Peripheral pulmonary artery stenosis (PPAS) is a rare form of congenital heart disease that is most frequently associated with Williams and Alagille syndromes. These patients typically have systemic level right ventricular pressures secondary to obstruction at the lobar, segmental, and subsegmental branches. The current management of patients with PPAS remains somewhat controversial. We have pioneered an entirely surgical approach for the reconstruction of PPAS. This approach initially entailed a surgical patch augmentation of all major lobar branches and effectively reduced the right ventricular pressures by more than half. This was the first report demonstrating an effective approach to this disease. Over the past 5 years, we have gradually evolved our technique of reconstruction to include segmental and subsegmental branch stenoses. An important technical aspect of this approach entails the 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 Heinecke-Miculicz-type ostioplasty for isolated ostial stenoses. The technical details of the surgical approach to PPAS are outlined in this article and can also be used for other complex peripheral pulmonary artery reconstructions.
Keywords: Congenital heart disease; Pulmonary arteries.
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