Long-term outcome after the mustard repair for simple transposition of the great arteries - 28-year follow-up

Objectives. This study examines the late outcome in patients with simple transposition of the great arteries (TGA) after a Mustard operation. Background. Continuing medical follow-up for patients after the Mustard procedure, now extending to three decades, is required. The quality of life of adult survivors has not been well documented. Methods. Survival and quality of life among 113 hospital survivors of the Mustard operation performed for simple TGA between 1964 and 1982 were assessed by medical review and a lifestyle questionnaire. The incidence of right ventricular failure and echocardiographic right ventricular dysfunction (RVD) were determined. A measure of lifestyle, the ability index, was determined. Results. Actuarial survival was 90%, 80%, and 80% at 10, 20, and 28 years, respectively, with 76% of survivors being New York Heart Association class 1, Sudden death, with an incidence of 7% without identifiable risk factors, was the most common cause of late demise. RVD was identified in 18% of patients who had echocardiography, but there was right ventricular failure in only two patients. Seventy-five percent of current survivors lead a normal life, 20% have some symptoms or lifestyle modification, and 5% are unable to work. Conclusions. The survival of patients to 28 Sears with the Mustard repair has been good. Late sudden death is the most worrisome feature. There is a 97% freedom from right ventricular failure to date, The quality of life of late survivors is good, most achieving a normal level of education and employment. (J Am Coll Cardiol 1998;32:758-65) (C)1998 by the American College of Cardiology.