O'Connor Matthew J, Elias Matthew D, Cohen Meryl S, Quartermain Michael D
Division of Cardiology, Department of Pediatrics, University of Pennsylvania School of Medicine, The Children's Hospital of Philadelphia, Philadelphia, PA 19104-4399, USA.
Pediatr Cardiol. 2012 Apr;33(4):547-53. doi: 10.1007/s00246-011-0147-x. Epub 2011 Nov 19.
Outcomes in patients with ventricular dysfunction undergoing superior cavopulmonary connection (SCPC) are not well known. We reviewed records of patients undergoing SCPC at our center from December 2005 to October 2009 and studied those whose pre-SCPC echocardiograms demonstrated at least moderate systemic ventricular dysfunction. Of the 213 patients undergoing SCPC, 19 (9%) met inclusion criteria. Diagnoses were hypoplastic left heart syndrome (n = 18) and rightward unbalanced atrioventricular canal with pulmonary stenosis (n = 1). In those surviving >2 months after SCPC, ventricular function was assessed by echocardiography 4.9 (range 3.5 to 9.7) months after SCPC and was improved in ten of 17 (59%), unchanged in six of 17 (35%), and worsened in one of 17 (16%) patients. After SCPC, three patients died, and one underwent heart transplant 21.9 months after SCPC. Transplant-free survival was attained by 15 of 19 (79%) patients during follow-up of 33.0 months (range 10.8 to 51.4). Fontan completion was performed on six survivors on reaching an appropriate age. Ventricular dysfunction before SCPC is not uncommon and occurs primarily in patients with a morphologic right ventricle. Ventricular performance improves in the majority of patients after SCPC, and midterm outcomes are comparable with previous reports, suggesting that these patients remain candidates for staged palliation.
接受上腔静脉肺动脉连接术（SCPC）的心室功能不全患者的预后情况尚不明确。我们回顾了2005年12月至2009年10月在我们中心接受SCPC的患者记录，并研究了那些SCPC术前超声心动图显示至少存在中度系统性心室功能不全的患者。在213例行SCPC的患者中，19例（9%）符合纳入标准。诊断为左心发育不全综合征（n = 18）和右向不平衡型房室管合并肺动脉狭窄（n = 1）。在SCPC术后存活超过2个月的患者中，于SCPC术后4.9（范围3.5至9.7）个月通过超声心动图评估心室功能，17例患者中有10例（59%）改善，17例中有6例（35%）不变，17例中有1例（16%）恶化。SCPC术后，3例患者死亡，1例在SCPC术后21.9个月接受心脏移植。在33.0个月（范围10.8至51.4）的随访期间，19例患者中有15例（79%）实现了无移植存活。6名存活患者在达到合适年龄时完成了Fontan手术。SCPC术前的心室功能不全并不少见，主要发生在形态学上为右心室的患者中。大多数患者在SCPC术后心室功能有所改善，中期预后与先前报告相当，表明这些患者仍是分期姑息治疗的候选者。
