Division of Congenital Heart Surgery, Department of Surgery, Baylor College of Medicine and Texas Children's Hospital, Houston, Texas.
Division of Pediatric Cardiology, Department of Pediatrics, Baylor College of Medicine and Texas Children's Hospital, Houston, Texas.
Ann Thorac Surg. 2024 Sep;118(3):644-653. doi: 10.1016/j.athoracsur.2024.04.020. Epub 2024 May 10.
We describe outcomes and management strategies for single-ventricle and bilaterally discontinuous pulmonary arteries (PAs) originating from bilateral ductus arteriosus.
We reviewed 22 patients with aforementioned anatomy and PA centralization from 1995 to 2023, excluding those with biventricular repair.
Median age at centralization was 9 days (minimum-maximum, 0 days-2 years). Centralization was performed with systemic-to-pulmonary shunt (n =20 [91%]; 2 after bilateral ductal stents) or bidirectional cavopulmonary connection (n = 2 [9%]) using pericardial roll (n = 14 [64%]), patch-augmented direct anastomosis (n = 7 [32%]), and interposition graft (n = 1 [5%]) techniques. Concurrent total anomalous pulmonary venous connection (TAPVC, n = 11 [50%]) was associated with significantly inferior survival (P = .01). Five patients (23%) died at a median of 59 days (minimum-maximum, 6-257 days) after centralization, all with noncardiac TAPVC. At the latest follow-up for 17 survivors (median, 13.5 years; minimum-maximum, 0.5-25.1 years after centralization), 12 completed Fontan, 4 completed second-stage palliation, and 1 received a transplant before second-stage palliation. PA reintervention was required in 14 patients (64%), including 3 with reoperations independent of staged palliation. Echocardiography from baseline to before the second stage demonstrated branch PA growth with significantly increased diameters (left, P = .0006; right, P = .0002); z-scores significantly increased for right (P = .004) but not left (P = .11).
Successful single-ventricle palliation is possible, although high risk, for patients with bilateral discontinuous ductal PAs. Early postcentralization mortality remains substantial, particularly with associated noncardiac TAPVC. Many require reintervention to maintain PA growth, typically concurrently with staged palliation.
我们描述了起源于双侧动脉导管的单心室和双侧不连续肺动脉（PA）的结局和管理策略。
我们回顾了 1995 年至 2023 年期间具有上述解剖结构和 PA 集中的 22 名患者，不包括那些接受双心室修复的患者。
集中治疗的中位年龄为 9 天（最小-最大，0-2 岁）。集中治疗采用体肺分流术（n=20 [91%]；2 例在双侧动脉导管支架置入后）或双向腔肺连接术（n=2 [9%]），使用心包卷（n=14 [64%]）、补片增强直接吻合术（n=7 [32%]）和中间移植术（n=1 [5%]）。同期完全性肺静脉异位连接（TAPVC，n=11 [50%]）与存活率显著降低相关（P=0.01）。5 名患者（23%）在集中治疗后中位时间 59 天（最小-最大，6-257 天）死亡，均伴有非心脏 TAPVC。在 17 名存活者的最新随访中（中位时间，13.5 年；集中治疗后 0.5-25.1 年），12 名完成了 Fontan 手术，4 名完成了第二期姑息治疗，1 名在第二期姑息治疗前接受了移植。14 名患者（64%）需要再次干预 PA，其中 3 名患者与分期姑息治疗无关的再次手术。基线至第二期前的超声心动图显示分支 PA 生长，直径明显增加（左，P=0.0006；右，P=0.0002）；右肺动脉（P=0.004）的 z 评分显著增加，但左肺动脉（P=0.11）没有显著增加。
尽管风险较高，但对于双侧不连续动脉导管 PA 的患者，成功的单心室姑息治疗是可能的。集中治疗后的早期死亡率仍然很高，特别是伴有非心脏 TAPVC。许多患者需要再次干预以维持 PA 生长，通常与分期姑息治疗同时进行。
