Stephens Elizabeth H, Han Jiho, Ginns Jonathan, Rosenbaum Marlon, Chai Paul, Bacha Emile, Kalfa David
1 Division of Cardiac, Thoracic, and Vascular Surgery, Section of Pediatric and Congenital Cardiac Surgery, Morgan Stanley Children's Hospital, New York Presbyterian, Columbia University Medical Center, New York, NY, USA.
2 Department of Adult Congenital Heart Disease, Morgan Stanley Children's Hospital of New York-Presbyterian, Columbia University Medical Center, New York, NY, USA.
World J Pediatr Congenit Heart Surg. 2017 May;8(3):346-353. doi: 10.1177/2150135117692974.
Adults with congenital heart disease (ACHD) undergoing systemic atrioventricular valve (SAVV) surgery are a complex, understudied population. We assessed midterm outcomes and prognostic factors in ACHD undergoing SAVV surgery.
We performed retrospective evaluation of ACHD undergoing SAVV surgery from January 2005 to February 2016: 14 (33%) patients with congenital mitral valve stenosis/regurgitation, 15 (35%) with atrioventricular septal defect (AVSD), and 14 (33%) with congenitally corrected transposition of the great arteries (ccTGA) with systemic tricuspid valve regurgitation. Adverse events were defined as mortality, reoperation on SAVV, and late more-than-moderate (> moderate) SAVV regurgitation. Statistical analysis was performed using Fisher's exact test and one-way analysis of variance as well as univariate and multivariate risk factor analysis.
Fifteen (35%) patients had preoperative systemic ventricular dysfunction, including 13 patients with ccTGA (93%, P < .001). Twenty-three (54%) patients underwent valve repair, 20 (47%) patients underwent replacement, and 20 (47%) patients underwent an associated procedure. Replacement was higher in patients with ccTGA (86%) than the other groups ( P < 0.01). Thirty-seven patients (91% of survivors) were free of significant SAVV regurgitation at last follow-up, with patients with AVSD having greater regurgitation grades compared to the other groups ( P < 0.01). In-hospital mortality, late mortality, late > moderate SAVV regurgitation, and SAVV reoperation rates were 5% (n = 2), 2% (n = 1), 9% (n = 3), and 7% (n = 3), respectively. On multivariate analysis, predischarge SAVV regurgitation grade was the only significant predictor of adverse events (odds ratio = 8.2, 95% confidence interval: 1.1-63.8, P = .045).
Overall outcomes in this challenging population are good. The single factor associated with adverse events was predischarge SAVV regurgitation grade.
接受体循环房室瓣（SAVV）手术的先天性心脏病成年患者是一个复杂且研究不足的群体。我们评估了接受SAVV手术的先天性心脏病成年患者的中期结局和预后因素。
我们对2005年1月至2016年2月期间接受SAVV手术的先天性心脏病成年患者进行了回顾性评估：14例（33%）患有先天性二尖瓣狭窄/反流，15例（35%）患有房室间隔缺损（AVSD），14例（33%）患有大动脉转位矫正型（ccTGA）并伴有体循环三尖瓣反流。不良事件定义为死亡、SAVV再次手术以及晚期中重度以上（>中度）SAVV反流。采用Fisher精确检验、单因素方差分析以及单因素和多因素危险因素分析进行统计分析。
15例（35%）患者术前存在体循环心室功能障碍，其中13例为ccTGA患者（93%，P <.001）。23例（54%）患者接受了瓣膜修复，20例（47%）患者接受了瓣膜置换，20例（47%）患者接受了相关手术。ccTGA患者的瓣膜置换率（86%）高于其他组（P < 0.01）。在最后一次随访时，37例患者（91%的幸存者）无明显SAVV反流，与其他组相比，AVSD患者的反流程度更高（P < 0.01）。住院死亡率、晚期死亡率、晚期中重度以上SAVV反流率和SAVV再次手术率分别为5%（n = 2）、2%（n = 1）、9%（n = 3）和7%（n = 3）。多因素分析显示，出院前SAVV反流程度是不良事件的唯一显著预测因素（比值比 = 8.2，95%置信区间：1.1 - 63.8，P =.045）。
这一具有挑战性群体的总体结局良好。与不良事件相关的单一因素是出院前SAVV反流程度。
