Long-Term Outcome After Mustard Repair at Young Age: Longitudinal Follow-Up Into the Fifth Decade After Surgery

BACKGROUND: 

Concerns remain regarding the high incidence of morbidity in transposition of the great arteries (TGA) patients who underwent Mustard repair.

OBJECTIVES: 

The purpose of this study was to evaluate the long-term outcome and quality of life of patients with TGA up to 48 years.

METHODS: 

Single-center, longitudinal cohort study evaluating consecutive patients with TGA who underwent Mustard repair between 1973 and 1980 at young age with extensive cardiac and subjective quality of life evaluation every decade.

RESULTS: 

Of the original cohort of 91 patients, 31 died and 2 underwent heart transplantation. Cumulative survival at 48 years was 61% (51% to 73%), with a shift in cause of death from sudden death to heart failure (HF) after 35 years of follow-up. Of the 44 eligible survivors, 34 (77%) were evaluated (71% male, median age 46 years) with a median follow-up of 46 (range: 40-48) years. Event-free survival was 14%, with cardiac reintervention (39%), symptomatic arrhythmia (39%), and HF (37%) being the most common events. Systemic right ventricular function (sRVF) declined over time, with 73% having an ejection fraction below 40%, and 95% having a reduced VO2max. Prolonged QRS duration was found to be a predictor for HF, early postoperative arrhythmias for late symptomatic arrhythmias, and a reduced sRVF for mortality. Self-perceived quality of life was good and remained stable over time.

CONCLUSIONS: 

Mortality and morbidity after Mustard repair are substantial, with an event-free survival of only 14% at 48 years. The clinical condition deteriorated over time, necessitating timely consideration of advanced treatment options.