Left ventricular cine-angiocardiography in endocardial cushion defect.

Endocardial cushion defects are complex mal-formations with a wide clinical spectrum. The abnormalities range from a small defect of the atrial septum in the position of the ostium primum, with an abnormal cleft mitral valve, to more extensive abnormalities which include tricuspid incompetence and defects of the ventricular septum. In the most severe form a common atrioventricular canal occurs. The pathology determines the clinical and radio-In general, the defects can be divided into two clinical groups (Barnard and Schrire, 1961). Endo-cardial cushion defects, without a significant ventricular septal defect, consist of partial absence of the lower portion of the atrial septum and a cleft mitral valve. A small insignificant ventricular septal defect or cleft tricuspid valve may be present. In defects with a significant ventricular septal defect, the ventricular septal defect dominates the clinical picture, producing a large left-to-right shunt and often severe pulmonary arterial hyper-tension (Schrire et al., 1963; Schrire and Vogelpoel, 1964). The electrocardiogram usually is diagnostic: it shows left axis deviation (-30° to-1200), a counterclockwise or figure-of-eight vector loop in the frontal plane, incomplete right bundle-branch block, and often a long P-R interval (it is undertaken with care, may not add much to the clinical findings. The presence of an endocardial cushion defect without a significant ventricular septal defect is confirmed by demonstrating a large left-to-right shunt at atrial level, normal or sub-normal pulmonary vascular resistance, typical low passage of the catheter across the defect, and very similar drainage pathways of the pulmonary veins from each lung (Swan et al. Defects with a significant ventricular septal defect have the haemodynamic pattern of a large combined ventricular and atrial septal defect, often with severe pulmonary arterial hypertension. Assessment of the degree of mitral and tricuspid incompetence may be difficult. Recently the value of selective left ventriculo-We have undertaken left ventricular cine-angiocardiography routinely and have confirmed that certain features occur only in patients with an endocardial cushion defect. This paper analyses the left ventricular cine-angiocardiogram in 13 patients and relates the findings to the abnormal embryology and anatomy. RESULTS The ages of the 13 patients ranged from 6 months to 45 years and 12 were children under the age of 15. The diagnosis was confirmed at operation in 10. These 10 had a low defect of the atrial septum and a cleft mitral valve. Five also had a cleft tricuspid