Karim Farida, Hiremath Gurumurthy, Samayoa Juan Carlos, Said Sameh M
Division of Pediatric Cardiology, Masonic Children's Hospital, University of Minnesota, Minneapolis, MN 55454, USA.
Division of Pediatric and Adult Congenital Cardiac Surgery, Department of Surgery, Maria Fareri Children's Hospital, Westchester Medical Center, New York Medical College, Valhalla, NY 10595, USA.
J Cardiovasc Dev Dis. 2024 Jul 25;11(8):232. doi: 10.3390/jcdd11080232.
In this paper, we share our single-center experience of successful multidisciplinary management of patients with Alagille syndrome. In addition, we aim to highlight the need for an Alagille program for effectively managing these patients, in general, and particularly peripheral pulmonary artery stenosis associated with this syndrome. This is a retrospective review of six children with Alagille syndrome and advanced liver involvement who underwent pulmonary artery reconstruction between 2021 and 2022. Cardiac diagnosis, co-existing liver disease burdens, management approach, and short-term outcomes were analyzed. All the patients underwent one-stage extensive bilateral branch pulmonary rehabilitation. Concomitant procedures included repair of tetralogy of Fallot in one patient and repair of supravalvar pulmonary artery stenosis in two. One patient had balloon pulmonary branch angioplasty before surgery. In all patients, there was a decrease in right ventricular systolic pressure post-operatively. Three patients underwent liver transplantation for pre-existing liver dysfunction. At a median 3-year follow-up, all the patients were alive with their right ventricular systolic pressure less than half of their systemic systolic pressure. One patient underwent balloon angioplasty due to new and recurrent left pulmonary artery stenosis 13 months after surgery. Pulmonary arteries can be successfully rehabilitated surgically in the presence of complex branch disease. Patients with advanced liver disease can undergo successful complex pulmonary artery reconstruction, which can facilitate their future liver transplantation course. A multidisciplinary team approach is a key for successful management of Alagille patients.
在本文中，我们分享了我们单中心成功对阿拉吉尔综合征患者进行多学科管理的经验。此外，我们旨在强调需要一个阿拉吉尔综合征项目，以有效管理这些患者，总体而言，特别是与该综合征相关的外周肺动脉狭窄。这是一项对2021年至2022年间接受肺动脉重建的6名患有阿拉吉尔综合征且肝脏受累严重的儿童的回顾性研究。分析了心脏诊断、并存的肝脏疾病负担、管理方法和短期结果。所有患者均接受了一期广泛的双侧肺分支修复。同期手术包括1例法洛四联症修复和2例肺动脉瓣上狭窄修复。1例患者在手术前进行了球囊肺分支血管成形术。所有患者术后右心室收缩压均下降。3例患者因术前存在肝功能不全接受了肝移植。在中位3年的随访中，所有患者均存活，其右心室收缩压低于体循环收缩压的一半。1例患者在术后13个月因新发和复发性左肺动脉狭窄接受了球囊血管成形术。在存在复杂分支疾病的情况下，肺动脉可通过手术成功修复。患有晚期肝病的患者可以成功进行复杂的肺动脉重建，这可以促进他们未来的肝移植进程。多学科团队方法是成功管理阿拉吉尔综合征患者的关键。
