The Heart Center, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
Eur J Cardiothorac Surg. 2012 Jul;42(1):42-8; discussion 48-9. doi: 10.1093/ejcts/ezr280. Epub 2012 Jan 26.
The Glenn bidirectional cavopulmonary connection (BCPC) is an established procedure in multistage palliation of various single ventricle (SV) anomalies. We aimed to report the current outcomes following BCPC and to examine risk factors affecting survival and progression to the next palliation stage.
Two hundred and twenty-seven consecutive children with variable SV pathologies underwent BCPC from 2002 to 2007. Competing risk analyses were performed to model events after BCPC (death and transition to Fontan) and subsequently after Fontan (death and cardiac reoperation) and to examine the associated risk factors for poor outcomes.
There were 139 males (61%) with a median age of 7.6 months [interquartile range (IQR) 6.0-10.8] and median weight of 6.2 kg (IQR 5.2-7.4). Forty-three patients (19%) had primary BCPC and 184 (81%) had prior palliation: aortopulmonary shunt (APS) (n=83), Norwood (n=55), pulmonary artery (PA) band (n=48), atrial septectomy (n=25), PA reconstruction (n=14), anomalous pulmonary venous connection repair (n=7) and other (n=8). Predominant ventricle was left morphology (n=122, 54%), right morphology (n=95, 42%) and two equally developed ventricles (n=10, 4%). Twenty-six patients (12%) had bilateral superior vena cava. Concomitant surgery included atrioventricular valve repair (n=18), PA augmentation (n=80), percutaneous Fontan preparation (n=34) and other (n=24). Competing risk analysis showed that 5 years following BCPC, ∼17% have died, 76% have undergone Fontan and 7% were alive awaiting or not qualifying for Fontan. On multivariable analysis, risk factors for death prior to Fontan were pulmonary vascular resistance (PVR) index of >3 WU/M2 [hazard ratio (HR) 3.9, P=0.001], dominant right ventricle (HR 2.1, P=0.03) and prior palliation other than APS (HR 0.4, P=0.03). Competing risk analysis showed that 3 years following 172 Fontan operations, ∼10% have died, 6% have undergone further cardiac surgery and 84% were alive and free from reoperation. Overall, 8-year survival following BCPC was only 74%.
Despite established selection criteria and improved surgical technique and medical management, there is a continuous failure and attrition risk following BCPC. Outcomes are influenced by underlying cardiac anomaly; patients with dominant left ventricle (i.e. tricuspid atresia, double inlet left ventricle) having the best survival while those with dominant right ventricle (i.e. hypoplastic left heart syndrome, double outlet right ventricle with heterotaxy) having the worst survival. Increased PVR remains a significant factor affecting mortality.
Glenn 双向腔肺连接（BCPC）是各种单心室（SV）畸形多阶段姑息治疗中的一种成熟手术。我们旨在报告接受 BCPC 后的当前结果，并研究影响生存和进展到下一个姑息治疗阶段的风险因素。
2002 年至 2007 年间，227 例不同 SV 病变的连续患儿接受了 BCPC。采用竞争风险分析对 BCPC 后（死亡和过渡到 Fontan）以及 Fontan 后（死亡和心脏再次手术）的事件进行建模，并检查与不良结果相关的相关风险因素。
139 例男性（61%），中位年龄为 7.6 个月[四分位距（IQR）6.0-10.8]，中位体重为 6.2 公斤（IQR 5.2-7.4）。43 例（19%）为原发性 BCPC，184 例（81%）为姑息治疗前患者：主动脉肺动脉分流术（APS）（n=83）、Norwood 手术（n=55）、肺动脉（PA）带（n=48）、房间隔切开术（n=25）、PA 重建术（n=14）、异常肺静脉连接修复术（n=7）和其他（n=8）。优势心室为左形态（n=122，54%）、右形态（n=95，42%）和两个等发育心室（n=10，4%）。26 例患者（12%）有双侧上腔静脉。同时进行的手术包括房室瓣修复术（n=18）、PA 增强术（n=80）、经皮 Fontan 准备术（n=34）和其他（n=24）。竞争风险分析显示，BCPC 后 5 年，约 17%的患者死亡，76%的患者进行了 Fontan 手术，7%的患者仍在等待或不符合 Fontan 手术条件。多变量分析显示，Fontan 手术前死亡的风险因素是肺血管阻力（PVR）指数>3 WU/M2[风险比（HR）3.9，P=0.001]、优势右心室（HR 2.1，P=0.03）和姑息治疗前的其他手术（HR 0.4，P=0.03）。竞争风险分析显示，Fontan 手术后 172 例手术 3 年后，约 10%的患者死亡，6%的患者进行了进一步的心脏手术，84%的患者存活且无再次手术。总体而言，BCPC 后 8 年生存率仅为 74%。
尽管有既定的选择标准以及改进的手术技术和医疗管理，但 BCPC 后仍存在持续的失败和损耗风险。结果受潜在的心脏异常影响；优势左心室（即三尖瓣闭锁、双入口左心室）的患者生存情况最好，而优势右心室（即左心发育不良综合征、右心室双出口伴异构）的患者生存情况最差。持续增高的 PVR 仍然是影响死亡率的一个重要因素。
