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Unexplained Left Ventricular Hypertrophy with Symptomatic High-Grade Atrioventricular Block in Elderly Patients: A

Tzu-Ping Yu1, Ju-Yi Chen1

  • 1Department of Internal Medicine, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan 704, Taiwan.

Journal of Clinical Medicine
|June 24, 2022
PubMed

Insights

Fabry disease (FD) can cause left ventricular hypertrophy (LVH) and heart block in older adults. Physiologic pacing is recommended over right ventricular pacing to prevent heart failure in these patients.

Area of Science:

  • Cardiology
  • Genetics
  • Internal Medicine

Background:

  • Left ventricular hypertrophy (LVH) is prevalent in older adults, with Fabry disease (FD) being a potential underlying cause, especially with a family history or specific symptoms.
  • Fabry disease is a rare genetic lysosomal storage disorder that can affect the heart, leading to cardiac complications like LVH and arrhythmias.

Observation:

  • A 76-year-old male presented with late-onset concentric LVH and symptomatic high-grade atrioventricular (AV) block.
  • Initial dual-chamber pacemaker implantation resulted in frequent right ventricular (RV) pacing, wide QRS duration, and subsequent heart failure with deteriorating LV systolic function.

Findings:

  • Pacing-induced cardiomyopathy (PICM) was suspected, leading to an upgrade to biventricular pacing.
  • Comprehensive investigations, including biochemical tests, cardiac biopsy, and genetic sequencing, confirmed a cardiac variant of Fabry disease.
  • The patient's presentation highlights the importance of considering FD in elderly patients with unexplained LVH and AV block.

Implications:

  • Physiologic pacing is strongly recommended as the initial pacing strategy for patients with FD and symptomatic high-grade AV block.
  • Avoiding traditional RV pacing may prevent the development of pacing-induced cardiomyopathy (PICM) and preserve LV systolic function in FD patients.
  • Early diagnosis and appropriate management, including tailored pacing strategies, are crucial for improving outcomes in cardiac Fabry disease.

Keywords:
Fabry diseaseelderlyhigh-grade atrioventricular blockunexplained hypertrophy

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