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 simp le transposition of the great arteries (TGA) after a Mustard operation . Background. Continuing medical follow-up for patients after the Must ard procedure, now extending to three decades, is required. The qualit y of life of adult survivors has not been well documented. Methods. Su rvival and quality of life among 113 hospital survivors of the Mustard operation performed for simple TGA between 1964 and 1982 were assesse d by medical review and a lifestyle questionnaire. The incidence of ri ght ventricular failure and echocardiographic right ventricular dysfun ction (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 Y ork Heart Association class 1, Sudden death, with an incidence of 7% w ithout identifiable risk factors, was the most common cause of late de mise. 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 sy mptoms or lifestyle modification, and 5% are unable to work. Conclusio ns. The survival of patients to 28 Sears with the Mustard repair has b een good. Late sudden death is the most worrisome feature. There is a 97% freedom from right ventricular failure to date, The quality of lif e of late survivors is good, most achieving a normal level of educatio n and employment. (J Am Coll Cardiol 1998;32:758-65) (C)1998 by the Am erican College of Cardiology.