Scottish Adult Congenital and Cardiac Service, Golden Jubilee National Hospital, Beardmore Road, Clydebank, Dunbartonshire, United Kingdom.
J Thorac Cardiovasc Surg. 2013 Jun;145(6):1493-501; discussion 1501-3. doi: 10.1016/j.jtcvs.2013.02.030. Epub 2013 Mar 13.
The study objective was to evaluate long-term trends in morbidity and mortality in a national cohort of adult patients with a systemic right ventricle due to the atrial switch for transposition of the great arteries or congenitally corrected transposition of the great arteries.
We performed a retrospective cohort study from a baseline of 18 years, including life table and Kaplan-Meier analysis for probability of death/transplant, arrhythmia, surgical or percutaneous intervention, and permanent pacemaker insertion.
A total of 97 adults with transposition of the great arteries-atrial switch (Mustard procedure in 80/Senning procedure in 17) and 32 adults with congenitally corrected transposition of the great arteries survived. The median ages at latest follow-up were 29 and 34 years, respectively. At 40 years of follow-up, freedom from death or transplant was 0.90 for those with transposition of the great arteries-atrial switch and 0.84 for those with congenitally corrected transposition of the great arteries (P = .833). Freedom from arrhythmia at 40 years of follow-up was 0.51 for those with transposition of the great arteries-atrial switch and 0.93 for those with congenitally corrected transposition of the great arteries (P = .007). Freedom from intervention at 40 years of follow-up was 0.33 for those with transposition of the great arteries-atrial switch after initial repair and 0.53 for those with congenitally corrected transposition of the great arteries (P = .938). Freedom from pacemaker insertion at 40 years of follow-up was 0.77 for those with transposition of the great arteries-atrial switch and 0.62 for those with congenitally corrected transposition of the great arteries (P = .161).
Those patients who survive to adulthood with a systemic right ventricle experience low mortality and good functional status up to 40 years of age. However, there is a substantial burden of atrial tachyarrhythmia, and this occurs significantly earlier in those with transposition of the great arteries-atrial switch. Management of atrial tachyarrhythmia, along with systemic right ventricular dysfunction and systemic atrioventricular valve regurgitation, is likely to be the major challenge for this group of patients over the next decade.
本研究旨在评估因大动脉转位或矫正性大动脉转位行心房调转术而致右心系统发育不全的成年患者的长期发病率和死亡率趋势。
我们进行了一项回顾性队列研究，基线为 18 岁，包括寿命表和 Kaplan-Meier 分析，以评估死亡/移植、心律失常、手术或经皮介入及永久性起搏器植入的概率。
共纳入 97 例大动脉转位-心房调转术（Mustard 术 80 例，Senning 术 17 例）患者和 32 例矫正性大动脉转位患者存活。末次随访时的中位年龄分别为 29 岁和 34 岁。40 年随访时，大动脉转位-心房调转术患者的无死亡或移植生存率为 0.90，矫正性大动脉转位患者为 0.84（P=0.833）。40 年随访时，大动脉转位-心房调转术患者的无心律失常生存率为 0.51，矫正性大动脉转位患者为 0.93（P=0.007）。40 年随访时，初始修复后大动脉转位-心房调转术患者的无干预生存率为 0.33，矫正性大动脉转位患者为 0.53（P=0.938）。40 年随访时，大动脉转位-心房调转术患者的无起搏器植入生存率为 0.77，矫正性大动脉转位患者为 0.62（P=0.161）。
那些存活至成年期且右心系统发育不全的患者，40 岁时死亡率低且功能状态良好。然而，这些患者存在显著的房性快速性心律失常负担，且在大动脉转位-心房调转术患者中更早出现。在未来十年内，这群患者的主要挑战可能是房性快速性心律失常的管理，以及右心系统功能障碍和全身房室瓣反流。
