Programmatic Approach to Management of Tetralogy of Fallot With Major Aortopulmonary Collateral Arteries A 15-Year Experience With 458 Patients
Programmatic Approach to Management of Tetralogy of Fallot With Major Aortopulmonary Collateral Arteries A 15-Year Experience With 458 Patients
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DOI：
10.1161/circinterventions.116.004952
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发表时间：
2017-04-01
影响因子：
5.6
通讯作者：
McElhinney, Doff B.
中科院分区：
文献类型：
--
作者：
Bauser-Heaton, Holly;Borquez, Alejandro;McElhinney, Doff B.
背景--法洛四联症合并主要的主-肺侧支动脉是一种复杂且异质性的疾病。我们对这种病变的机构治疗方法强调早期完全修复，合并所有肺段和广泛的肺叶和节段性肺动脉重建。方法和结果-我们回顾了自2001年11月以来在斯坦福大学卢西尔帕卡德儿童医院(LPCHS)接受手术治疗的所有法洛四联症和主要主肺侧支动脉的患者。共有458名患者接受了手术，其中291名(%)在LPCHS接受了第一次手术。患者在第一次LPCHS手术后平均随访2.7年(平均4.3年)，估计第一次手术干预后5年的存活率为85%。与生存不良相关的因素包括首次LPCHS手术类型，而不是完全修复和Alagille综合征。在整个队列中，402名患者获得了完全的单一定位和修复，要么是一期手术(n=186)，经过我们中心的初步缓解(n=74)，要么是在其他地方手术后在LPCHS修复/翻修(n=142)。修复后右室/主动脉压中位数比值为0.35。术后10年生存率为92.5%，染色体异常、高龄、侧支循环数目多、右室压高的患者术后存活率较低。结论：采用强调早期完全定位修复并结合所有肺血管供应的方法，对法洛四联症患者和既往手术的法洛四联症患者及主要的主-肺侧支动脉患者均取得了良好的效果。
Background-Tetralogy of Fallot with major aortopulmonary collateral arteries is a complex and heterogeneous condition. Our institutional approach to this lesion emphasizes early complete repair with the incorporation of all lung segments and extensive lobar and segmental pulmonary artery reconstruction.Methods and Results-We reviewed all patients who underwent surgical intervention for tetralogy of Fallot and major aortopulmonary collateral arteries at Lucile Packard Children's Hospital Stanford (LPCHS) since November 2001. A total of 458 patients underwent surgery, 291 (64%) of whom underwent their initial procedure at LPCHS. Patients were followed for a median of 2.7 years (mean 4.3 years) after the first LPCHS surgery, with an estimated survival of 85% at 5 years after first surgical intervention. Factors associated with worse survival included first LPCHS surgery type other than complete repair and Alagille syndrome. Of the overall cohort, 402 patients achieved complete unifocalization and repair, either as a single-stage procedure (n=186), after initial palliation at our center (n=74), or after surgery elsewhere followed by repair/revision at LPCHS (n=142). The median right ventricle: aortic pressure ratio after repair was 0.35. Estimated survival after repair was 92.5% at 10 years and was shorter in patients with chromosomal anomalies, older age, a greater number of collaterals unifocalized, and higher postrepair right ventricle pressure.Conclusions-Using an approach that emphasizes early complete unifocalization and repair with incorporation of all pulmonary vascular supply, we have achieved excellent results in patients with both native and previously operated tetralogy of Fallot and major aortopulmonary collateral arteries.