A 3-kg infant girl was delivered after a labor complicated by meconium-stained fluid and mild fetal distress. Apgar scores were 2 and 9. Initial examination showed decreased breath sounds over the right side. A grade 2/6 systolic ejection murmur was heard along the right parasternal border.
Other than mild circumoral cyanosis on exertion, the child has grown and developed normally to age 3 years.
Radioisotope pulmonary scanning during infancy showed virtually complete absence of perfusion and ventilation on the right side. A typical chest roentgenogram at 1 year of age is shown in Fig 1. A cardiac catheterization was performed and a right ventricular angiogram (Fig 2) was obtained at age 3 years.
Denouement and Discussion
The "Scimitar syndrome" is an uncommon familial syndrome characterized by hypoplasia of the right lung and right pulmonary artery, dextroposition of the heart, anomalous pulmonary venous drainage to the subdiaphragmatic inferior vena cava, and anomalous arterial supply