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Updated: Jun 28, 2026

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

Adriana E Viñas-Mendieta1, Jesús K Cárdenas-Gallegos2, Roberto Baltodano-Arellano1

  • 1Hospital Nacional Guillermo Almenara Irigoyen, Lima, Peru. Hospital Nacional Guillermo Almenara Irigoyen Lima Peru.

Archivos Peruanos De Cardiologia Y Cirugia Cardiovascular
|December 4, 2023
PubMed

Insights

Marfan syndrome patients can experience heart failure due to arrhythmias and aortic dilation. Successful treatment improved heart function and reduced key cardiac markers.

Area of Science:

  • Cardiovascular Medicine
  • Genetics
  • Connective Tissue Disorders

Background:

  • Marfan syndrome is a genetic disorder affecting connective tissue, leading to cardiovascular complications like aortic root dilation and non-ischemic cardiomyopathy.
  • Arrhythmias are a known complication, but their impact on heart function in Marfan syndrome is not well-documented.

Observation:

  • A 21-year-old male with Marfan syndrome presented with heart failure, frequent supraventricular arrhythmias, and significant aortic root dilation.
  • The patient underwent ablation for arrhythmias and aortic root repair surgery (Tirone David Procedure).

Findings:

  • Post-treatment, the patient showed significant clinical improvement.
  • Left ventricular ejection fraction increased from 33% to 46%.
  • Left ventricular end-diastolic volume decreased from 90 ml/m² to 77 ml/m², and NT-proBNP levels dropped from 1100 pg/mL to 180 pg/mL.

Implications:

  • This case highlights the potential benefits of combined arrhythmia ablation and surgical repair in managing heart failure in Marfan syndrome.
  • Aggressive management of cardiovascular manifestations can lead to substantial improvements in cardiac function and patient outcomes.
  • Further research is warranted to understand the prevalence and management strategies for arrhythmias in Marfan syndrome.
Keywords:
ArrhythmiasCase ReportHeart FailureMarfan’s Syndrome

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