Staged Repair of Unilateral Absence of the Right Proximal Pulmonary Artery Using Autologous Azygos Vein Graft Interposition

Abstract

The isolated unilateral absence of a proximal pulmonary artery is a rare congenital lesion that presents various symptoms. Although some reports have shown one-stage reconstruction of the pulmonary circulation in infants, the two-stage approach is required in the situation of pulmonary arterial hypoplasia. In these cases, the usual approach is systemic pulmonary shunting for the first operation, to obtain growth of the pulmonary vascular bed, and then connecting bilateral pulmonary arteries for the second operation. Moreover, in the majority of patients without a right proximal pulmonary artery, some material is required for reconstructive surgery that corresponds to the patient's growth. A girl aged 2 years and 10 months with absent right proximal pulmonary artery, underwent modified Blalock-Taussig shunting with a free autograft of the azygos vein. The shunt was banded to prevent excessive pulmonary blood flow. Reconstructive surgery was performed 10 months after the first operation. At the second operation, growth of the right distal pulmonary artery and azygos autograft was satisfactory. Therefore, we used this autograft as an interposed graft of the right and main pulmonary arteries. Her postoperative course was uneventful. We advocate the usefulness of the azygos vein for graft material possessing the possibility of growth. This autograft is useful for pulmonary arterial reconstruction, such as the present case, and also may be useful for a systemic-pulmonary shunt, while growth is anticipated for more complex heart diseases.