Related Experiment Videos
[A case of right ventricular dilated cardiomyopathy, which involved left ventricle]
K Ishikawa1, K Yokoyama, T Araki
1Department of Medicine, Yamagata Prefectural Central Hospital.
Insights
This case report details a rare form of right ventricular dilated cardiomyopathy (RVDC) that also affected the left ventricle in a young woman. The findings highlight the diverse presentations of cardiomyopathy.
Area of Science:
- Cardiology
- Cardiovascular Medicine
- Pathology
Background:
- Dilated cardiomyopathy (DCM) is a disease of the heart muscle that makes it harder to pump blood to the rest of the body.
- Right ventricular dilated cardiomyopathy (RVDC) is a specific form of DCM primarily affecting the right ventricle.
- This case presents an unusual involvement of both ventricles, suggesting an intermediate form of DCM.
Observation:
- A 16-year-old female presented with fatigue, cardiomegaly, and multifocal premature ventricular contractions (PVCs).
- Echocardiography revealed significant dilation of both the right ventricle (RV) and left ventricle (LV), with reduced ejection fractions.
- Cardiac catheterization and myocardial perfusion imaging showed no shunt disease but indicated impaired ventricular contractility and perfusion defects.
Findings:
- Endomyocardial biopsy confirmed marked hypertrophy, atrophy, myocyte disarray, and interstitial fibrosis in the right ventricle.
- Histopathological findings supported the diagnosis of RVDC with secondary left ventricular involvement.
- The patient exhibited LBBB-type PVCs, right axis deviation, low voltage, and T-wave changes on ECG.
Implications:
- This case illustrates the spectrum of expression in dilated cardiomyopathy, emphasizing that RVDC can present with significant left ventricular compromise.
- Understanding such intermediate forms is crucial for accurate diagnosis and management of complex cardiomyopathies.
- Further research into the genetic and environmental factors contributing to atypical DCM presentations is warranted.
Abstract:
A case of right ventricular dilated cardiomyopathy which also involved the left ventricle was reported. On health screening, a 16-year old woman was pointed out to have multifocal PVC and cardiomegaly. Subsequently, she was admitted to our hospital because of general fatigue. CTR was enlarged to 54.9% on chest X-ray. ECG showed LBBB-type PVC, right axis deviation, low voltage and T wave changes. On UCG, RVdD was dilated to 40 mm and LVdD was 37 mm. There was no finding of abnormality of the tricuspid valve. On cardiac catheterization, there was no shunt disease. Intracardiac pressure was normal. The end-diastolic volume index (ml/m2) of RV and LV was 196.7 and 67.4, respectively. And ejection fraction (%) was 20 and 40. Ventriculography revealed diffuse dilatation of the right ventricle. And lowered contractility existed not only in the right ventricle but also in the anterior and apical segment of the left ventricle. T(1)201 myocardial perfusion imaging showed irregular perfusion defect of the left ventricle. Endomyocardial biopsy revealed marked hypertrophy, partial atrophy, disarrangement of myocyte and interstitial fibrosis of the right ventricle. This case was considered to be right ventricular dilated cardiomyopathy. It seemed to be an intermediate form of dilated cardiomyopathy since it also involved the left ventricle. It was an interesting case to illustrate the spectrum of expression of cardiomyopathy.