Right Lung Agenesis with Left Pulmonary Artery Sling

We report on a 2-month-old infant girl who had right pulmonary agenesis and an unusual course of the left pulmonary artery. Computed tomography and cardiac catheterization showed that the left pulmonary artery arose from the main pulmonary artery, crossing the midline, and reaching the left lung via an aberrant course between the esophagus and trachea. The coexistence of right pulmonary agenesis and left pulmonary sling is extremely rare. Unlike in other reports, our patient remained symptom-free and in good health, with normal growth and development until age 2 years, when she died from complications during an attack of bronchiolitis caused by respiratory syncytial virus. Pediatr Pulmonol, 2000; 29:239-241. (C) 2000 Wiley-Liss, Inc