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A patient with hypertrophic cardiomyopathy accompanied by right ventricular dilation of unknown cause
K Suzuki1, K Hasegawa, R Nohara
1Department of Cardiovascular Medicine, Graduate School of Medicine, Kyoto University, Japan.
Insights
Hypertrophic cardiomyopathy (HCM) can progress to a dilated phase, resembling dilated cardiomyopathy. This case suggests right ventricular dilation may precede left ventricular changes in some HCM patients.
Area of Science:
- Cardiology
- Pathology
Background:
- Hypertrophic cardiomyopathy (HCM) is defined by unexplained ventricular hypertrophy.
- The dilated phase of HCM presents with ventricular dilation, thinning, and reduced ejection fraction, mimicking dilated cardiomyopathy.
Observation:
- A patient exhibited left ventricular concentric hypertrophy and right ventricular dilation of unknown etiology.
- Endomyocardial biopsy of the right ventricle revealed characteristic myocardial disarray.
Findings:
- The findings suggest a potential progression of hypertrophic cardiomyopathy (HCM) toward a dilated phase.
- Right ventricular dilation may be an early indicator in the transition to the dilated phase of HCM.
Implications:
- This case highlights the potential for right ventricular involvement in the early stages of hypertrophic cardiomyopathy (HCM).
- Understanding the progression of HCM, particularly the dilated phase, is crucial for patient management and diagnosis.
Abstract:
Hypertrophic cardiomyopathy (HCM) is a disease characterized by an unknown cause of hypertrophy in the left or right ventricle. The dilated phase of HCM shows disease conditions resembling dilated cardiomyopathy, such as ventricular dilation, thin ventricular wall, and reduction of the ejection fraction. A patient presented with left ventricular concentric hypertrophy accompanied by right ventricular dilatation of unknown cause. Right ventricular endomyocardial biopsy specimens showed characteristic myocardial disarray. Therefore, there is the possibility that the patient had right and left ventricular HCM in the process toward the dilated phase, in which dilatation first occurred in the right ventricle.