Severe left ventricular hypoplasia in patients with unbalanced incomplete atrioventricular septal defect and pulmonary hypertension: feasibility of biventricular repair.

We report three patients with partial atrioventricular septal defect who presented in the neonatal period with excessive left to right shunting and progressive pulmonary hypertension. Successful biventricular repair was accomplished despite the fact that left ventricular area and left sided structures did not meet the criteria established previously for biventricular management in children with critical aortic stenosis or complete atrioventricular septal defect. Indication for biventricular management was based on the fact that none of our patients had morphological mitral or aortic stenosis and that the hypoplastic left ventricle proved capable to maintain the systemic circulation following closure of the arterial duct. Fenestrated closure of the atrial septum and an individualized approach regarding the closure of the cleft in the left sided atrioventricular valve appear to be important surgical options to reduce postoperative left atrial pressure and to allow the ventricles to adapt to the new loading conditions.