Long-term Follow-up (10 to 17 Years) After Mustard Repair for Transposition of the Great Arteries

Background: The management strategies of patients who underwent Mustard repair for transposition (of the great arteries were changed in the 1970s: infants became eligible for direct surgical repair, so Blalock-Hanlon atrioseptostomy could be avoided, and cold cardioplegia was introduced for myocardial preservation. Data are lacking, however, regarding whether these changes have had positive effects on the long-term outcome. We therefore conducted a follow-up study on all 91 patients who underwent a Mustard repair for transposition of the great arteries in our institution between 1973 and 1980 to assess the incidence and clinical importance of sequelae as well as health-related quality of life for these patients.

Methods: Patients who were alive and could be traced through local registrar's offices received an invitation to participate in the follow-up study, which consisted of an interview, physical examination, echocardiography, exercise testing, and standard 12-lead and 24-hour electrocardiography.

Results: Patients operated on in the first 4 years had a significantly higher mortality rate and higher incidence of sinus node dysfunction than did patients operated on in the subsequent 4 years (25% vs 2% and 41% vs 3%, respectively). In contrast, the incidence of baffle obstruction necessitating reoperation was significantly higher in the second group. There were no significant differences in echocardiographic findings and exercise capacity between patients operated on in the first 4 years and in the subsequent 4 years. None of the patients had right ventricular failure; a mild degree of baffle leakage or obstruction was seen in 22% of the patients, and the mean exercise capacity was decreased to 84% +/- 16% of normal.

Conclusion: The changes introduced between 1973 and 1980 have resulted in a considerable reduction of mortality and incidence of sinus node dysfunction but have also resulted in a more frequent need for reoperation.