Late gadolinium enhancement confined to the right ventricular insertion points in hypertrophic cardiomyopathy: an

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Paco E Bravo1, Hong-Chang Luo2, Iraklis Pozios2

  • 1Department of Cardiology, University of Washington, 1959 NE Pacific Street, PO Box 356422, Seattle, WA 98195, USA Johns Hopkins Medical Institutions, Baltimore, MD, USA pbravo@uw.edu.

European Heart Journal. Cardiovascular Imaging
|June 17, 2015
PubMed

Insights

Hypertrophic cardiomyopathy (HCM) patients with right ventricular insertion point (RVIP) late gadolinium enhancement (LGE) show intermediate cardiac function and structure. This RVIP LGE phenotype is distinct from no LGE or left ventricular intramural LGE.

Area of Science:

  • Cardiology
  • Medical Imaging
  • Cardiovascular Disease

Background:

  • Hypertrophic cardiomyopathy (HCM) is a complex genetic heart disease.
  • Late gadolinium enhancement (LGE) on cardiac MRI is a key imaging biomarker in HCM.
  • The distribution of LGE may correlate with disease severity and phenotype.

Purpose of the Study:

  • To compare phenotypic characteristics of HCM patients with LGE confined to right ventricular insertion points (RVIP) versus those with no LGE or left ventricular (LV) intramural LGE.
  • To investigate the potential of RVIP LGE as an intermediate phenotype in HCM.

Main Methods:

  • Cardiac MRI for LGE quantification and LV thickness measurement in 62 HCM patients.
  • Classification into three groups: no-LGE (n=18), LGE-RVIP (n=19), and intramural LGE (n=25).
  • N-13 ammonia PET for myocardial blood flow (MBF) and myocardial flow reserve (MFR) assessment; echocardiography for LV longitudinal strain.

Main Results:

  • LGE extent and LV thickness were significantly higher in the intramural LGE group, intermediate in the LGE-RVIP group, and lowest in the no-LGE group.
  • Myocardial blood flow (MBF), myocardial flow reserve (MFR), and LV longitudinal strain were significantly reduced in the intramural LGE group, intermediate in the LGE-RVIP group, and highest in the no-LGE group.
  • These findings indicate distinct functional and structural differences across the LGE patterns.

Conclusions:

  • HCM patients with LGE restricted to RVIP represent an intermediate phenotype.
  • This intermediate phenotype lies between patients with no LGE and those with LV intramural LGE.
  • RVIP LGE may signify a distinct stage in HCM progression, identifiable through advanced cardiac imaging.
Abstract

Keywords:
CMRLGERV insertion points

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