CASE 13
An 11-month-old female infant with cyanosis was driven to the emergency room by her parents. The physical examination revealed clubbing of the digits and that the infant was underdeveloped. Upon palpation, the physician detected a right ventricular impulse and thrill along the left sternal margin. Another finding was an audible systolic murmur over the pulmonary area. Hypoxemia and polycythemia were notable laboratory findings. Examination of the radiographs showed right ventricular hypertrophy, a narrow shadow of the great vessels, and a boot-shaped heart (coeur en sabot). Cardiac catheterization and angiocardiography showed stenosis of the right ventricular outflow track, right to left ventricular shunting of blood flow, diminished caliber of pulmonary trunk and pulmonary arteries, and normal morphology of the coronary arteries. The infant was diagnosed with tetralogy of Fallot and the congenital defects were surgically corrected.
