Persistent Pulmonary Hypertension in a Neonate with Cystic Adenomatoid Malformation of the Lung following Lobectomy

Abstract

A full-term neonate is reported with congenital cystic adenomatoid malformation of the lung treated by lobectomy with development of pulmonary hypertension. The infant was successfully treated with extracorporeal membrane oxygenation (ECMO) for persistent pulmonary hypertension, which developed postoperatively. An 18-day course of veno-venous ECMO was necessary to effectively reverse the severe pulmonary hypertension. This was probably a result of significant pulmonary hypoplasia of the compressed lung. Although not all congenital cystic adenomatoid malformations of the lung are associated with pulmonary hypoplasia and persistent pulmonary hypertension, this is one case where severe pulmonary hypertension developed secondary to a mass effect by a large lesion in the chest.