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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...

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Autores principales: Viñas-Mendieta, Adriana E., Cárdenas-Gallegos, Jesús K., Baltodano-Arellano, Roberto, Chipa-Ccasani, Fredy, Lévano-Pachas, Gerald C., Keirns, Candace, Espinola-Zavaleta, Nilda
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Instituto Nacional Cardiovascular - INCOR 2023
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.
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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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