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[Marked right ventricular outflow obstruction in two children with hypertrophic cardiomyopathy]
Insights
Hypertrophic cardiomyopathy in children can cause severe right ventricular outflow obstruction. Early diagnosis and management are crucial for improving outcomes in affected young patients.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Pathology
Background:
- Hypertrophic cardiomyopathy (HCM) is a genetic heart muscle disease.
- In pediatric cases, HCM can present with significant right ventricular outflow tract obstruction.
Observation:
- Two children with HCM exhibited severe right ventricular outflow obstruction.
- Echocardiography revealed asymmetrical septal hypertrophy and systolic anterior motion of the mitral valve.
- Cardiac catheterization confirmed significant pressure gradients in the left and right ventricular outflow tracts.
Findings:
- Angiocardiograms demonstrated severe right ventricular outflow obstruction and interventricular septal hypertrophy.
- Histological examination of resected tissue confirmed hypertrophic cardiomyopathy.
- One patient experienced sudden death at age 10, highlighting potential risks.
Implications:
- Pediatric hypertrophic cardiomyopathy often involves predominant right ventricular outflow tract obstruction.
- This condition can lead to progressive congestive heart failure and mortality.
- Accurate diagnosis and prompt, careful treatment are essential for managing pediatric HCM.
Abstract:
Two children, aged one year and nine months, and six years, both with hypertrophic cardiomyopathy, presented as severe right ventricular outflow obstruction were presented. Initially, both of them were suspected of having heart disease because of precordial systolic murmurs. Their tentative diagnosis was pulmonary stenosis. Their apex cardiograms and carotid pulse waves were not diagnostic. Asymmetrical septal hypertrophy with systolic anterior motion of the mitral valve was identified by echocardiography in both cases. Cardiac catheterization revealed left ventricular outflow tract pressure gradients of 5 mmHg and 30 mmHg, respectively. The right ventricular peak systolic gradient was 100 mmHg and 82 mmHg and left ventricular end-diastolic pressure was 26 mmHg and 18 mmHg, respectively. Selective right and left ventricular angiocardiograms demonstrated severe right ventricular outflow obstruction, and hypertrophy of the interventricular septum. One of them underwent resection of the right ventricular outflow tract muscle, which showed histological findings characteristic of hypertrophic cardiomyopathy. This patient died suddenly at the age of 10 years. Infants or young children with hypertrophic cardiomyopathy often have associated predominant right ventricular outflow tract obstruction, progressive congestive heart failure, and death. Therefore, correct diagnosis and careful treatment are mandatory for proper management.