Total Correction of Tetralogy of Fallot

Abstract

One hundred and forty-six consecutive patients underwent open-heart surgery for correction of tetralogy of Fallot at The Johns Hopkins Hospital from September, 1956 to January, 1962. Forty-two died in the early postoperative period and 104 were discharged from the hospital alive. Three late deaths have occurred after discharge from the hospital; all of these deaths occurred in patients with small residual ventricular septal defects. The major factors that influenced the immediate mortality included cyanosis and degree of disability, both of which correlated with the severity of the pulmonic stenosis. More than one previous shunt procedure, a Pott''s anastomosis, the presence of severe associated defects, or an outflow patch in young patients under 10 years of age, were all associated with an unfavorable prognosis. Of the 104 patients in the follow-up group, 41 have been followed for less than 1 year, 52 from between 1 and 3 years, and 11 between 3 and 5 years. Complete follow-up data including X-rays were available in 98. Systolic murmurs persisted in all but 8 patients. Pulmonary valvular insufficiency was present in 59 patients and was twice as frequent in those with right ventricular outflow patches. Of the 101 patients living at completion of the study, 37 have normal cardiothoracic ratios and no residual defects. Thirty-six patients are clinically well with slight cardiac enlargement. Twenty-two patients are clinically asymptomatic but have residual cardiac defects including small residual ventricular defects in 8, aortic insufficiency in 5, and complete heart block in 2. Despite the residual cardiac defects, all of the patients have had symptomatic improvement, and in many this improvement has been dramatic.