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MRI Characteristics, Prevalence, and Outcomes of Hypertrophic Cardiomyopathy with Restrictive Phenotype

Shuang Li1, Bailing Wu1, Gang Yin1

  • 1Department of Magnetic Resonance Imaging (S.L., B.W., G.Y., S.Z., M.L.), Key Laboratory of Cardiovascular Imaging (Cultivation), Chinese Academy of Medical Sciences, Beijing, China (M.L., G.Y.); Department of Cardiology (L.S.), Department of Echocardiography (Y.J.), and Heart-Lung Testing Center (J.H.), State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037, China; and Department of Radiology, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei, China (B.W.).

Radiology. Cardiothoracic Imaging
|March 29, 2021
PubMed

Insights

Hypertrophic cardiomyopathy with a restrictive phenotype is rare and linked to worse outcomes. MRI reveals enlarged atria and mild hypertrophy, indicating a poor prognosis for this HCM subtype.

Area of Science:

  • Cardiology
  • Cardiovascular Imaging
  • Medical Diagnostics

Background:

  • Hypertrophic cardiomyopathy (HCM) is a primary genetic heart muscle disease.
  • A restrictive phenotype within HCM presents unique diagnostic and prognostic challenges.
  • Understanding MRI characteristics is crucial for managing HCM subtypes.

Purpose of the Study:

  • To define the MRI features of hypertrophic cardiomyopathy with a restrictive phenotype.
  • To determine the prevalence and clinical outcomes associated with this HCM subtype.
  • To compare MRI findings and survival rates between restrictive HCM and control groups.

Main Methods:

  • Retrospective analysis of 2592 HCM patients to identify 34 with a restrictive phenotype.
  • Comparison with 34 age- and sex-matched HCM controls.
  • Utilized MRI for assessing cardiac dimensions, wall thickness, and myocardial fibrosis (late gadolinium enhancement).
  • Kaplan-Meier survival analysis was employed for outcome assessment.

Main Results:

  • The restrictive phenotype group showed significantly larger left and right atria compared to controls.
  • Maximum left ventricular wall thickness was lower in the restrictive group (16 mm ± 2 vs. 19 mm ± 3).
  • No significant difference in late gadolinium enhancement fraction was observed.
  • Five-year event-free survival was poorer in the restrictive group (81% vs. 94%).

Conclusions:

  • Restrictive phenotype is a rare HCM subtype associated with severe symptoms and a poor prognosis.
  • Key MRI findings include mild-to-moderate left ventricular hypertrophy, markedly enlarged atria, moderate fibrosis, and potential pericardial effusion.
  • These MRI characteristics aid in identifying patients with a worse clinical outlook.
Abstract

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