Department of Cardiothoracic Surgery, The Ohio State University School of Medicine, 2650Nationwide Children's Hospital, Columbus, OH, USA.
Division of Cardiology, The Ohio State University School of Medicine, 2650Nationwide Children's Hospital, Columbus, OH, USA.
World J Pediatr Congenit Heart Surg. 2021 Jan;12(1):9-16. doi: 10.1177/2150135120947692. Epub 2020 Aug 12.
Tricuspid valve (TV) and right ventricular (RV) function are major determinants of morbidity and mortality in patients with hypoplastic left heart syndrome (HLHS). We sought to retrospectively evaluate these parameters throughout the hybrid palliation strategy.
From 2002 to 2018, 203 patients with HLHS and variants presented for hybrid stage I (HS1). Echocardiographic evaluation of tricuspid regurgitation (TR) and RV function was assessed at multiple time points. Clinical outcomes including tricuspid valvuloplasty, transplantation, and death were reviewed.
The most prevalent HLHS subtype was aortic atresia/mitral atresia. The presence of significant TR and/or RV dysfunction was 14.78% and 9.36%, respectively, at the time of initial HS1. There were 185 survivors following HS1 (91.13%, n = 185/203), while 147 patients underwent comprehensive stage II or bidirectional Glenn shunt (72.41%, n = 147/203). Tricuspid valvuloplasty was undertaken in nine patients (4.86%, n = 9/185). Ultimately, 100 patients underwent the Fontan procedure. The odds of development of significant TR and/or RV dysfunction were not statistically different throughout the stages of palliation (TR: odds ratio [OR] = 0.14-0.25, 5260; RV dysfunction: OR = 0.02-0.13, = .3992). However, the risk of death and/or transplant was 2.5- to 3.8-fold when either were present alone or in combination (TR: OR = 2.58, = .0356; RV dysfunction: OR = 3.84, = .0262). Transplant-free survival at 15 years was 44.8%.
Following hybrid palliation for HLHS, the majority of survivors have normal RV and TV functions. Tricuspid valvuloplasty was required in few patients. Once significant TR and/or RV dysfunction ensues, there is a two- to three-fold risk of death and/or transplant.
三尖瓣（TV）和右心室（RV）功能是左心发育不全综合征（HLHS）患者发病率和死亡率的主要决定因素。我们试图通过回顾性评估整个杂交姑息治疗策略来评估这些参数。
从 2002 年到 2018 年，203 例 HLHS 及变异患者接受了杂交一期（HS1）治疗。在多个时间点评估三尖瓣反流（TR）和 RV 功能的超声心动图评估。回顾了包括三尖瓣成形术、移植和死亡在内的临床结果。
最常见的 HLHS 亚型为主动脉狭窄/二尖瓣狭窄。在初始 HS1 时，分别有 14.78%和 9.36%的患者存在显著的 TR 和/或 RV 功能障碍。HS1 后有 185 例患者存活（91.13%，n = 185/203），147 例患者接受了全面二期或双向 Glenn 分流术（72.41%，n = 147/203）。9 例（4.86%，n = 9/185）患者接受了三尖瓣成形术。最终，100 例患者接受了 Fontan 手术。在姑息治疗的各个阶段，发展为显著 TR 和/或 RV 功能障碍的几率没有统计学差异（TR：比值比[OR] = 0.14-0.25，5260；RV 功能障碍：OR = 0.02-0.13， =.3992）。然而，当单独或联合存在时，死亡和/或移植的风险为 2.5-3.8 倍（TR：OR = 2.58， =.0356；RV 功能障碍：OR = 3.84， =.0262）。15 年无移植生存率为 44.8%。
HLHS 接受杂交姑息治疗后，大多数存活者的 RV 和 TV 功能正常。只有少数患者需要进行三尖瓣成形术。一旦出现明显的 TR 和/或 RV 功能障碍，死亡和/或移植的风险增加两到三倍。
