Ünlütürk Sahra, Kauling Robert M, Cuypers Judith A A E, van den Bosch Annemien E, Hirsch Alexander, Driessen Mieke M P, Pelosi Chiara, Bowen Daniel J, Bogers Ad J J C, Helbing Willem A, Kardys Isabella, Roos-Hesselink Jolien W
Department of Cardiology, Cardiovascular Institute, Thorax Center, Erasmus Medical Center, Rotterdam, the Netherlands.
Department of Cardiology, Cardiovascular Institute, Thorax Center, Erasmus Medical Center, Rotterdam, the Netherlands; European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERN GUARD-Heart), Amsterdam, The Netherlands.
JACC Adv. 2025 Jul 11;4(8):101984. doi: 10.1016/j.jacadv.2025.101984.
Concerns remain regarding the high incidence of morbidity in transposition of the great arteries (TGA) patients who underwent Mustard repair.
The purpose of this study was to evaluate the long-term outcome and quality of life of patients with TGA up to 48 years.
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.
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 VOmax. 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.
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.
对于接受Mustard修复术的大动脉转位（TGA）患者，其高发病率问题仍然令人担忧。
本研究旨在评估年龄达48岁的TGA患者的长期预后和生活质量。
单中心纵向队列研究，评估1973年至1980年间年轻时接受Mustard修复术的连续TGA患者，每十年进行广泛的心脏及主观生活质量评估。
在最初的91例患者队列中，31例死亡，2例接受心脏移植。48岁时的累积生存率为61%（51%至73%），随访35年后死亡原因从猝死转变为心力衰竭（HF）。在44例符合条件的幸存者中，34例（77%）接受了评估（71%为男性，中位年龄46岁），中位随访时间为46年（范围：40 - 48年）。无事件生存率为14%，心脏再次干预（39%）、症状性心律失常（39%）和HF（37%）是最常见的事件。体循环右心室功能（sRVF）随时间下降，73%的患者射血分数低于40%，95%的患者最大摄氧量降低。发现QRS时限延长是HF的预测指标，术后早期心律失常是晚期症状性心律失常的预测指标，sRVF降低是死亡率的预测指标。自我感知的生活质量良好且随时间保持稳定。
Mustard修复术后的死亡率和发病率很高，48岁时无事件生存率仅为14%。临床状况随时间恶化，需要及时考虑先进的治疗方案。
