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Univentricular heart of right ventricular type: clinical, angiographic and electocardiographic features
Insights
Univentricular heart disease (UVHD) in patients with a right ventricular type presents with a main right ventricle and a rudimentary left ventricle. Angiography is crucial for diagnosing this complex congenital heart defect.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Imaging
Background:
- Univentricular heart (UVH) is a rare congenital anomaly.
- Right ventricular type UVH involves a dominant right ventricle with varying atrioventricular (A-V) connections.
- Accurate diagnosis and understanding of associated anomalies are critical for management.
Purpose of the Study:
- To describe the clinical, angiographic, and hemodynamic features of 18 patients with right ventricular type UVH.
- To identify key diagnostic criteria for this condition.
- To correlate clinical presentation with specific anatomical and hemodynamic findings.
Main Methods:
- Retrospective analysis of 18 patients diagnosed with right ventricular type UVH between 1971 and 1979.
- Sequential chamber analysis, electrocardiography (ECG), chest roentgenography, and hemodynamic assessments.
- Angiography was utilized for detailed anatomical evaluation.
Main Results:
- All patients had a main right ventricular chamber and a rudimentary left ventricular chamber without A-V connection.
- Common findings included double inlet (12 patients), absent A-V connections (4 right, 2 left), right ventricular hypertrophy on ECG, and variable QRS axis.
- Associated anomalies like pulmonary stenosis and persistent ductus arteriosus were frequent; clinical presentation varied based on pulmonary blood flow.
Conclusions:
- Right ventricular type UVH is characterized by a dominant right ventricle and a rudimentary left ventricle.
- Accurate diagnosis mandates angiographic demonstration of the posterior rudimentary chamber.
- Clinical presentation is diverse, influenced by pulmonary blood flow and associated cardiac defects.
Abstract:
The sequential chamber analysis and angiographic, hemodynamic and clinical features, including those seen on electrocardiography, are presented in 18 patients with a univentricular heart of right ventricular type. In all cases a main chamber with a right ventricular trabecular pattern received both (or the sole) atrioventricular (A-V) connections, and a rudimentary chamber with a left ventricular trabecular pattern receiving no A-V connection was also present. The 18 patients were seen between 1971 and 1979; 6 died and the diagnosis was confirmed at autopsy. Twelve patients had double inlet, four absent right and two absent left A-V connections. Electrocardiography revealed right ventricular hypertrophy in all, and 11 had a superior mean frontal QRS axis. Chest roentgenography disclosed dextrocardia in 7 and levocardia in 11. Of 12 infants presenting with a marked decrease in pulmonary flow, 10 presented as ill neonates with hypoxia and acidosis. Four infants with increased pulmonary flow presented before 8 weeks with heart failure and mild cyanosis. Two with moderate pulmonary stenosis had mild cyanosis, but were asymptomatic. All had associated cardiac anomalies, pulmonary stenosis and persistent ductus arteriosus being the most common. For accurate diagnosis, angiographic demonstration of a posterior rudimentary chamber in addition to the main chamber of right ventricular trabecular pattern is mandatory.