Chacon-Portillo Martin A, Zea-Vera Rodrigo, Zhu Huirong, Dickerson Heather A, Adachi Iki, Heinle Jeffrey S, Fraser Charles D, Mery Carlos M
Division of Congenital Heart Surgery, Texas Children's Hospital, Houston, Texas.
Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas.
Congenit Heart Dis. 2018 Nov;13(6):927-934. doi: 10.1111/chd.12664. Epub 2018 Oct 2.
There are limited studies analyzing pulsatile Glenn as a long-term palliation strategy for single ventricle patients. This study sought to determine their outcomes at a single institution.
A retrospective review was performed.
Study performed at a single pediatric hospital.
All single ventricle patients who underwent pulsatile Glenn from 1995 to 2016 were included.
Pulsatile Glenn failure was defined as takedown, transplant, or death. Further palliation was defined as Fontan, 1.5, or biventricular repair. Risk factors were assessed by Cox multivariable competing risk analyses.
Seventy-eight patients underwent pulsatile Glenn at age 9 months (interquartile range, 5-14). In total, 28% had heterotaxy, 18% had a genetic syndrome, and 24% had an abnormal inferior vena cava. There were 3 (4%) perioperative mortalities. Further palliation was performed in 41 (53%) patients with a median time-to-palliation of 4 years (interquartile range, 3-5). Pulsatile Glenn failure occurred in 10 (13%) patients with 8 total mortalities. Five- and 10-year transplant-free survival were 91% and 84%, respectively. At a median follow-up of 6 years (interquartile range, 2-8), 27 patients (35%) remained with PG (age 7 years [interquartile range, 3-11], oxygen saturation 83% ± 4%). Preoperative moderate-severe atrioventricular valve regurgitation (AVVR) (hazard ratio 7.77; 95% confidence interval 1.80-33.43; P =.005) and higher pulmonary vascular resistance (hazard ratio 2.59; 95% confidence interval 1.08-6.15; P =.031) were predictors of pulsatile Glenn failure after adjusting for covariates. Reaching further palliation was less likely in patients with preoperative moderate-severe AVVR (hazard ratio 0.22, 95% confidence interval 0.08-0.59; P =.002).
Pulsatile Glenn can be an effective tool to be used in challenging circumstances, these patients can have a favorable long-term prognosis without reducing their suitability for further palliation.
分析搏动性格林分流术作为单心室患者长期姑息治疗策略的研究有限。本研究旨在确定单中心此类患者的治疗结果。
进行回顾性分析。
在一家儿科医院开展研究。
纳入1995年至2016年期间接受搏动性格林分流术的所有单心室患者。
搏动性格林分流术失败定义为拆除分流、移植或死亡。进一步姑息治疗定义为Fontan手术、1.5心室修复或双心室修复。通过Cox多变量竞争风险分析评估危险因素。
78例患者在9个月大时（四分位间距，5 - 14个月）接受了搏动性格林分流术。总体而言，28%患有内脏异位，18%患有遗传综合征，24%下腔静脉异常。围手术期死亡3例（4%）。41例（53%）患者进行了进一步姑息治疗，姑息治疗的中位时间为4年（四分位间距，3 - 5年）。10例（13%）患者出现搏动性格林分流术失败，共8例死亡。5年和10年无移植生存率分别为91%和84%。中位随访6年（四分位间距，2 - 8年）时，27例患者（35%）仍保留搏动性格林分流术（年龄7岁[四分位间距，3 - 11岁]，血氧饱和度83%±4%）。术前中重度房室瓣反流（AVVR）（风险比7.77；95%置信区间1.80 - 33.43；P = 0.005）和较高的肺血管阻力（风险比2.59；95%置信区间1.08 - 6.15；P = 0.031）是调整协变量后搏动性格林分流术失败的预测因素。术前中重度AVVR患者进行进一步姑息治疗的可能性较小（风险比0.22，95%置信区间0.08 - 0.59；P = 0.002）。
搏动性格林分流术可作为应对复杂情况的有效手段，这些患者可获得良好的长期预后，且不影响其接受进一步姑息治疗的适宜性。
