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The angiocardiographic appearance of the endocardial cushion defect in selected transposition and malposition
Insights
Endocardial cushion defects (ECD) show a characteristic "gooseneck" deformity on angiography, even in complex congenital heart disease. This finding is crucial for surgical planning in patients with single ventricle or double outlet right ventricle.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Endocardial cushion defect (ECD) is a congenital heart anomaly.
- Angiocardiographic features of ECD are well-documented in simple heart defects.
- Limited data exists on ECD appearance in complex cardiovascular disease.
Observation:
- Two patients with complex congenital heart disease and ECD were studied.
- One patient had D-loop, single left ventricle, D-transposition of great arteries, and pulmonary stenosis.
- The other had dextrocardia, situs inversus, double outlet right ventricle, common atrioventricular canal, and pulmonary stenosis.
Findings:
- Left ventricular angiography revealed a "gooseneck" deformity in both patients.
- This occurred despite aortic-mitral discontinuity and complex cardiac anomalies.
- Elongation, scalloping, and serration characterized the left ventricular outflow tract.
Implications:
- The "gooseneck" deformity in ECD can manifest even with complex congenital heart disease.
- Accurate angiocardiographic assessment of ECD is vital for surgical management.
- This study expands understanding of ECD presentation in intricate cardiac conditions.
Abstract:
Although the angiocardiographic features of the endocardial cushion defect (ECD) have been well described in the patient with two normally related ventricles (D-ventricular loop in situs solitus) and normally related great arteries, little attention has been focused on the angiocardiographic appearance of the ECD in patients with complex cardiovascular disease. Because of recent surgical advances in the treatment of single ventricle and double outlet right ventricle complexes, it has become increasingly important to document the status of the atrioventricular valves in these patients. The angiocardiographic features of the ECD are therefore described in two patients, one with D-loop, single left ventricle and outlet chamber, D-transposition of the great arteries, bilateral conus and pulmonary stenosis and the other with dextrocardia, situs inversus, double outlet right ventricle, common atrioventricular canal and pulmonary stenosis. In both patients, the left ventricular outflow tract, despite aortic-mitral discontinuity, is characterized by elongation, scalloping and serration, and the classic appearance of the "gooseneck" deformity is observed on left ventricular angiography. It thus appears that aortic-mitral continuity and two well-formed, normally related ventricles and great arteries are not invariable necessary to the manifestation of the "gooseneck" deformity in the patient with complex cardiovascular disease and associated endocardial cushion defect.