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Updated: Apr 11, 2026

Morphological and Functional Assessment of the Right Ventricle Using 3D Echocardiography
Published on: October 28, 2020
Left Ventricular Involvement in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy Assessed by
Thomas P Mast1, Arco J Teske1, Jeroen F vd Heijden1
1Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands.
Insights
Deformation imaging detects significant left ventricular involvement in arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C), identifying patients at higher risk for cardiac events. This advanced technique surpasses conventional echocardiography in assessing ARVD/C progression.
Area of Science:
- Cardiology
- Medical Imaging
- Genetics
Background:
- Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) phenotype studies show discrepancies in left ventricular (LV) involvement.
- The ability of echocardiographic techniques to detect LV involvement in ARVD/C is unclear.
- Accurate LV involvement detection may identify ARVD/C patients at increased risk for cardiac events.
Purpose of the Study:
- To evaluate conventional and deformation imaging for detecting LV involvement in ARVD/C.
- To determine if LV involvement detected by echocardiography predicts cardiac events in ARVD/C patients.
Main Methods:
- 38 ARVD/C patients, 16 mutation-positive relatives, and 55 controls underwent conventional and deformation echocardiography.
- Cardiac MRI with late enhancement was performed in a subgroup (n=27).
- All subjects were followed for cardiac events.
Main Results:
- Deformation imaging identified LV involvement in 68% of ARVD/C patients and 25% of relatives, correlating with MRI findings.
- Conventional echocardiography detected LV involvement in only 32% of ARVD/C patients.
- LV involvement by deformation imaging (HR 4.9) and RV outflow tract enlargement (HR 1.2) were independent predictors of adverse outcomes.
Conclusions:
- Deformation imaging is superior to conventional echocardiography for detecting LV involvement in ARVD/C.
- A high incidence of LV involvement exists in ARVD/C patients and their relatives.
- LV involvement and RV outflow tract enlargement are key prognostic markers in ARVD/C.
Background:
Among studies describing the phenotype of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C), significant discrepancy exists regarding the extent and impact of left ventricular (LV) involvement. The capability of conventional and new quantitative echocardiographic techniques to accurately detect LV involvement in ARVD/C remains unknown. The aim of this study was to test the hypothesis that accurate detection of LV involvement on echocardiography identifies patients at additional risk for cardiac events during follow-up.
Methods:
Thirty-eight patients with ARVD/C, 16 pathogenic mutation-positive relatives, and 55 healthy control subjects were prospectively enrolled. Conventional echocardiography with additional deformation imaging was performed in all subjects to detect LV involvement. In a subgroup (n = 27), cardiac magnetic resonance imaging was performed with late enhancement. All patients and relatives were prospectively followed for events (sustained ventricular tachycardia, appropriate implantable cardioverter-defibrillator intervention, sudden cardiac death, and heart transplantation).
Results:
Conventional echocardiography detected LV involvement in 32% of patients with ARVD/C and in none of the relatives. Deformation imaging revealed LV involvement in 68% of patients with ARVD/C and 25% of relatives and was correlated closely with late enhancement on cardiac magnetic resonance imaging. During a mean follow-up period of 5.9 ± 2.3 years, 20 patients with ARVD/C (53%) experienced events, and no events occurred in the relatives. LV involvement detected by deformation imaging (hazard ratio, 4.9; 95% CI, 1.7-14.2) and right ventricular outflow tract enlargement (hazard ratio, 1.2; 95% CI, 1.1-1.3) were the only independent predictors of outcomes.
Conclusions:
Deformation imaging detected a high incidence of LV involvement in patients with ARVD/C and their relatives. Compared with conventional echocardiography, deformation imaging is superior in detecting minor LV involvement. LV involvement and an enlarged right ventricular outflow tract are independent prognostic markers of outcomes.
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