Kadowaki Sachiko, Venet Maelys, Tocharoenchok Teerapong, Yap Kok Hooi, Fan Chun-Po Steve, Zahiri Yasmin, Deng Mimi X, Villemain Olivier, Floh Alejandro, Barron David J, Honjo Osami
Division of Cardiovascular Surgery The Hospital for Sick Children Toronto ON Canada.
Department of Surgery University of Toronto Toronto ON Canada.
J Am Heart Assoc. 2025 May 6;14(9):e037348. doi: 10.1161/JAHA.124.037348. Epub 2025 Apr 23.
This study aimed to assess impact of atrioventricular valve (AVV) regurgitation onset, timing of AVV repair (AVVr), ventricular morphology, and era effect on AVVr outcomes in a single ventricle population.
A retrospective review of 155 patients with single ventricle physiology who underwent AVVr between 1998 and 2022 was conducted. Transplant-free survival, discharge alive, and AVV reoperation were assessed using the Kaplan-Meier survival method, stratified by the timing of AVVr (Group1 [G1], prebidirectional cavopulmonary shunt, N=33; G2, at or post-shunt, N=93; G3, at or post-Fontan, N=29). Cox proportional hazard models were used to assess the association of the timing of AVVr with death or transplant.
Transplant-free survival at 10 years was lowest in G1 (G1, 16% [95% CI, 4%-35%]; G2, 65% [95% CI, 53%-74%]; G3, 85% [95% CI, 65%-94%], <0.001). In the multivariate analysis, AVVr prebidirectional cavopulmonary shunt was an independent risk factor for failure to be discharged alive (<0.001) but not for overall survival (=0.12). Meanwhile, the likelihood of discharge alive improved over the period in the entire cohort (<0.001), and right ventricle morphology (=0.02) and weight <5 kg (<0.01) at AVVr were significantly associated with death. In the multistate model, persistent or recurrent AVV regurgitation and ventricular dysfunction post-sAVVr were significantly associated with death, with hazard ratios of 3.8 (95% CI, 2.0-7.3, <0.001) and 32 (95% CI, 13-77, <0.001), respectively.
Patients with single ventricles who required AVVr, particularly before bidirectional cavopulmonary shunt, have poorer transplant-free survival with no meaningful improvement over the past 2 decades. Small weight and morphologic right ventricle were strongly associated with increased mortality. Alternative treatment strategies should be considered for this high-risk subgroup.
本研究旨在评估房室瓣（AVV）反流的起始、房室瓣修复（AVVr）的时机、心室形态以及时代效应对单心室人群AVVr结局的影响。
对1998年至2022年间接受AVVr的155名单心室生理患者进行回顾性研究。采用Kaplan-Meier生存法评估无移植生存、出院存活情况以及AVV再次手术情况，并根据AVVr的时机进行分层（第1组[G1]，双向腔肺分流术前，N = 33；第2组，分流时或分流后，N = 93；第3组，Fontan手术时或Fontan手术后，N = 29）。使用Cox比例风险模型评估AVVr时机与死亡或移植之间的关联。
G1组10年无移植生存率最低（G1组，16%[95%CI，4%-35%]；G2组，65%[95%CI，53%-74%]；G3组，85%[95%CI，65%-94%]，P<0.001）。在多变量分析中，双向腔肺分流术前进行AVVr是未能出院存活的独立危险因素（P<0.001），但不是总体生存的危险因素（P = 0.12）。同时，整个队列在此期间出院存活的可能性有所改善（P<0.001），AVVr时右心室形态（P = 0.02）和体重<5 kg（P<0.01）与死亡显著相关。在多状态模型中，持续性或复发性AVV反流以及AVVr后心室功能障碍与死亡显著相关，风险比分别为3.8（95%CI，2.0-7.3，P<0.001）和32（95%CI，13-77，P<0.001）。
需要进行AVVr的单心室患者，尤其是在双向腔肺分流术前进行手术的患者，无移植生存率较低，在过去20年中没有显著改善。体重轻和右心室形态与死亡率增加密切相关。对于这个高危亚组应考虑替代治疗策略。
