Hungary, and Vall d'Hebron Barcelona Hospital Campus

Subjects
Male, Transposition of Great Vessels, pregnancy outcomes, enfermedades cardiovasculares::anomalías cardiovasculares::cardiopatías congénitas::transposición de los grandes vasos [ENFERMEDADES], Disease, 030204 cardiovascular system & hematology, Sistema cardiovascular - Malalties, Ventricular tachycardia, Vasos sanguinis - Cirurgia, 0302 clinical medicine, Pregnancy, Clinical endpoint, Registries, 030212 general & internal medicine, Cardiovascular Diseases::Pregnancy Complications, Cardiovascular [DISEASES], Original Research, Aortic dissection, Pregnancy Outcome, Congenital Heart Disease, Other subheadings::Other subheadings::/surgery [Other subheadings], arterial switch operation, pregnancy and cardiac disease, transposition of the great arteries, Europe, Great arteries, Cardiology, enfermedades cardiovasculares::complicaciones cardiovasculares del embarazo [ENFERMEDADES], Female, Maternal death, Cardiology and Cardiovascular Medicine, Adult, medicine.medical_specialty, diagnóstico::pronóstico::resultado del embarazo [TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS], Pregnancy Complications, Cardiovascular, Embaràs - Complicacions, Cardiovascular Diseases::Cardiovascular Abnormalities::Heart Defects, Congenital::Transposition of Great Vessels [DISEASES], Risk Assessment, 03 medical and health sciences, SDG 3 - Good Health and Well-being, Internal medicine, medicine, Humans, Heart Failure, business.industry, Infant, Newborn, Otros calificadores::Otros calificadores::/cirugía [Otros calificadores], Diagnosis::Prognosis::Pregnancy Outcome [ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT], medicine.disease, Arterial Switch Operation, Heart failure, Tachycardia, Ventricular, business
Abstract

Embaràs i malaltia cardíaca; Resultats de l’embaràs; Transposició de les grans artèries Embarazo y enfermedad cardíaca; Resultados del embarazo; Transposición de las grandes arterias Pregnancy and cardiac disease; Pregnancy outcomes, Transposition of the great arteries Background In the past 3 decades, the arterial switch procedure has replaced the atrial switch procedure as treatment of choice for transposition of the great arteries. Although survival is superior after the arterial switch procedure, data on pregnancy outcomes are scarce and transposition of the great arteries after arterial switch is not yet included in the modified World Health Organization classification of maternal cardiovascular risk. Methods and Results The ROPAC (Registry of Pregnancy and Cardiac disease) is an international prospective registry of pregnant women with cardiac disease, part of the European Society of Cardiology EURObservational Research Programme. Pregnancy outcomes in all women after an arterial switch procedure for transposition of the great arteries are described. The primary end point was a major adverse cardiovascular event, defined as combined end point of maternal death, supraventricular or ventricular arrhythmias requiring treatment, heart failure, aortic dissection, endocarditis, ischemic coronary events, and thromboembolic events. Altogether, 41 pregnant women (mean age, 26.7±3.9 years) were included, and there was no maternal mortality. A major adverse cardiovascular event occurred in 2 women (4.9%): heart failure in one (2.4%) and ventricular tachycardia in another (2.4%). One woman experienced fetal loss, whereas no neonatal mortality was observed. Conclusions Women after an arterial switch procedure for transposition of the great arteries tolerate pregnancy well, with a favorable maternal and fetal outcome. During counseling, most women should be reassured that the risk of pregnancy is low. Classification as modified World Health Organization risk class II seems appropriate. Funding from “Zabawas Foundation” and “De Hoop Foundation” in addition to the support from EURObservational Research Programme (EORP) is greatly acknowledged. Since the start of EORP, the following companies have supported the program: Abbott Vascular Int (2011–2021), Amgen Cardiovascular (2009–2018), AstraZeneca (2014–2021), Bayer AG (2009–2018), Boehringer Ingelheim (2009–2019), Boston Scientific (2009–2012), The Bristol Myers Squibb and Pfizer Alliance (2011–2019), Daiichi Sankyo Europe GmbH (2011–2020), The Alliance Daiichi Sankyo Europe GmbH and Eli Lilly and Company (2014–2017), Edwards (2016–2019), Gedeon Richter Plc (2014–2016), Menarini Int Op (2009–2012), MSD‐Merck & Co (2011–2014), Novartis Pharma AG (2014–2020), ResMed (2014–2016), Sanofi (2009–2011), SERVIER (2009–2021), and Vifor (2019–2022).

Published
2021

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