A 78-year-old man was admitted to our hospital after an episode of unexplained syncope. A systolic murmur had previously been detected, and there was a history of cyanosis in his youth, which had prevented him from participation in competitive sports. His daughter had undergone surgery for tetralogy of Fallot at the age of 15 years. However, the patient had not undergone formal cardiologic evaluation until this admission. His medical history included hypertension, gout, psoriasis, and suspected pulmonary tuberculosis. He had been able to lead an active life, and he reported having had no other cardiac symptoms. His exercise tolerance was acceptable for his age.
On admission, clinical examination revealed that the patient had a plethoric complexion. There was no cyanosis, but clubbing of the fingers was evident. His pulse was 71 beats per minute and regular, and his blood pressure was 135/71 mm Hg; his oxygen saturation was 98% while he was breathing ambient air. On auscultation, there was a pansystolic murmur audible throughout the precordium. A prominent parasternal heave was detectable. There were no signs of congestive cardiac failure.
Electrocardiography showed sinus rhythm, right bundle-branch block, and right ventricular hypertrophy with secondary ST–T wave abnormalities. During ambulatory monitoring, the only arrhythmia was one three-beat episode of atrial fibrillation. The hemoglobin level was 17.5 g per deciliter, and the hematocrit was 54%. His electrolytes, renal function, and arterial-blood gases were normal. Chest radiography showed an enlarged cardiac silhouette, dilated pulmonary arteries, and a left-sided aortic arch. Cardiac magnetic resonance imaging was consistent with a diagnosis of balanced, uncorrected tetralogy of Fallot (Figure 1). There was no patent ductus arteriosus; however, both pulmonary arteries were dilated.
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