Scimitar syndrome with peripheral left pulmonary artery branch stenoses

Abstract

Partial anomalous pulmonary venous return most commonly involves the right pulmonary veins, with the site of connection most frequently the superior vena cava. Other sites of connection include the right atrium, inferior vena cava, superior vena cava plus right atrium, azygos vein, and coronary sinus.1-4 The pathologic features of a partial anomalous pulmonary venous drainage to the inferior vena cava associated with hypoplasia of the right lung were initially described by Parks in 1912. The term “scimitar syndrome” was coined by Neil1 et a1.2 in 1960 to describe the syndrome of partial anomalous pulmonary venous drainage of the right lung into the inferior vena cava, hypoplasia of the right lung with bronchial abnormalities and abnormal lobation, and partial systemic arterial supply to the right lung. Herein, we report a case of scimitar syndrome with an unusual associated vascular abnormality which presented a confusing clinical picture. A 19-year-old female with congenital heart disease was referred prior to a contemplated pregnancy for evaluation of a murmur. Her medical history was notable for pneumonia at the age of 4 years, at which time a heart murmur was first noted, and several recurrent bouts of pneumonia throughout her childhood and teenage years. She denied a history of cyanosis. Physical examination was notable for a prominent right ventricular heave and palpable third and fourth heart sounds. A loud grade IV/VI systolic murmur was heard, as well as a nearly pandiastolic murmur, both heard loudest over the entire left chest and only faintly audible over the right chest. The ECG revealed right axis deviation with right ventricular hypertrophy. The chest roentgenogram (Fig. 1) showed dextroposition of the heart and the typical scimitar configuration with a cardiothoracic ratio of 13.2122.6. The lung fields showed increased vascularity of the right lung. A lung scan showed increased perfusion of the right lung compared with that of the left (Fig. 2). Cardiac catheterization was carried out and revealed moderate elevation of right heart pressures. The main pulmonary artery pressure was 55/20 with a mean of 35 mm Hg. Pulmonary capillary wedge pressure was normal. Oximetry and green dye curves were compatible with partial anomalous right pulmonary venous return with an oxygen step-up in the inferior vena cava, 2 cm below its junction with the right atrium. An anomalous pulmonary vein emptying into the