Reversible cardiomyopathy in a patient with Marfan’s syndrome. Case report

Adriana E. Viñas-Mendieta; Jesús K. Cárdenas-Gallegos; Roberto Baltodano-Arellano; Fredy Chipa-Ccasani; Gerald C. Lévano-Pachas; Candace Keirns; Nilda Espinola-Zavaleta

doi:10.47487/apcyccv.v4i3.309

Autores/as

Adriana E. Viñas-Mendieta Hospital Nacional Guillermo Almenara Irigoyen, Lima, Peru. https://orcid.org/0000-0003-1018-8695
Jesús K. Cárdenas-Gallegos Cardiologist. Hospital Nacional Dos de Mayo, Lima, Peru. https://orcid.org/0000-0002-1802-433X
Roberto Baltodano-Arellano Hospital Nacional Guillermo Almenara Irigoyen, Lima, Peru. https://orcid.org/0000-0002-7538-2125
Fredy Chipa-Ccasani Hospital Nacional Guillermo Almenara Irigoyen, Lima, Peru. https://orcid.org/0000-0001-9914-946X
Gerald C. Lévano-Pachas Hospital Nacional Guillermo Almenara Irigoyen, Lima, Peru. https://orcid.org/0000-0002-4136-5802
Candace Keirns Shelby County Health Department, Memphis, TN, USA. https://orcid.org/0000-0001-5208-1525
Nilda Espinola-Zavaleta National Institute of Cardiology Ignacio Chavez. ABC Medical Center, P.A.I. Observatorio, Mexico City, Mexico. https://orcid.org/0000-0002-4136-5802

DOI:

https://doi.org/10.47487/apcyccv.v4i3.309

Palabras clave:

Marfan’s Syndrome, Arrhythmias, Heart Failure, Case Report

Resumen

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/m2 to 77 ml/m2 and the NT-proBNP decrease from 1100 pg/mL at 180 pg/mL.

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