Status of the adult and adolescent after repair of tetralogy of Fallot.

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Abstract
Because increasing numbers of patients with surgically repaired tetralogy of Fallot (TOF) are surviving to adulthood, follow-up data for physicians concerned with their care were compiled. The current status of 233 patients born before January 1962 who had repair of TOF at a median age of 9.7 yr (range 11 mo. to 36 yr) was reviewed. There were 26 surgical deaths (11.1%), 12 late cardiac-related deaths (5.9%) and 3 noncardiac deaths (1.3%). Of the 12 late deaths, 8 occurred suddenly and unexpectedly. In each of these 8 patients elevated right ventricular systolic pressure and ventricular premature depolarizations (VPD) were previously documented. Of 95 patients who responded to a questionnaire, 84 denied symptoms, 28 were married, 44 had attended college and all were employed. Cardiac catheterization revealed unsatisfactory hemodynamics in 39 of 120 patients, 30 of whom were asymptomatic. The poor results were due to a large left-to-right ventricular shunt in 8 patients, persistent right ventricular outflow obstruction in 25 patients, pulmonary vascular obstructive disease in 5 patients, and left ventricular cadiomyopathy in 1 patient. Among the 21 patients with VPD, 17 underwent cardiac catheterization: 15 had a right ventricular systolic pressure .gtoreq. 60 mm Hg and all had elevated right ventricular end-diastolic pressure. Clinical assessment alone is not predictive of hemodynamics and thus cardiac catheterization is indicated in all patients. The combination of persistent elevation of right ventricular systolic pressure above 60 mm Hg and VPD placed the patient at risk for sudden death. Over 80% of adults with repaired TOF can lead a normal life without impairment of intellect, exercise tolerance or fertility.

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