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

Author

Van Hagen I. M., Baart S., Fong Soe Khioe R., Sliwa-Hahnle K., Taha N., Lelonek M., Tavazzi L., Maggioni A. P., Johnson M. R., Maniadakis N., Fordham R., Hall R., Roos-Hesselink J. W., Ferrari R., Marelli A., Webb G., Kaemmerer H., Popelova J., Sliwa K., Parsonage W. A., Stein J., Elkayam U., Thilen U., Budts W., Ruys T., Vardas P., Komajda M., Pinto F., Alonso A., Wood D., Ferreira T., Gracia G., Laroche C., Missiamenou V., Taylor C., Konte M., Andarala M., Fiorucci E., Lefrancq E. F., Glemot M., McNeill P-A., Pommier C., Lafay M., Aquieri A., Vega H. R., Blanco M. V., 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. V., 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. G., Saad A., Aboleineen W., Ashour Z., Sorour K., Mahdy M. A. M., 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. V., Shotan A., Goland S., Festa P., Ali L. A., Sinagra G., Puggia I., Mottolese B. D., 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. M., Gaisin I. R., Shilina L. V., 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-Subias P., Ruiz-Cano M. J., Delgado-Jimenez J., Furenas E., Dellborg M., 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., 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., Cardiology, Van Hagen, I. M., Baart, S., Fong Soe Khioe, R., Sliwa-Hahnle, K., Taha, N., Lelonek, M., Tavazzi, L., Maggioni, A. P., Johnson, M. R., Maniadakis, N., Fordham, R., Hall, R., Roos-Hesselink, J. W., Ferrari, R., Marelli, A., Webb, G., Kaemmerer, H., Popelova, J., Sliwa, K., Parsonage, W. A., Stein, J., Elkayam, U., Thilen, U., Budts, W., Ruys, T., Vardas, P., Komajda, M., Pinto, F., Alonso, A., Wood, D., Ferreira, T., Gracia, G., Laroche, C., Missiamenou, V., Taylor, C., Konte, M., Andarala, M., Fiorucci, E., Lefrancq, E. F., Glemot, M., Mcneill, P-A., Pommier, C., Lafay, M., Aquieri, A., Vega, H. R., Blanco, M. V., 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. V., 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. G., Saad, A., Aboleineen, W., Ashour, Z., Sorour, K., Mahdy, M. A. M., 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. V., Shotan, A., Goland, S., Festa, P., Ali, L. A., Sinagra, G., Puggia, I., Mottolese, B. D., 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. M., Gaisin, I. R., Shilina, L. V., 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-Subias, P., Ruiz-Cano, M. J., Delgado-Jimenez, J., Furenas, E., Dellborg, M., 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., 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.

Subjects
Registrie, Pediatrics, Cardiac & Cardiovascular Systems, Heart disease, global health, heart failure, Disease, 030204 cardiovascular system & hematology, Cardiovascular, 0302 clinical medicine, Pregnancy, Residence Characteristics, Prospective Studies, Registries, PREDICTORS, 1102 Cardiorespiratory Medicine and Haematology, CARDIOLOGY, 030219 obstetrics & reproductive medicine, valvular heart disease, Pregnancy Outcome, SOUTH-AFRICA, congenital heart disease, pregnancy, Adult, Analysis of Variance, Female, Global Health, Heart Diseases, Humans, Maternal Age, Pregnancy Complications, Cardiovascular, Socioeconomic Factors, EUROPEAN-SOCIETY, Heart Disease, CARDIOVASCULAR-DISEASE, RISK-ASSESSMENT, Cardiology and Cardiovascular Medicine, Life Sciences & Biomedicine, Human, Acute coronary syndrome, medicine.medical_specialty, ROPAC investigators, Birth rate, 03 medical and health sciences, medicine, CARDIAC-DISEASE, MATERNAL OUTCOMES, Socioeconomic status, Science & Technology, business.industry, INCOME INEQUALITY, medicine.disease, Pregnancy Complications, Prospective Studie, Cardiovascular System & Hematology, Residence Characteristic, Heart failure, REGISTRY, Cardiovascular System & Cardiology, business
Abstract

ObjectiveCardiac 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.MethodsThe 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).ResultsA 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.ConclusionWhile 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

3. Pregnancy Outcomes in Women With Rheumatic Mitral Valve Disease Results From the Registry of Pregnancy and Cardiac Disease

Author

Van Hagen I. M., Thorne S. A., Taha N., Youssef G., Elnagar A., Gabriel H., ElRakshy Y., Iung B., Johnson M. R., Hall R., Roos-Hesselink J. W., Ferrari R., Maggioni A. P., Marelli A., Webb G., Kaemmerer H., Popelova J., Sliwa K., Tavazzi L., Anthony Parsonage W., Stein J., Elkayam U., Thilen U., Budts W., Ruys T., Ferreira T., Missiamenou V., Folkesson Lefrancq E., Aquieri A., Ruda Vega H., Vazquez Blanco M., 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., Vilas Freire C., 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., A. El Sayed Makled W., Dardier A., Shabaan M., Eltamawey K., Gamal Abd-El Aziz M., Saad A., Aboleineen W., Ashour Z., Sorour K., A. Meguid Mahdy M., Iserin L., Ladouceur M., Cohen S., Maisuradze D., Mebus S., Gembruch U., Hammerstingl C., Merz W. M., Wald C., Hellige A., Baumgartner H., 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., Vazan Fuhrmann A., Shotan A., Goland S., Festa P., Ait Ali L., Sinagra G., Puggia I., D'Agata Mottolese B., 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., PG Pieper E., 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., Mircea Coman I., Ravilevich Gaisin I., Valeryevna Shilina L., 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., M. Oliver J., Escribano-Subias P., J. Ruiz-Cano M., Delgado-Jimenez J., Furenas E., Dellborg M., Schwerzmann M., Bouchardy J., Rutz T., Tobler D., Sarac L., Batukan Esen O., Catirli Enar S., Al Mulla A., Bazargani N., Al Hatou E., Farook F., Almahmeed W., Cardiology, Van Hagen, I. M., Thorne, S. A., Taha, N., Youssef, G., Elnagar, A., Gabriel, H., Elrakshy, Y., Iung, B., Johnson, M. R., Hall, R., Roos-Hesselink, J. W., Ferrari, R., Maggioni, A. P., Marelli, A., Webb, G., Kaemmerer, H., Popelova, J., Sliwa, K., Tavazzi, L., Anthony Parsonage, W., Stein, J., Elkayam, U., Thilen, U., Budts, W., Ruys, T., Ferreira, T., Missiamenou, V., Folkesson Lefrancq, E., Aquieri, A., Ruda Vega, H., Vazquez Blanco, M., 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., Vilas Freire, C., 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., A. El Sayed Makled, W., Dardier, A., Shabaan, M., Eltamawey, K., Gamal Abd-El Aziz, M., Saad, A., Aboleineen, W., Ashour, Z., Sorour, K., A. Meguid Mahdy, M., Iserin, L., Ladouceur, M., Cohen, S., Maisuradze, D., Mebus, S., Gembruch, U., Hammerstingl, C., Merz, W. M., Wald, C., Hellige, A., Baumgartner, H., 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., Vazan Fuhrmann, A., Shotan, A., Goland, S., Festa, P., Ait Ali, L., Sinagra, G., Puggia, I., D'Agata Mottolese, B., 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., PG Pieper, E., 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., Mircea Coman, I., Ravilevich Gaisin, I., Valeryevna Shilina, L., 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., M. Oliver, J., Escribano-Subias, P., J. Ruiz-Cano, M., Delgado-Jimenez, J., Furenas, E., Dellborg, M., Schwerzmann, M., Bouchardy, J., Rutz, T., Tobler, D., Sarac, L., Batukan Esen, O., Catirli Enar, S., Al Mulla, A., Bazargani, N., Al Hatou, E., Farook, F., and Almahmeed, W.

Subjects
Registrie, Cardiac & Cardiovascular Systems, VALVULAR HEART-DISEASE, heart disease, Disease, 030204 cardiovascular system & hematology, Cardiovascular, 0302 clinical medicine, Pregnancy, Models, Mitral valve, Rheumatic heart disease, Valvular heart disease, Women, Women and minorities, Adult, Female, Humans, Prospective Studies, Mitral Valve Insufficiency, Models, Cardiovascular, Pregnancy Complications, Cardiovascular, Pregnancy Outcome, Registries, Rheumatic Heart Disease, Prospective cohort study, 1102 Cardiorespiratory Medicine and Haematology, 030219 obstetrics & reproductive medicine, valvular heart disease, ROPAC Investigators and EORP Team, SOUTH-AFRICA, EUROPEAN-SOCIETY, OUTPUT, medicine.anatomical_structure, cardiovascular system, Cardiology, Cardiology and Cardiovascular Medicine, Life Sciences & Biomedicine, Women and minoritie, Human, medicine.medical_specialty, DEATHS, STENOSIS, 1117 Public Health and Health Services, 03 medical and health sciences, Physiology (medical), Internal medicine, MANAGEMENT, medicine, Pregnancy outcomes, Science & Technology, business.industry, 1103 Clinical Sciences, medicine.disease, Pregnancy Complications, Prospective Studie, Stenosis, Peripheral Vascular Disease, Cardiovascular System & Hematology, Cardiovascular System & Cardiology, rheumatic, business
Abstract

Background: Cardiac disease is 1 of the major causes of maternal mortality. We studied pregnancy outcomes in women with rheumatic mitral valve disease. Methods: The Registry of Pregnancy and Cardiac Disease is an international prospective registry, and consecutive pregnant women with cardiac disease were included. Pregnancy outcomes in all women with rheumatic mitral valve disease and no prepregnancy valve replacement is described in the present study (n=390). A maternal cardiac event was defined as cardiac death, arrhythmia requiring treatment, heart failure, thromboembolic event, aortic dissection, endocarditis, acute coronary syndrome, and hospitalization for other cardiac reasons or cardiac intervention. Associations between patient characteristics and cardiac outcomes were checked in a 3-level model (patient–center–country). Results: Most patients came from emerging countries (75%). Mitral stenosis (MS) with or without mitral regurgitation (MR) was present in 273 women, isolated MR in 117. The degree of MS was mild in 20.9%, moderate in 39.2%, severe in 19.8%, and severity not classified in the remainder. Maternal death during pregnancy occurred in 1 patient with severe MS. Hospital admission occurred in 23.1% of the women with MS, and the main reason was heart failure (mild MS 15.8%, moderate 23.4%, severe 48.1%; P 1 was an independent predictor of maternal cardiac events. Follow-up at 6 months postpartum was available for 53%, and 3 more patients died (1 with severe MS, 1 with moderate MS, 1 with moderate to severe MR). Conclusions: Although mortality was only 1.9% during pregnancy, ≈50% of the patients with severe rheumatic MS and 23% of those with significant MR developed heart failure during pregnancy. Prepregnancy counseling and considering mitral valve interventions in selected patients are important to prevent these complications.

Published
2018

4. 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

5. 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

6. 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

7. 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

9. Genetic Risk of Arrhythmic Phenotypes in Patients With Dilated Cardiomyopathy

Author

Dobromir Slavov, William J. McKenna, Marco Merlo, Marta Gigli, Matthew R.G. Taylor, Francesca Brun, Teisha J. Rowland, Gianfranco Sinagra, Giulia Barbati, Andrea Cocciolo, Sharon L. Graw, Mary E. Haywood, Davide Stolfo, Francisco G. La Rosa, Gaetano Morea, Alessandro Altinier, Federica Ramani, Matteo Dal Ferro, Ilaria Puggia, Luisa Mestroni, Merlo, M., Gigli, M., Graw, S. L., Barbati, G., Rowland, T. J., Slavov, D. B., Stolfo, D., Haywood, M. E., Dal Ferro, M., Altinier, A., Ramani, F., Brun, F., Cocciolo, A., Puggia, I., Morea, G., Mckenna, W. J., La Rosa, F. G., Taylor, M. R. G., Sinagra, G., and Mestroni, L.

Subjects
medicine.medical_specialty, desmosomal mutations, medicine.medical_treatment, genotype-phenotype correlation, 030204 cardiovascular system & hematology, dilated cardiomyopathy, prognosis, Sudden cardiac death, LMNA, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, Medicine, cardiovascular diseases, 030212 general & internal medicine, Heart transplantation, Ejection fraction, business.industry, Dilated cardiomyopathy, medicine.disease, Phenotype, desmosomal mutation, Ventricular assist device, Ventricular fibrillation, cardiovascular system, Cardiology, Cardiology and Cardiovascular Medicine, business
Abstract

Background Genotype-phenotype correlations in dilated cardiomyopathy (DCM) and, in particular, the effects of gene variants on clinical outcomes remain poorly understood. Objectives The purpose of this study was to investigate the prognostic role of genetic variant carrier status in a large cohort of DCM patients. Methods A total of 487 DCM patients were analyzed by next-generation sequencing and categorized the disease genes into functional gene groups. The following composite outcome measures were assessed: 1) all-cause mortality; 2) heart failure–related death, heart transplantation, or destination left ventricular assist device implantation (DHF/HTx/VAD); and 3) sudden cardiac death/sustained ventricular tachycardia/ventricular fibrillation (SCD/VT/VF). Results A total of 183 pathogenic/likely pathogenic variants were found in 178 patients (37%): 54 (11%) Titin; 19 (4%) Lamin A/C (LMNA); 24 (5%) structural cytoskeleton-Z disk genes; 16 (3.5%) desmosomal genes; 46 (9.5%) sarcomeric genes; 8 (1.6%) ion channel genes; and 11 (2.5%) other genes. All-cause mortality was no different between variant carriers and noncarriers (p = 0.99). A trend toward worse SCD/VT/VF (p = 0.062) and DHF/HTx/VAD (p = 0.061) was found in carriers. Carriers of desmosomal and LMNA variants experienced the highest rate of SCD/VT/VF, which was independent of the left ventricular ejection fraction. Conclusions Desmosomal and LMNA gene variants identify the subset of DCM patients who are at greatest risk for SCD and life-threatening ventricular arrhythmias, regardless of the left ventricular ejection fraction.

Published
2019
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10. Molecular and Cellular Mechanisms in Heart Failure

Author

Shelley D. Miyamoto, Luisa Mestroni, Gianfranco Sinagra, Teisha J. Rowland, Ilaria Puggia, John L. Jefferie, Anthony C. Chang, Joseph W. Rossano, Robert E. Shaddy, Jeffrey A. Towbin, Puggia, I., Rowland, T. J., Miyamoto, S. D., Sinagra, G., and Mestroni, L.

Subjects
Heart transplantation, Pressure overload, pediatric heart failure, business.industry, medicine.medical_treatment, Cardiomyopathy, Inflammation, medicine.disease, Bioinformatics, Fibrosis, Heart failure, Circulatory system, Medicine, medicine.symptom, business, Ventricular remodeling
Abstract

Pathophysiology and treatment of pediatric heart failure (HF) is poorly understood. A growing body of literature demonstrates age-related differences in mechanisms and in therapies efficacy. HF results from ventricular dysfunction due to volume or/and pressure overload. Circulatory, neurohormonal, and molecular alterations promote the progression of HF and ventricular remodeling; they include inflammation, oxidative stress, mitochondrial dysfunction, loss of cardiomyocytes, and fibrosis. Children and young affected by cardiomyopathies have the greatest risk of HF and heart transplantation. Genetic mutations of sarcomere, cytoskeleton, cell membrane proteins, and ion channels have been recognized as the main causes of many cardiomyopathy phenotypes. In particular, sarcomeric and cytoskeleton genes mutations seem to have an important role in the progression of HF. Prognostic stratification and clinical management could benefit from identification of biomarkers such as inflammatory mediators or microRNA (miRNA). miRNA and myocardial regenerative strategies are under investigations as potential novel therapeutic approaches.

Published
2018
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11. Filamin C Truncation Mutations Are Associated With Arrhythmogenic Dilated Cardiomyopathy and Changes in the Cell-Cell Adhesion Structures.

Author

Begay RL, Graw SL, Sinagra G, Asimaki A, Rowland TJ, Slavov DB, Gowan K, Jones KL, Brun F, Merlo M, Miani D, Sweet M, Devaraj K, Wartchow EP, Gigli M, Puggia I, Salcedo EE, Garrity DM, Ambardekar AV, Buttrick P, Reece TB, Bristow MR, Saffitz JE, Mestroni L, and Taylor MRG

Subjects
Arrhythmias, Cardiac, Cell Adhesion genetics, DNA Mutational Analysis, Europe, Humans, Immunohistochemistry, Polymorphism, Single Nucleotide genetics, Prospective Studies, United States, Cardiomyopathy, Dilated epidemiology, Cardiomyopathy, Dilated genetics, Cardiomyopathy, Dilated pathology, Filamins genetics, Mutation genetics, Myocardium cytology, Myocardium pathology
Abstract

Objectives: The purpose of this study was to assess the phenotype of Filamin C (FLNC) truncating variants in dilated cardiomyopathy (DCM) and understand the mechanism leading to an arrhythmogenic phenotype., Background: Mutations in FLNC are known to lead to skeletal myopathies, which may have an associated cardiac component. Recently, the clinical spectrum of FLNC mutations has been recognized to include a cardiac-restricted presentation in the absence of skeletal muscle involvement., Methods: A population of 319 U.S. and European DCM cardiomyopathy families was evaluated using whole-exome and targeted next-generation sequencing. FLNC truncation probands were identified and evaluated by clinical examination, histology, transmission electron microscopy, and immunohistochemistry., Results: A total of 13 individuals in 7 families (2.2%) were found to harbor 6 different FLNC truncation variants (2 stopgain, 1 frameshift, and 3 splicing). Of the 13 FLNC truncation carriers, 11 (85%) had either ventricular arrhythmias or sudden cardiac death, and 5 (38%) presented with evidence of right ventricular dilation. Pathology analysis of 2 explanted hearts from affected FLNC truncation carriers showed interstitial fibrosis in the right ventricle and epicardial fibrofatty infiltration in the left ventricle. Ultrastructural findings included occasional disarray of Z-discs within the sarcomere. Immunohistochemistry showed normal plakoglobin signal at cell-cell junctions, but decreased signals for desmoplakin and synapse-associated protein 97 in the myocardium and buccal mucosa., Conclusions: We found FLNC truncating variants, present in 2.2% of DCM families, to be associated with a cardiac-restricted arrhythmogenic DCM phenotype characterized by a high risk of life-threatening ventricular arrhythmias and a pathological cellular phenotype partially overlapping with arrhythmogenic right ventricular cardiomyopathy., (Copyright © 2018 The Authors. Published by Elsevier Inc. All rights reserved.)

Published
2018
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13. Natural History of Dilated Cardiomyopathy in Children.

Author

Puggia I, Merlo M, Barbati G, Rowland TJ, Stolfo D, Gigli M, Ramani F, Di Lenarda A, Mestroni L, and Sinagra G

Subjects
Adolescent, Adult, Age Factors, Arrhythmias, Cardiac epidemiology, Blood Pressure, Bundle-Branch Block epidemiology, Cardiomyopathy, Dilated epidemiology, Cardiomyopathy, Dilated genetics, Cardiomyopathy, Dilated therapy, Child, Death, Sudden, Cardiac epidemiology, Disease Progression, Female, Follow-Up Studies, Humans, Italy epidemiology, Male, Middle Aged, Prognosis, Stroke Volume, Time Factors, Cardiomyopathy, Dilated physiopathology, Heart Failure epidemiology, Heart Transplantation, Registries
Abstract

Background: The long-term progression of idiopathic dilated cardiomyopathy (DCM) in pediatric patients compared with adult patients has not been previously characterized. In this study, we compared outcome and long-term progression of pediatric and adult DCM populations., Methods and Results: Between 1988 and 2014, 927 DCM patients were consecutively enrolled. The pediatric population (aged <18 years at enrollment) included 47 participants (5.1%). At presentation, the pediatric population compared with adult patients had a significantly increased occurrence of familial forms (P=0.03), shorter duration of heart failure (P=0.04), lower systolic blood pressure (P=0.01), decreased presence of left bundle-branch block (P=0.001), and increased left ventricular ejection fraction (P=0.03). Despite these baseline differences, long-term longitudinal trends of New York Heart Association class III to IV, left ventricular dimensions, left ventricular ejection fraction, and restrictive filling pattern were similar between the 2 populations. Regarding survival analysis, because of the size difference between the 2 populations, we compared the pediatric population with a sample of adult patients randomly matched using the above-mentioned baseline differences in a 3:1 ratio (141 adult versus 47 pediatric patients). During a median follow-up of 110 months, survival free from heart transplantation was significantly lower among pediatric patients compared with adults (P<0.001). Furthermore, pediatric age (ie, <18 years) was found to be associated with an increasing risk of both death from pump failure and life-threatening arrhythmias., Conclusions: Despite the pediatric DCM population having higher baseline left ventricular ejection fraction and similar long-term echocardiographic progression compared with the adult DCM population, the pediatric DCM patients had worse cardiovascular prognosis., (© 2016 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.)

Published
2016
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14. Long-term outcome of 'super-responder' patients to cardiac resynchronization therapy.

Author

Zecchin M, Proclemer A, Magnani S, Vitali-Serdoz L, Facchin D, Muser D, Nordio A, Barbati G, Puggia I, Sinagra G, and Proclemer A

Subjects
Aged, Combined Modality Therapy, Comorbidity, Death, Sudden, Cardiac prevention & control, Female, Humans, Italy, Longitudinal Studies, Male, Middle Aged, Prevalence, Prognosis, Risk Factors, Treatment Outcome, Cardiac Resynchronization Therapy mortality, Death, Sudden, Cardiac epidemiology, Defibrillators, Implantable statistics & numerical data, Heart Failure mortality, Heart Failure prevention & control, Hospital Mortality
Abstract

Aims: To evaluate the long-term changes of clinical and echocardiographic parameters, the incidence of cardiac events and parameters associated with late cardiac events in 'super-responders' to cardiac resynchronization therapy (CRT) with [CRT defibrillator (CRT-D)] or without defibrillator back-up., Methods and Results: In all consecutive patients treated with CRT in two Italian centres (Trieste and Udine) with left ventricular ejection fraction (LVEF) ≤0.35 at implantation (Timp) and LVEF > 0.50 1 and/or 2 years (Tnorm) after implantation, the long-term outcome and the evolution of echocardiographic parameters were assessed; factors associated with a higher risk of cardiac events, defined as hospitalization or death for heart failure (HF), sudden death, or CRT-D appropriate interventions, were also analysed. Among the 259 patients evaluated, 62 (24%) had LVEF ≥ 0.50 at Tnorm (n = 44 with at 1 year, n = 18 at 2 years). During a mean follow-up of 68 ± 30 months, one cardiac death (for HF) and eight cardiovascular events (two hospitalization for HF and six appropriate CRT-D interventions) occurred. At the last echo evaluation (Tfup) performed 51 ± 26 months after Timp, LVEF was <0.50 in five patients (>0.45 in four of them). At univariable analysis, only LV end-systolic volume evaluated at Tfup was associated with a higher risk of cardiac events during follow-up., Conclusion: In 'super-responders' to CRT long-term outcome is excellent. However, cardiac events, mainly CRT-D appropriate interventions, can occur despite the persistence of LVEF > 0.50. Early identification of these patients is still an unsolved issue.

Published
2014
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