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Published on: August 8, 2022
Right ventricular involvement in hypertrophic cardiomyopathy: Patterns and implications
Kalliopi Keramida1, George Lazaros1, Petros Nihoyannopoulos2
1Unit of Inherited Cardiovascular Diseases/Heart Center of the Young and Athletes, First Department of Cardiology, Hippokration General Hospital, National and Kapodistrian University of Athens, Greece.
Insights
Hypertrophic cardiomyopathy (HCM) often affects the right ventricle (RV), causing subtle yet significant changes. Routine RV assessment is crucial for managing HCM patients and improving prognosis.
Area of Science:
- Cardiology
- Genetics
- Pathology
Background:
- Hypertrophic cardiomyopathy (HCM) is a common inherited heart disease.
- Current diagnostic criteria and research predominantly focus on the left ventricle.
- Right ventricular (RV) involvement in HCM is often overlooked but clinically significant.
Purpose of the Study:
- To highlight the structural and functional changes in the right ventricle in HCM patients.
- To emphasize the prognostic implications of RV involvement.
- To advocate for routine inclusion of RV assessment in HCM evaluation.
Main Methods:
- Review of existing literature on RV involvement in HCM.
- Analysis of histological findings and pathogenetic mechanisms.
- Evaluation of echocardiographic indices, including global longitudinal strain.
Main Results:
- RV involvement in HCM presents with variable hypertrophy and potential dynamic obstruction.
- Subtle systolic dysfunction is detected by impaired global longitudinal strain, not classical indices.
- Diastolic dysfunction is evident, linked to fibrosis and impaired RV filling.
- RV involvement correlates with increased arrhythmias, heart failure, and sudden cardiac death risk.
Conclusions:
- Right ventricular changes are integral to HCM pathophysiology and prognosis.
- Standard echocardiography may underestimate RV dysfunction; advanced techniques like strain imaging are valuable.
- Comprehensive RV assessment is essential for accurate HCM diagnosis and management.
Abstract:
Although hypertrophic cardiomyopathy (HCM) is the most common inherited cardiomyopathy worldwide, the criteria for its definition and most of the literature concern the left ventricle, thus confirming the theory that the right ventricle is the neglected one. Right ventricular (RV) involvement includes structural and functional changes with significant impact on clinical presentation and prognosis. The pattern of RV hypertrophy can be variable with possible dynamic obstruction. Histological findings suggest similar pathogenetic changes in both ventricles supporting the common myopathic process with sarcomeric mutations. Systolic dysfunction of the RV is subtle, and the classical echocardiographic indices are usually within normal limits, while global longitudinal strain is significantly impaired. Diastolic dysfunction of the RV is also evident in patients with HCM possibly due to fibrosis of the RV free wall and/or the obstruction of the RV filling with significant prognostic implications. RV involvement in HCM is associated with increased incidence of supraventricular and ventricular arrhythmias, severe dyspnea, pulmonary thromboembolism, progressive heart failure, and increased risk of sudden cardiac death. Therefore, the RV should be routinely included in the detailed assessment of patients with HCM.
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