Long-term results of treatments for functional single ventricle associated with extracardiac type total anomalous pulmonary venous connection
Long-term results of treatments for functional single ventricle associated with extracardiac type total anomalous pulmonary venous connection
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DOI：
10.1093/ejcts/ezs594
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发表时间：
2013-05-01
影响因子：
3.4
通讯作者：
Ichikawa, Hajime
中科院分区：
文献类型：
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作者：
Hoashi, Takaya;Kagisaki, Koji;Ichikawa, Hajime
功能性单心室患者的手术效果有所改善，但并发心外型完全性肺静脉异位联管（TAPVC）的患者的手术效果仍然较差。我们回顾性地回顾了我们 21 年针对这一具有挑战性的群体的手术经验。从 1990 年到 2010 年，连续 48 名功能性单心室并发心外 TAPVC 患者（26 名男性，46 名右心房异构）在我们中心接受了初次姑息手术。手术时的中位年龄和体重分别为 69 天和 3.5 公斤。 TAPVC 类型为心上型 31 例，心下型 14 例，混合型 3 例。双向 Glenn (BDG) 前修复 TAPVC 25 例，BDG 修复 18 例，保留 3 例。自2007年起，针对术前肺静脉阻塞的患者进行阻塞性引流静脉支架植入术，以及用于缓解术后肺静脉狭窄（PVS）的无缝线造袋术。平均随访时间为 4.2 +/- 5.1 年。初次手术干预后 1、3 和 5 年的总生存率分别为 58.3%、41.1 和 31.3%。 16名患者完成了Fontan手术（33.3%）。修复后 1 年和 3 年无术后 PVS 率分别为 68.7% 和 63.4%。单变量分析发现，心下TAPVC（P = 0.036）、共存主要主动脉肺动脉循环（P = 0.017）和BDG前TAPVC修复（P = 0.036）均会降低生存率，多变量分析表明BDG前TAPVC修复是唯一的危险因素（P = 0.032）。而术后PVS的发生并没有降低生存率，对Fontan手术的实现有显着的负面影响（P=0.008）。累积生存率并没有因手术时代而提高。功能性单心室患者因静脉引流通路阻塞而在新生儿期接受心外TAPVC修复的手术结果仍然较差。针对阻塞性引流静脉进行支架植入，以延迟手术矫正和无缝线造袋术的时间，因为术后 PVS 的缓解预计将改善后期结果；但效果仍然有限。
Surgical outcomes of patients with functional single ventricle have improved, though those for patients whose condition is complicated by extracardiac type total anomalous pulmonary venous connection (TAPVC) remain poor. We retrospectively reviewed our 21 years of surgical experiences with this challenging group.From 1990 to 2010, 48 consecutive patients with functional single ventricle complicated by extracardiac TAPVC (26 males, 46 with right atrial isomerism) underwent initial surgical palliation at our centre. The median age and body weight at surgery were 69 days and 3.5 kg, respectively. The type of TAPVC was supracardiac in 31 patients, infracardiac in 14 and mixed type in 3. TAPVC was repaired in 25 patients before bidirectional Glenn (BDG) and 18 at BDG, while it remained in 3 patients. Since 2007, stent implantation for obstructive drainage veins for patients with preoperative pulmonary venous obstruction and sutureless marsupialization for relief of postoperative pulmonary venous stenosis (PVS) have been initiated. The mean follow-up period was 4.2 +/- 5.1 years.The overall survival rates at 1, 3 and 5 years after the initial surgical intervention were 58.3, 41.1 and 31.3%, respectively. Sixteen patients achieved the Fontan operation (33.3%). The freedom from postoperative PVS rates at 1 and 3 years after repair was 68.7 and 63.4%, respectively. Univariate analysis detected that infracardiac TAPVC (P = 0.036), coexisting major aortopulmonary collaterals (P = 0.017), and TAPVC repair before BDG (P = 0.036) all reduced survival, and multivariable analysis indicated the repair of TAPVC before BDG as the only risk factor (P = 0.032). Whereas the occurrence of postoperative PVS did not reduce survival, which had a significant negative impact on achieving the Fontan operation (P = 0.008). The cumulative survival rate did not improve by surgical era.Surgical outcomes of patients with functional single ventricle undergoing the repair of extracardiac TAPVC in the neonatal period due to obstruction of the venous drainage pathway remain poor. Stent implantation for obstructive drainage veins to delay the timing of surgical correction and sutureless marsupialization as relief of postoperative PVS are expected to improve the late outcomes; however, the effect is still limited.