A 20 year old refugee presents with recurrent episodes of exertional dyspnea, palpitations and easy fatiguability since childhood. He had not undergone evaluation in his native country. His family history is unremarkable, and he is not on any medications.
WBC/DC: 7,000/mm3 Hb: 18.2 g/dl Hct (PCV): 60% Platelets: 300,000/mm3
The x-ray reveals oligemic lung fields and a boot-shaped heart.
The ECG shows P-pulmonale, right axis deviation, and a right bundle branch block.
The findings include a 2 cm membranous VSD with a right to left shunt; a 45% aortic override with a normal aortic diameter; right ventricular hypertrophy, right atrial dilatation and infundibular stenosis; a small left ventricle; and an ejection fraction of 56%.