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

Abstract

Because increasing numbers of patients with surgically repaired tetralogy of Fallot (TOF) are surviving to adulthood, this study was done to provide follow-up data for physicians concerned with their care.We reviewed the current status of 233 patients born before January 1962 who had repair of TOF at a me- dian age of 9.7 years (range 11 months to 36 years).There were 26 surgical deaths (11.1%), 12 late cardiac- related deaths (5.9%) and three noncardiac deaths (1.3%).Eight of the 12 late deaths occurred suddenly and unexpectedly.In each of these eight patients we had previously documented elevated right ventricular systolic pressure as well as ventricular premature depolarizations (VPDs).Among the 95 patients who responded to a questionnaire, 84 denied symptoms, 28 were married, 44 had attended college and all were employed.Cardiac catheterization revealed an unsatisfactory hemodynamic result in 39 of 120 patients, 30 of whom were asymptomatic.The poor result was due to a large left-to-right ventricular shunt in eight patients, persistent right ven- tricular outflow obstruction in 25 patients, pulmonary vascular obstructive disease in five patients, and left ven- tricular cardiomyopathy in one patient.Among the 21 patients with VPDs, 17 underwent cardiac catheterization: 15 had a right ventricular systolic pressure > 60 mm Hg and all had elevated right ventricular end-diastolic pressure.We conclude that 1) clinical assessment alone is nonpredictive of the hemodynamic result and thus cardiac catheterization is indicated in all patients; 2) the combination of persistent elevation of right ventricular systolic pressure above 60 mm Hg and VPDs placed the patient at risk for sudden death; and 3) over 80% of adults with repaired TOF can lead a normal life without impairment of intellect, exercise tolerance or fertility.IN THE UNITED STATES each year an estimated 24,500 newborns have a congenital heart defect.1 2 Approximately 1%, or 2700, have tetralogy of Fallot (TOF).3-5Based on current surgical treatment, we esti- mate that 89% of these patients, or 2400 per year, will survive to adulthood.The purposes of this study were: 1) to present the characteristics of these patients; 2) to determine if symptomatology, physical examination and laboratory findings are adequate predictors of the postoperative hemodynamic result; and 3) to examine the late complications and causes of late death in an effort to predict and prevent these occurrences by identifying patients at risk. Materials and Methods DefinitionPatients were included if they had typical anatomy of TOF with a nonrestrictive ventricular septal defect and pulmonary stenosis with essentially equal peak systolic pressure in two ventricles.Patients were excluded from the study if they had preoperative right ventricular outflow or main pulmonary artery atresia