The Prevalence and Long-Term Outcomes of Extreme Right versus Extreme Left Ventricular Hypertrophic Cardiomyopathy

Cardiology. 2016;133(1):35-43. doi: 10.1159/000439345. Epub 2015 Sep 29.

Abstract

Objectives: Extreme left ventricular hypertrophy (LVH) is a known risk factor for sudden cardiac death in hypertrophic cardiomyopathy (HCM). Extreme right ventricular hypertrophy (RVH) is rare, and whether it is linked to a poor outcome is unknown. This study was designed to investigate differences between HCM patients with extreme RVH and those with extreme LVH.

Methods: Among 2,413 HCM patients, 31 with extreme RVH (maximum right ventricular wall thickness ≥ 10 mm) and 194 with extreme LVH (maximum left ventricular wall thickness ≥ 30 mm) were investigated. The main clinical features and natural history were compared between the 2 groups.

Results: The prevalence of extreme RVH and extreme LVH was 1.3 and 8.0%, respectively. Patients with extreme RVH tended to be younger and female (p < 0.01). Cardiovascular-related mortality and morbidity within 10 years were significantly greater in the extreme RVH group (p < 0.05). Multivariate analysis demonstrated 3 independent predictors for cardiovascular mortality - extreme RVH, left ventricular end-diastolic dimension ≥ 50 mm, and age ≤ 18 years at baseline - and 2 for morbidity - extreme RVH and presyncope.

Conclusions: Compared with extreme LVH, extreme RVH was quite uncommon in HCM and had a worse prognosis. A right ventricle examination should be performed in routine HCM evaluation.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Cardiomyopathy, Hypertrophic / mortality*
  • Echocardiography
  • Electrocardiography, Ambulatory
  • Female
  • Heart Ventricles / physiopathology*
  • Humans
  • Hypertrophy, Left Ventricular / diagnosis*
  • Hypertrophy, Left Ventricular / drug therapy
  • Hypertrophy, Right Ventricular / diagnosis*
  • Hypertrophy, Right Ventricular / drug therapy*
  • Kaplan-Meier Estimate
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Multivariate Analysis
  • Prevalence
  • Prognosis
  • Proportional Hazards Models
  • Young Adult