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Volume 329:593-599 August 26, 1993 Number 9 Next

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ABSTRACT

Background Although corrective surgery for tetralogy of Fallot has been available for more than 30 years, the occurrence of late sudden death in patients in whom surgery was apparently successful remains worrisome.

Methods We studied long-term survival among 163 patients who survived 30 days after complete repair of tetralogy of Fallot, examining follow-up hospital records and death certificates when relevant.

Results The overall 32-year actuarial survival rate among all patients who survived surgery was 86 percent, as compared with an expected rate of 96 percent in a control population matched for age and sex (P<0.01). Thirty-year actuarial survival rates were calculated for the patient subgroups. The survival rates among patients less than 5 years old, 5 to 7 years old, and 8 to 11 years old were 90, 93, and 91 percent, respectively -- slightly less than the expected rates (P<0.001, P = 0.06, and P = 0.02). Among patients 12 years old or older at the time of surgery, the survival rate was 76 percent, as compared with an expected rate of 93 percent (P<0.001). The performance of a palliative Blalock-Taussig shunt procedure before repair, unlike the performance of a Waterston or Potts shunt procedure, was not associated with reduced long-term survival, nor was the need for a transannular patch at the time of surgery. Independent predictors of long-term survival were older age at operation (P = 0.02) and a higher ratio of right ventricular to left ventricular systolic pressure after surgery (P = 0.008). Late sudden death from cardiac causes occurred in 10 patients during the 32-year period.

Conclusions Among patients with surgically repaired tetralogy of Fallot, the rate of long-term survival after the postoperative period is excellent but remains lower than that in the general population. The risk of late sudden death is small.

Source Information

From the Division of Cardiovascular Diseases and Internal Medicine (J.G.M., B.J.G., M.D.M.), the Section of Pediatric Cardiology (D.D.M.), the Section of Biostatistics (D.M.I.), and the Division of Thoracic and Cardiovascular Surgery (D.C.M. [emeritus member], G.K.D.), Mayo Clinic and Mayo Foundation, Rochester, Minn.; the Division of Cardiology, Massachusetts General Hospital, Boston (V.F.); and the Department of Surgery, University of Alabama Medical Center, Birmingham (J.W.K.).

Address reprint requests to Dr. Murphy at the Mayo Clinic, 200 First St. SW, Rochester, MN 55905.

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