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Rat Model of Right-Sided Cardiac Remodeling and Arrhythmia Using Pulmonary Artery Banding
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Progressive Deterioration of the Right Ventricular Function in a Patient with Non-Obstructive HCM Complicated by

Jiamin Xu1,2, Changshou Tan3, Jianzhou Chen4

  • 1Cardiovascular Disease Center, The Central Hospital of Enshi Tujia and Miao Autonomous Prefecture, Enshi Clinical College of Wuhan University, Enshi, People's Republic of China.

International Medical Case Reports Journal
|July 14, 2025
PubMed

Insights

This case study details a rare instance of hypertrophic cardiomyopathy (HCM) with progressive right ventricular dysfunction. The findings highlight the importance of considering right heart involvement in HCM patients, even when uncommon.

Area of Science:

  • Cardiology
  • Genetics
  • Molecular Biology

Background:

  • Hypertrophic cardiomyopathy (HCM) is a common inherited heart disease affecting the left ventricle.
  • Right ventricular dysfunction is an uncommon manifestation of HCM.
  • This study focuses on a rare case of HCM with significant right ventricular impairment.

Observation:

  • A patient diagnosed with HCM via next-generation sequencing (NGS) developed atrial fibrillation.
  • The patient exhibited progressive right ventricular dysfunction, leading to peripheral edema and ascites.
  • This case presents non-obstructive HCM with declining right heart function.

Findings:

  • The patient was found to have a specific mutation, c.470C>T (p.Ala157Val), in the TNNI3 gene.
  • This genetic finding is linked to the observed hypertrophic cardiomyopathy and right ventricular dysfunction.
  • Next-generation sequencing facilitated the genetic diagnosis.

Implications:

  • This case underscores the need for thorough cardiac assessment, including right ventricular function, in HCM patients.
  • Understanding the genetic basis (TNNI3 mutation) may guide future diagnostic and therapeutic strategies.
  • The findings offer valuable insights for clinicians managing complex HCM cases.
Abstract

Keywords:
atrial fibrillationhypertrophic cardiomyopathynext generation sequencingright heart failure

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