Three patients with pulmonary lymphangiectasis were studied by cardiac catheterization. All
had evidence of alveolar hypoventilation and pulmonary hypertension. Postmortem injection
studies of the abnormal lungs demonstrated greatly dilated and intracommunicating pleural,
interlobular, and perivascular lymphatics. Two of the patients had obstructed total
anomalous pulmonary venous return, while the third had a ventricular septal defect. In the
latter, a premortem diagnosis of pulmonary lymphangiectasis was made at 4 months of age …