Direct Communication of a Pulmonary Artery with the Left Atrium

Abstract

Direct communication of a pulmonary artery with the left atrium is described and considered a variant of pulmonary arteriovenous fistula. A 3-year-old girl presented with clinical evidence of a lesion of the central nervous system. The history revealed that she had been cyanotic and had had clubbing of the digits and polycythemia. A rounded mass was noted in the region of the right pulmonary hilus in the posteroanterior thoracic roentgenogram. A pulmonary arteriovenous fistula was suspected, but the patient succumbed with a cerebral abscess before definitive diagnostic studies and therapy were undertaken. Anatomic examination revealed an unusual variant of pulmonary arteriovenous fistula, namely, direct communication of the right lower pulmonary artery with the left atrium and absence of the middle and lower lobes of the right lung. It is important that pulmonary arteriovenous fistulas be suspected clinically, since definitive diagnosis and therapy are now possible. While the hemodynamic effects of this type of lesion are usually not severe, untreated patients suffer a high morbidity and mortality through systemic arterial oxygen desaturation, paradoxic embolization, cerebral abscess, and rupture of the fistula.