Correspondence

Tetralogy of Fallot in a 78-Year-Old Man

N Engl J Med 2007; 357:1160-1161September 13, 2007

Article

To the Editor:

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 1Figure 1Cardiac Magnetic Resonance Imaging in the Patient.). There was no patent ductus arteriosus; however, both pulmonary arteries were dilated.

Tetralogy of Fallot is the most common form of cyanotic congenital heart disease. First described in 1888, it consists of a ventricular septal defect, overriding aorta, obstruction of the right ventricular outflow tract, and right ventricular hypertrophy. Without surgical repair, survival past the fifth decade of life is rare but has been reported. The mechanisms for prolonged survival may be initially mild pulmonary stenosis that progresses1 or adaptations that ameliorate the right-to-left shunting, such as the formation of systemic–pulmonary collaterals,2 persistent patent ductus arteriosus,3 or systemic hypertension.4

Surgery for uncorrected tetralogy of Fallot in elderly patients is associated with increased mortality and is indicated only in symptomatic patients.5 We propose that a combination of progressive mild pulmonary stenosis, systemic hypertension, and mild erythrocytosis may have contributed to the longevity of our patient. At 81 years of age, he remains well, without further symptoms.

Badri Chandrasekaran, B.Sc., M.R.C.P.
Royal Brompton Hospital, London SW3 6NP, United Kingdom

Peter Wilde, M.R.C.P., F.R.C.R.
Bristol Royal Infirmary, Bristol BS2 H8W, United Kingdom

William A. McCrea, F.R.C.P.
Great Western Hospital, Swindon SN3 6BB, United Kingdom

5 References

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   Liberthson RR, Miller SW, Drew F, Palacios I, Singh J. Congenital extracardiac shunts with tetralogy of Fallot. Cardiovasc Intervent Radiol 1981;4:131-135
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   Sokol I, Vincelj J, Kirin M. Echocardiographic features of adult tetralogy of Fallot with natural palliative correction by patent ductus arteriosus. Croat Med J 2003;44:234-238
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   Hoffmann A, Gunthardt J, Gatzi H, Haller M. A 63-year-old man with uncorrected tetralogy of Fallot. Z Kardiol 1995;84:1039-1042
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   Dittrich S, Vogel M, Dahnert I, Berger F, Alexi-Meskishvili V, Lange PE. Surgical repair of tetralogy of Fallot in adults today. Clin Cardiol 1999;22:460-464
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