Related Experiment Videos
[Apical hypertrophic cardiomyopathy with right ventricular involvement]
F M Albanesi Filho1, M B Castier, J D Diamant
1Hospital Universitário Pedro Ernesto-UERJ.
Insights
Right ventricular involvement in apical hypertrophic cardiomyopathy (HCMP) indicates a worse prognosis. This condition presents differently from left ventricular disease, often leading to severe diastolic restriction and poorer outcomes.
Area of Science:
- Cardiology
- Cardiovascular Diseases
- Genetics
Background:
- Apical hypertrophic cardiomyopathy (HCMP) is a genetic heart muscle disease.
- Left ventricular involvement typically shows a good clinical evolution.
- Right ventricular (RV) involvement in HCMP is less understood.
Observation:
- Three patients (2 women, 1 man, aged 19-44) with apical HCMP and RV involvement were studied.
- Patients presented with severe diastolic restriction, significant atrial enlargement, and symptoms distinct from isolated left ventricular disease.
- Two patients experienced atrial fibrillation, leading to fatal thromboembolism.
Findings:
- Right ventricular involvement in apical HCMP is associated with a significantly worse prognosis compared to isolated left ventricular disease.
- RV involvement in apical HCMP does not present typical electrocardiographic changes.
- Clinical presentation includes ventricular restriction and tricuspid regurgitation.
Implications:
- Early identification of RV involvement in apical HCMP is crucial for risk stratification.
- Understanding RV involvement may lead to improved therapeutic strategies for managing apical HCMP.
- This finding highlights the importance of comprehensive cardiac assessment in apical HCMP patients.
Abstract:
We report right ventricular involvement in apical hypertrophic cardiomyopathy (HCMP) in two women and one man, aged from 19 to 44 years-old, who presented different signs from those with left ventricular disease (who have good clinical evolution), with signs and symptoms of severe diastolic restriction, showing great atrial enlargement and two with atrial fibrillation and death due to thromboembolism. The right ventricular involvement in apical HCMP leads to a worst prognosis than isolated left ventricular disease, does not have the typical electrocardiographic changes and presents with ventricular restriction and tricuspid regurgitation.