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影响新生儿改良布莱洛克-陶西格分流术后死亡及进入下一阶段进展的因素。

Factors affecting death and progression towards next stage following modified Blalock-Taussig shunt in neonates.

作者信息

Alsoufi Bahaaldin, Gillespie Scott, Mori Makoto, Clabby Martha, Kanter Kirk, Kogon Brian

机构信息

Division of Cardiothoracic Surgery, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, GA, USA

Department of Pediatrics, Division of Cardiology, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, GA, USA.

出版信息

Eur J Cardiothorac Surg. 2016 Jul;50(1):169-77. doi: 10.1093/ejcts/ezw017. Epub 2016 Feb 23.

Abstract

OBJECTIVES

The modified Blalock-Taussig shunt (BTS) is utilized to palliate neonates born with restrictive pulmonary blood flow including those with single ventricle (SV) or biventricular (BV) cardiac anomalies. We aim in the current study to report palliation outcomes of neonates with BTS and to examine factors affecting death and progression to the subsequent stage of palliation or repair.

METHODS

Between 2002 and 2012, 341 patients underwent BTS including 175 with SV and 166 with BV anomalies. Competing risk analysis modelled events after BTS (death or transplantation, transition to Glenn shunt or biventricular repair) and examined risk factors affecting outcomes.

RESULTS

SV patients had a higher incidence of extracorporeal membrane oxygenation (ECMO) support requirement (12 vs 4%, P = 0.004) and unplanned cardiac reoperation (14 vs 7%, P = 0.051) than their BV counterparts. Additionally, hospital mortality was higher in SV than in BV patients (15 vs 3%, P < 0.001). In SV patients, competing risk analysis showed that, 2 years following BTS, 27% of patients had died or received transplantation and 73% had undergone the Glenn shunt. On multivariable analysis, factors associated with time until death or transplantation prior to Glenn were cardiopulmonary bypass [hazard ratio (HR) 3.6 (2.0-6.4), P < 0.001], unplanned cardiac reoperation [HR 2.4 (1.3-4.6), P = 0.007], pulmonary atresia [HR 2.0 (1.1-3.7), P = 0.026] and the shunt size/weight ratio [HR 1.3 (1.1-1.4) per 0.1 increase, P = 0.001]. In BV patients, competing risk analysis showed that, 2 years following BTS, 13% of patients had died or received transplantation, 85% had undergone biventricular repair and 2% were alive without biventricular repair. On multivariable analysis, factors associated with time until death or transplantation prior to biventricular repair were genetic syndromes and extracardiac malformations [HR 6.1 (2.0-18.2), P = 0.001], weight ≤2.5 kg [HR 5.6 (2.0-16.0), P = 0.001] and male gender [HR 3.4 (1.1-11.0), P = 0.041].

CONCLUSIONS

Palliation with BTS continues to be associated with significant operative morbidity and mortality. In addition to hospital death, there is an important interstage attrition risk prior to subsequent palliation or biventricular repair. Inherent patient characteristics (i.e. genetic syndromes and low weight) and anatomical details (i.e. SV, pulmonary atresia and concomitant cardiac anomalies) are associated with worse survival.

摘要

目的

改良布莱洛克 - 陶西格分流术(BTS)用于缓解患有限制性肺血流的新生儿,包括那些患有单心室(SV)或双心室(BV)心脏畸形的新生儿。我们在本研究中的目的是报告接受BTS的新生儿的缓解结果,并检查影响死亡以及进展至后续缓解或修复阶段的因素。

方法

2002年至2012年间,341例患者接受了BTS,其中175例为SV畸形,166例为BV畸形。竞争风险分析对BTS后的事件(死亡或移植、过渡到格林分流术或双心室修复)进行建模,并检查影响结果的风险因素。

结果

与BV患者相比,SV患者需要体外膜肺氧合(ECMO)支持的发生率更高(12%对4%,P = 0.004),以及计划外心脏再次手术的发生率更高(14%对7%,P = 0.051)。此外,SV患者的医院死亡率高于BV患者(15%对3%,P < 0.001)。在SV患者中,竞争风险分析显示,BTS后2年,27%的患者死亡或接受了移植,73%的患者接受了格林分流术。多变量分析显示,在格林分流术前直至死亡或移植的时间相关因素包括体外循环[风险比(HR)3.6(2.0 - 6.4),P < 0.001]、计划外心脏再次手术[HR 2.4(1.3 - 4.6),P = 0.007]、肺动脉闭锁[HR 2.0(1.1 - 3.7),P = 0.026]以及分流尺寸/体重比[每增加0.1,HR 1.3(1.1 - 1.4),P = 0.001]。在BV患者中,竞争风险分析显示,BTS后2年,13%的患者死亡或接受了移植,85%的患者接受了双心室修复,2%的患者存活但未接受双心室修复。多变量分析显示,在双心室修复术前直至死亡或移植的时间相关因素包括遗传综合征和心外畸形[HR 6.1(2.0 - 18.2),P = 0.001]、体重≤2.5 kg[HR 5.6(2.0 - 16.0),P = 0.001]以及男性性别[HR 3.4(1.1 - 11.0),P = 0.041]。

结论

BTS缓解术仍然与显著的手术发病率和死亡率相关。除了医院死亡外,在后续缓解或双心室修复之前存在重要的阶段间损耗风险。内在的患者特征(即遗传综合征和低体重)以及解剖学细节(即SV、肺动脉闭锁和合并的心脏畸形)与较差的生存率相关。

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