Search

Lung autopsies from four neonates with Ebstein's anomaly or tricuspid valve dysplasia and gross cardiomegaly were examined. The mean cardiothoracic ratio was 92 +/- 0.5%. The degrees of pulmonary hypoplasia and pulmonary artery muscularization were assessed and were compared with those in lungs from four controls and four patients with diaphragmatic hernias. Lung and body weight ratios and radial alveolar counts, which reflect pulmonary hypoplasia and immaturity, were significantly decreased only in patients with diaphragmatic hernia. The thickness of the medical muscle layer in small pulmonary arteries was greater in patients with diaphragmatic hernia; however, in patients with tricuspid valve disease, it was relatively small. Abnormal vascular muscle extension was seen only in patients with diaphragmatic hernia. In the cases of tricuspid valve disease, although cardiomegaly and lung compression were severe, lung hypoplasia and immaturity were not, and neither abnormal medial thickening nor extension were found. Our results suggest that, at least in full-term infants with tricuspid valve disease, surgical relief of lung compression may improve respiratory function, even if the cardiomegaly is severe.

A respirator-dependent 11-day-old boy with Ebstein's anomaly is presented. His cardiac anomaly with progressive cardiomegaly was first noticed at 24 weeks' gestation on fetal echocardiography. After birth, he required mechanical ventilation because of massive tricuspid regurgitation and restricted pulmonary blood flow. The operation of tricuspid valve patch closure, resection of right atrial wall, and central shunt successfully weaned him from the respirator on postoperative day 13. Cavopulmonary anastomosis was performed without problem when the patient was 8 months of age.