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Reversible cardiomyopathy in a patient with Marfan’s syndrome. Case report
Marfan´s syndrome is a multisystemic, autosomal dominant congenital abnormality of variable penetrance that affects the integrity of connective tissue. In the cardiovascular system, the dysfunction of the physiology of the aortic root and the myocardial fibrosis originates non-ischemic cardiomyopath...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Instituto Nacional Cardiovascular - INCOR
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10688408/ https://www.ncbi.nlm.nih.gov/pubmed/38046228 http://dx.doi.org/10.47487/apcyccv.v4i3.309 |
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author | Viñas-Mendieta, Adriana E. Cárdenas-Gallegos, Jesús K. Baltodano-Arellano, Roberto Chipa-Ccasani, Fredy Lévano-Pachas, Gerald C. Keirns, Candace Espinola-Zavaleta, Nilda |
author_facet | Viñas-Mendieta, Adriana E. Cárdenas-Gallegos, Jesús K. Baltodano-Arellano, Roberto Chipa-Ccasani, Fredy Lévano-Pachas, Gerald C. Keirns, Candace Espinola-Zavaleta, Nilda |
author_sort | Viñas-Mendieta, Adriana E. |
collection | PubMed |
description | Marfan´s syndrome is a multisystemic, autosomal dominant congenital abnormality of variable penetrance that affects the integrity of connective tissue. In the cardiovascular system, the dysfunction of the physiology of the aortic root and the myocardial fibrosis originates non-ischemic cardiomyopathy independent of valve lesions. Few data have been reported on the prevalence of arrhythmias and its impact on heart function. We present a 21-year-old man with Marfan’s syndrome and heart failure with frequent supraventricular arrhythmias and aortic root dilation. After ablation in the posteroseptal area of the mitral ring and Tirone David Surgery, there was clinical improvement, the left ventricular ejection fraction increased dramatically from 33% to 46%, the left ventricular end-diastolic volume decreased from 90 ml/m(2) to 77 ml/m(2) and the NT-proBNP decrease from 1100 pg/mL at 180 pg/mL. |
format | Online Article Text |
id | pubmed-10688408 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Instituto Nacional Cardiovascular - INCOR |
record_format | MEDLINE/PubMed |
spelling | pubmed-106884082023-12-01 Reversible cardiomyopathy in a patient with Marfan’s syndrome. Case report Viñas-Mendieta, Adriana E. Cárdenas-Gallegos, Jesús K. Baltodano-Arellano, Roberto Chipa-Ccasani, Fredy Lévano-Pachas, Gerald C. Keirns, Candace Espinola-Zavaleta, Nilda Arch Peru Cardiol Cir Cardiovasc Case Report Marfan´s syndrome is a multisystemic, autosomal dominant congenital abnormality of variable penetrance that affects the integrity of connective tissue. In the cardiovascular system, the dysfunction of the physiology of the aortic root and the myocardial fibrosis originates non-ischemic cardiomyopathy independent of valve lesions. Few data have been reported on the prevalence of arrhythmias and its impact on heart function. We present a 21-year-old man with Marfan’s syndrome and heart failure with frequent supraventricular arrhythmias and aortic root dilation. After ablation in the posteroseptal area of the mitral ring and Tirone David Surgery, there was clinical improvement, the left ventricular ejection fraction increased dramatically from 33% to 46%, the left ventricular end-diastolic volume decreased from 90 ml/m(2) to 77 ml/m(2) and the NT-proBNP decrease from 1100 pg/mL at 180 pg/mL. Instituto Nacional Cardiovascular - INCOR 2023-09-30 /pmc/articles/PMC10688408/ /pubmed/38046228 http://dx.doi.org/10.47487/apcyccv.v4i3.309 Text en https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License |
spellingShingle | Case Report Viñas-Mendieta, Adriana E. Cárdenas-Gallegos, Jesús K. Baltodano-Arellano, Roberto Chipa-Ccasani, Fredy Lévano-Pachas, Gerald C. Keirns, Candace Espinola-Zavaleta, Nilda Reversible cardiomyopathy in a patient with Marfan’s syndrome. Case report |
title | Reversible cardiomyopathy in a patient with Marfan’s syndrome. Case report |
title_full | Reversible cardiomyopathy in a patient with Marfan’s syndrome. Case report |
title_fullStr | Reversible cardiomyopathy in a patient with Marfan’s syndrome. Case report |
title_full_unstemmed | Reversible cardiomyopathy in a patient with Marfan’s syndrome. Case report |
title_short | Reversible cardiomyopathy in a patient with Marfan’s syndrome. Case report |
title_sort | reversible cardiomyopathy in a patient with marfan’s syndrome. case report |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10688408/ https://www.ncbi.nlm.nih.gov/pubmed/38046228 http://dx.doi.org/10.47487/apcyccv.v4i3.309 |
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