Department of Pediatrics, Lucille Packard Children's Hospital Stanford and Stanford University, Palo Alto, CA.
Department of Pediatrics, Lucille Packard Children's Hospital Stanford and Stanford University, Palo Alto, CA.
J Pediatr. 2021 Feb;229:86-94.e4. doi: 10.1016/j.jpeds.2020.09.053. Epub 2020 Sep 24.
To assess outcomes in a large cohort of patients with Alagille syndrome (ALGS) who underwent pulmonary artery reconstruction surgery for complex pulmonary artery disease.
Patients with ALGS who underwent pulmonary artery reconstruction surgery at Lucile Packard Children's Hospital Stanford were reviewed. Patients were examined as an overall cohort and based on the primary cardiovascular diagnosis: severe isolated branch pulmonary artery stenosis, tetralogy of Fallot (TOF) without major aortopulmonary collateral arteries (MAPCAs), or TOF with MAPCAs.
Fifty-one patients with ALGS underwent pulmonary artery surgery at our center, including 22 with severe branch pulmonary artery stenosis, 9 with TOF without MAPCAs, and 20 with TOF and MAPCAs. Forty-one patients (80%) achieved a complete repair. Five of the patients with TOF with MAPCAs (25%) underwent complete repair at the first surgery, compared with 8 (89%) with TOF without MAPCAs and 19 (86%) with isolated branch pulmonary artery stenosis. At a median follow-up of 1.7 years after the first surgery, 39 patients (76%) were alive, 36 with a complete repair and a median pulmonary artery:aortic systolic pressure of 0.38. Nine patients (18%), including 8 with isolated branch pulmonary artery stenosis, underwent liver transplantation.
Most patients with ALGS and complex pulmonary artery disease can undergo complete repair with low postoperative right ventricular pressure. Patients with TOF/MAPCAs had the worst outcomes, with higher mortality and more frequent pulmonary artery interventions compared with patients with TOF without MAPCAs or isolated branch pulmonary artery stenosis. Complex pulmonary artery disease is not a contraindication to liver transplantation in patients with ALGS.
评估在因复杂肺动脉疾病而接受肺动脉重建手术的大量 Alagille 综合征（ALGS）患者中，手术结果。
回顾在斯坦福露西尔·帕卡德儿童医院接受肺动脉重建手术的 ALGS 患者。根据主要心血管诊断，将患者作为一个整体队列进行检查：严重孤立性肺分支动脉狭窄、无主要体肺侧支动脉的法洛四联症（TOF）或伴有体肺侧支动脉的 TOF。
我们中心有 51 名 ALGS 患者接受了肺动脉手术，包括 22 名严重的肺分支动脉狭窄、9 名无主要体肺侧支动脉的 TOF 和 20 名伴有主要体肺侧支动脉的 TOF。41 名患者（80%）实现了完全修复。5 名伴有主要体肺侧支动脉的 TOF 患者（25%）在第一次手术中完成了完全修复，而 8 名无主要体肺侧支动脉的 TOF 患者（89%）和 19 名孤立性肺分支动脉狭窄患者（86%）也是如此。在第一次手术后的中位数 1.7 年随访中，39 名患者（76%）存活，其中 36 名患者实现了完全修复，肺动脉：主动脉收缩压中位数为 0.38。9 名患者（18%），包括 8 名孤立性肺分支动脉狭窄患者，接受了肝移植。
大多数 ALGS 伴复杂肺动脉疾病的患者可通过肺动脉完全修复实现较低的术后右心室压力。TOF/MAPCAs 患者的预后最差，死亡率较高，且与无主要体肺侧支动脉的 TOF 或孤立性肺分支动脉狭窄患者相比，更频繁地进行肺动脉介入治疗。复杂肺动脉疾病并不是 ALGS 患者进行肝移植的禁忌症。
