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Updated: May 9, 2026

Morphological and Functional Assessment of the Right Ventricle Using 3D Echocardiography
07:11

Morphological and Functional Assessment of the Right Ventricle Using 3D Echocardiography

Published on: October 28, 2020

Mutation-positive arrhythmogenic right ventricular dysplasia/cardiomyopathy: the triangle of dysplasia displaced

Anneline S J M Te Riele1, Cynthia A James, Binu Philips

  • 1Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands; Department of Medicine, Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.

Journal of Cardiovascular Electrophysiology
|July 30, 2013
PubMed

Insights

Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) involves characteristic RV and LV abnormalities, challenging the traditional Triangle of Dysplasia. The RV apex is affected late in ARVD/C progression.

Area of Science:

  • Cardiology
  • Genetics
  • Medical Imaging

Background:

  • The traditional Triangle of Dysplasia model for Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) predates genetic advancements and does not fully encompass biventricular disease patterns.
  • Genetic testing has identified a broader spectrum of ARVD/C phenotypes beyond the classic right ventricular (RV) involvement.

Purpose of the Study:

  • To characterize the regional distribution of cardiac abnormalities in mutation-positive ARVD/C patients using Cardiac Magnetic Resonance (CMR) and electroanatomic mapping.
  • To reassess the anatomical boundaries of the "Triangle of Dysplasia" in light of genetic findings and biventricular disease.

Main Methods:

  • Analysis of CMR studies in 74 mutation-positive ARVD/C patients using 5-segment RV and 17-segment LV models.
  • Correlation of CMR findings with electroanatomic mapping data (scar location, VT ablation sites) in 11 ARVD/C subjects.

Main Results:

  • 73% of patients showed CMR abnormalities, with RV involvement in 96% and biventricular involvement in 52%.
  • The most common RV abnormalities were basal inferior and anterior wall dyskinesis. The most frequent LV abnormality was posterolateral subepicardial fat infiltration.
  • Electoanatomic mapping confirmed scar in the RV basal inferior/anterior walls and LV posterolateral wall, correlating with Ventricular Tachycardia (VT) origins.

Conclusions:

  • Mutation-positive ARVD/C presents a distinct pattern of disease affecting the basal RV and posterolateral LV, expanding beyond the traditional "Triangle of Dysplasia."
  • The RV apex is involved late in the disease, usually with global RV dysfunction, and should be reconsidered in the ARVD/C dysplasia model.
  • These findings offer new insights into ARVD/C pathophysiology and disease distribution.
Abstract

Keywords:
arrhythmogenic right ventricular dysplasia/cardiomyopathyelectroanatomic mappinggeneticsimplantable cardioverter defibrillatormagnetic resonance imagingphenotypeventricular tachcardia

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