Department of Pediatric Cardiac Surgery, Bambino Gesù Children's Hospital, Rome, Italy.
J Thorac Cardiovasc Surg. 2010 Nov;140(5):1092-103. doi: 10.1016/j.jtcvs.2010.07.087. Epub 2010 Sep 17.
Identification of variables influencing surgical outcome in patients treated for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.
A total of 90 consecutive patients (median age, 12 months; range, 20 days to 35 years), who had primarily undergone either 1-stage unifocalization (n = 69) or palliation to promote native pulmonary arterial development (n = 21), were studied. Chromosome 22q11 deletion had occurred in 37% of the cases. Ventricular septal defect closure was accomplished in 70 patients (78%), with a mean postoperative right/left ventricular pressure ratio of 0.48 ± 0.14.
The rate of 14-year survival, freedom from conduit reintervention, and freedom from percutaneous intervention on the pulmonary arteries was 75%, 46%, and 52%, respectively. At a median interval of 95 months (range, 1.5-164 months), the right/left ventricular pressure ratio did not differ significantly from early postoperatively. Univariate analysis showed that an absence of confluent intrapericardial pulmonary arteries favorably affected the postoperative right/left ventricular pressure ratio after ventricular septal defect closure (P = .04). Kaplan-Meier estimates showed age of 30 days or younger (P = .0004) and weight of 3 kg or less (P = .0004) at unifocalization and chromosome 22q11 deletion (P = .001) significantly affected survival. Chromosome 22q11 deletion was significantly associated with mortality, even in the Cox regression model (hazard ratio, 8.26; P = .003). Finally, ventricular septal defect closure during single-stage and single/multiple-stage procedures significantly correlated with both early (P = .0013 and P < .00001, respectively) and overall (P = .013 and P = .0007, respectively) survival.
The results of surgery were satisfactory and durable, despite the need for repeated percutaneous or surgical reinterventions. The outcomes were negatively affected by neonatal age and low body weight and positively affected by simultaneous or staged ventricular septal defect closure. Finally, chromosome 22q11 deletion remained an independent variable affecting survival.
确定影响接受肺动脉瓣闭锁伴大型体肺侧支动脉患儿手术结果的变量。
本研究纳入了 90 例连续性病例（中位年龄 12 个月；范围：20 天至 35 年），这些患儿主要接受了一期矫治术（n=69）或姑息性治疗以促进肺血管发育（n=21）。37%的患儿存在 22q11 号染色体缺失。70 例患者（78%）行室间隔缺损修补术，术后右/左心室压力比为 0.48±0.14。
14 年生存率、免于管道再干预和免于肺动脉介入治疗率分别为 75%、46%和 52%。中位随访时间为 95 个月（范围：1.5-164 个月），术后右/左心室压力比与早期相比无显著差异。单因素分析显示，无融合性心包内肺血管对室间隔缺损修补术后右/左心室压力比有有利影响（P=0.04）。Kaplan-Meier 估计显示，在一期和单/多期手术中，年龄 30 天或更小（P=0.0004）和体重 3kg 或更轻（P=0.0004）以及 22q11 号染色体缺失（P=0.001）显著影响生存率。即使在 Cox 回归模型中，22q11 号染色体缺失也与死亡率显著相关（危险比，8.26；P=0.003）。最后，一期和单/多期手术中行室间隔缺损修补术与早期（P=0.0013 和 P<0.00001）和总体生存率（P=0.013 和 P=0.0007）显著相关。
尽管需要多次经皮或手术再干预，但手术结果令人满意且持久。新生儿年龄和低体重对结果有负面影响，而同期或分期行室间隔缺损修补术则有积极影响。最后，22q11 号染色体缺失仍然是影响生存率的独立变量。
