Surgical Techniques for Repair of Peripheral Pulmonary Artery Stenosis

Peripheral pulmonary artery stenosis is a rare form of congenital heart disease frequently associated with Williams and Alagille syndromes. Patients with this disease typically have systemic level right ventricular pressures secondary to obstruction at the lobar, segmental, and subsegmental branches. The current management of patients with peripheral pulmonary artery stenosis remains somewhat controversial. We have pioneered an entirely surgical approach to the reconstruction of peripheral pulmonary artery stenosis. This approach initially entailed surgical patch augmentation of all major lobar branches and effectively reduced right ventricular pressures by more than half. This was the first report demonstrating an effective approach to the disease. Over the past 5 years, we have gradually evolved the technique to extend the reconstruction's reach to include segmental and subsegmental branch stenoses. An important technical aspect of this approach entails division of the main pulmonary and separation of the branch pulmonary arteries to access the lower lobe branches. Pulmonary artery homograft patches are used to augment hypoplastic pulmonary artery branches. In addition, we perform a Heineke-Mikulicz type ostioplasty for isolated ostial stenoses. The technical details of the surgical approach to peripheral pulmonary artery stenosis are outlined in this article, and can also be used for other complex peripheral pulmonary artery reconstructions.