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We describe a rare type of atrioventricular septal defect with common atrioventricular orifice and malaligned atrial septum, the latter feature resulting in severe obstruction of the left atrial egress. Excision of the atrial septum and banding of the pulmonary trunk was performed at the age of thirteen months. Lung biopsy demonstrated marked medial hypertrophy with severe intimal fibrosis in the small pulmonary arteries and mild thickening of the pulmonary veins, considered contraindications for corrective surgery.

We describe a two-year-old boy suffering from pulmonary hypertension due to pulmonary venous stenosis. Previous reports suggest that it is not easy to diagnose this entity during life, and most cases are diagnosed at operation or autopsy. Our patient was diagnosed relatively early in life and treated successfully by pericardial patch venoplasty. Histologic examination of the lung biopsy performed at operation was considered to be useftil prognostically. The minimal pulmonary vascular changes noted were consistent with a good outcome after the surgical repair.

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Double outlet right atrium and complete atrioventricular septal defect with abnormal findings of the biopsied lung

Surgical repair of unilateral pulmonary venous stenosis

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2 results

Double outlet right atrium and complete atrioventricular septal defect with abnormal findings of the biopsied lung

Surgical repair of unilateral pulmonary venous stenosis