Section of Pediatric Cardiothoracic Surgery, Medical University of South Carolina, Charleston, South Carolina.
Section of Congenital Cardiac Surgery, University of Colorado, Aurora, Colorado.
Ann Thorac Surg. 2021 Jul;112(1):147-154. doi: 10.1016/j.athoracsur.2020.07.038. Epub 2020 Sep 25.
Norwood palliation for hypoplastic left heart syndrome typically results in retrograde perfusion of the native aortic root. This may predispose to native aortic root thrombosis (NART).
Patients who underwent stage 1 Norwood palliation at Children's Hospital Colorado between 2003 and 2019 were retrospectively reviewed for NART. Additional patients were identified by a systematic literature review. Patient factors, operative details, presentation, diagnostic methods, treatments, and outcomes were analyzed.
Three patients with NART were identified among 241 patients who had undergone stage 1 Norwood palliation at Children's Hospital Colorado (mean follow-up, 48 months). A fourth patient identified at Children's Hospital Colorado had undergone stage 1 palliation elsewhere. The systematic literature review identified 12 additional patients. Among the 16 patients, patients presented with new arrhythmias (38% [n = 6]), evidence of ischemia (31% [n = 5]), incidentally (19% [n = 3]), inability to wean from postoperative support (6% [n = 1]), and cardiac arrest (6% [n = 1). The diagnosis was made by transthoracic echocardiography (TTE) in 50% (n = 8), transesophageal echocardiography in 19% (n = 3), cardiac catheterization in 25% (n = 4), and cardiac computed tomography in 6% (n = 1). Importantly, 44% (n = 7) of patients were missed by TTE and subsequently diagnosed by other methods. Treatment strategies were surgical thrombectomy in 38% (n = 6), anticoagulation alone in 38% (n = 6), direct catheter thrombolysis in 19% (n = 3), and systemic thrombolysis in 6% (n = 1). The rate of mortality or transplant listing was 44% (n = 7).
NART is an underappreciated complication of Norwood palliation with high mortality. There should be a high index of suspicion for NART in patients after Norwood palliation with an unexplained complicated clinical course. Importantly, NART is frequently missed by TTE.
左心发育不全综合征的 Norwood 姑息术通常会导致原生主动脉根部逆行灌注。这可能使原生主动脉根部血栓形成（NART）的易感性增加。
对 2003 年至 2019 年间在科罗拉多儿童医院接受一期 Norwood 姑息术的患者进行回顾性研究，以确定 NART。通过系统文献回顾确定了其他患者。分析患者因素、手术细节、临床表现、诊断方法、治疗方法和结果。
在科罗拉多儿童医院接受一期 Norwood 姑息术的 241 例患者中，发现 3 例（平均随访时间为 48 个月）有 NART。在科罗拉多儿童医院发现的第 4 例患者在其他地方接受了一期姑息治疗。系统文献回顾确定了 12 例其他患者。在 16 例患者中，患者出现新发心律失常（38%[n=6]）、缺血证据（31%[n=5]）、偶发（19%[n=3]）、无法从术后支持中脱机（6%[n=1]）和心脏骤停（6%[n=1]）。诊断方法为经胸超声心动图（TTE）50%（n=8）、经食管超声心动图 19%（n=3）、心导管检查 25%（n=4）、心脏计算机断层扫描 6%（n=1）。重要的是，TTE 漏诊 44%（n=7），随后通过其他方法诊断。治疗策略为外科血栓切除术 38%（n=6）、单独抗凝治疗 38%（n=6）、直接导管溶栓治疗 19%（n=3）、全身溶栓治疗 6%（n=1）。死亡率或移植名单率为 44%（n=7）。
NART 是 Norwood 姑息术的一种被低估的并发症，死亡率很高。在 Norwood 姑息术后出现不明原因复杂临床过程的患者中，应高度怀疑 NART。重要的是，TTE 常漏诊 NART。
