Robinson Reice, Gaboyan Samvel, Alshawabkeh Laith, Reeves Ryan, Golts Eugene, Pretorius Victor, Nigro John, Kearns Mark, El-Said Howaida, Carazo Matthew, Hong Kimberly, Adler Eric, Kim Paul J, Topik Amanda, Raleigh Deborah, Meier Angela, Ellis Sarah, Sanchez Ramon, Afshar Kamyar, Kafi Aarya, Lin Christine, Yung Gordon, Brubaker Aleah, Schnickel Gabriel, Mekeel Kristin, Berumen Jennifer, Parekh Justin, Barman Pranab, Ajmera Veeral, Kono Yuko, Vodkin Irine, Urey Marcus A
Department of Medicine, University of California, San Diego, San Diego, California.
Department of Medicine, Division of Pulmonary, Critical Care, and Sleep Medicine & Physiology, University of California, San Diego, San Diego, California.
JHLT Open. 2025 Mar 7;8:100243. doi: 10.1016/j.jhlto.2025.100243. eCollection 2025 May.
Additional data are needed to define the optimal listing strategy and peri-transplant management for adults with congenital heart disease (ACHD). In this study, we evaluated the perioperative management and 1-year outcomes for 30 patients who underwent orthotopic heart transplantation (OHT) for ACHD.
We conducted a single-center retrospective case series of all patients who received an OHT at our institution for ACHD from January 1, 2017 through June 30, 2024. Descriptive statistical analyses were used to illustrate the baseline characteristics, peri-transplant management, and 1-year outcomes of participants.
Seventeen (56.7%) patients received a heart-only transplant, 4 (13.3%) had a heart-lung transplant, 8 (26.7%) had a heart-liver transplant, and 1 (3.3%) had a heart-liver-kidney transplant. Embolization of aortopulmonary and venous collateral vessels before transplantation was performed in 12 (80%) Fontan patients, with a median of 2 procedures per patient. Twenty-eight (93.3%, = 30) patients were alive at 90 days, and 26 (92.9%, = 28) were alive at 1 year.
This cohort of ACHD patients had a 92.9% 1-year survival rate, consistent with other high-volume centers in the United States. In this paper, we discuss our institutional practices that address barriers to transplant for ACHD patients, such as early referral for transplant, indications for listing, pretransplant embolization of collateral vessels, and multiorgan transplant.
需要更多数据来确定先天性心脏病成人患者(ACHD)的最佳登记策略和移植围手术期管理。在本研究中,我们评估了30例接受原位心脏移植(OHT)治疗ACHD患者的围手术期管理及1年结局。
我们对2017年1月1日至2024年6月30日在我院接受OHT治疗ACHD的所有患者进行了单中心回顾性病例系列研究。采用描述性统计分析来说明参与者的基线特征、移植围手术期管理及1年结局。
17例(56.7%)患者接受了单纯心脏移植,4例(13.3%)接受了心肺移植,8例(26.7%)接受了心-肝移植,1例(3.3%)接受了心-肝-肾移植。12例(80%)Fontan患者在移植前进行了主肺动脉和静脉侧支血管栓塞,每位患者的栓塞手术中位数为2次。28例(93.3%,n = 30)患者在90天时存活,26例(92.9%,n = 28)患者在1年时存活。
这组ACHD患者的1年生存率为92.9%,与美国其他大容量中心一致。在本文中,我们讨论了我们机构针对ACHD患者移植障碍所采取的措施,如早期转诊进行移植、登记指征、移植前侧支血管栓塞及多器官移植。