Objective-To describe regional incidence, presentation, and outcome of idiopathic (familial) and Noonan syndrome related infant hypertrophic cardiomyopathy (HCM)
A complex case of congenital heart disease is presented which features isomerism of the left atrial appendages combined with bronchoatrial discordance and an anteriorly placed non-interrupted inferior caval vein draining to the left-sided atrium. This combination of anomalies has, as far as we are aware, not been described before. It emphasizes the importance of direct confirmation of atrial arrangement by atrial angiography or transesophageal echocardiography.
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