Pulmonary Hypertension Caused by Medial Hypertrophy Associated With Aortic Stenosis and Preductal Coarctation

A 7-month-old female infant with aortic stenosis, preductal coarctation, and pulmonary hypertension underwent operation. Intraoperative lung biopsy revealed marked medial hypertrophy of the pulmonary arterioles. This histopathology is compatible with persistent pulmonary hypertension in the newborn. She is alive about 5 years after the operation, but pulmonary hypertension remains. The pathogenesis is discussed.