Longitudinal follow-up of 32 to 39 years after Mustard correction of transposition of the great arteries

Abstract

Purpose: To describe mortality and morbidity in the total cohort of patients who underwent a Mustard operation for transposition of the great arteries (TGA) between 1971-1980 in our center and were followed prospectively every 10 years. Methods: Survival status was obtained from the Dutch National Population Registry. Patients who participated in one or both of the previous studies were invited for thorough in-hospital clinical investigation. Patients who could or would not participate were asked to fulfill a written questionnaire. Events were defined as death or heart transplantation, re-intervention, arrhythmia, heart failure, pacemaker or ICD implantation and endocarditis. Results: Information on survival status was obtained in 86 patients (95%). Mean follow-up was 35±2 years, with a range of 32-39 years. Cumulative survival was 80% after 20 years, 77% after 30 years and 68% after 37 years. Event free survival was 36%, 25% and 19% after 20, 30 and 37 years respectively. In the last 10 years 2 patients died due to ventricular fibrillation and 1 patient underwent heart transplantation, three patients (6%) underwent re-interventions for baffle stenosis, 5 (10%) had their first episode of supraventricular tachycardia, three (6%) needed a pacemaker (1 for sick sinus syndrome and 2 for AV-blok) and 3 (6%) received an ICD (1 for secondary and 2 for primary prevention), 5 patients (10%) were treated for de novo heart failure. Survival after Mustard operation for TGA Conclusion: Long-term outcome of Mustard patients is clearly hampered, with 68% survival and only 19% event-free survival after 37 years.