Cedars Ari, Kutty Shelby, Danford David, Schumacher Kurt, Auerbach S R, Bearl D, Chen S, Conway J, Dykes J C, Jaworski N, Joong A, Lorts A, Mascio C E, Morales D L S, Niebler R A, O'Connor M, Peng D M, Philip J, Reichman J R, Rosenthal D N, Zafar F, VanderPluym C, Villa C, Zinn M
Department of Medicine and Pediatrics, Johns Hopkins Children's Center, Baltimore, Maryland.
Department of Medicine and Pediatrics, Johns Hopkins Children's Center, Baltimore, Maryland.
J Heart Lung Transplant. 2021 May;40(5):368-376. doi: 10.1016/j.healun.2021.01.011. Epub 2021 Jan 19.
The size of the Fontan population with end-stage heart failure is growing. In this population, heart transplantation has been the only option. This study sought to investigate the efficacy of ventricular assist device (VAD) support in Fontan patients.
We conducted a retrospective study of Fontan patients in the Advanced Cardiac Therapies Improving Outcomes Network. We evaluated patient characteristics, and the clinical and physiologic outcomes after VAD implantation.
We identified 45 Fontan patients implanted with VAD. The average age of patients was 10 years (interquartile range: 4.5-18) and 30% were female. The majority had a morphologic right ventricle (69%), moderate or greater ventricular dysfunction (83%), and moderate or greater atrioventricular valve regurgitation (65%). The majority of implants were as a bridge to transplantation (76%), and the majority of patients were Interagency Registry for Mechanically Assisted Circulatory Support Profile 2 (56%). The most commonly employed device was the Medtronic HeartWare HVAD (56%). A total of 13 patients were discharged on device support, and 67% of patients experienced adverse events, the most common of which were neurologic (25%). At 1 year after device implantation, the rate of transplantation was 69.5%, 9.2% of patients continued to be VAD supported, and 21.3% of patients had died. Hemodynamically, VAD was effective in decreasing both Fontan and ventricular end-diastolic pressures in some individuals.
VAD is effective in supporting patients with end-stage Fontan failure awaiting heart transplantation. Future research should focus on identifying clinical and physiologic characteristics predictive of a favorable response to VAD support.
患有终末期心力衰竭的Fontan患者群体正在扩大。在这一群体中,心脏移植一直是唯一的选择。本研究旨在探讨心室辅助装置(VAD)支持对Fontan患者的疗效。
我们对高级心脏治疗改善预后网络中的Fontan患者进行了一项回顾性研究。我们评估了患者特征以及VAD植入后的临床和生理结局。
我们确定了45例植入VAD的Fontan患者。患者的平均年龄为10岁(四分位间距:4.5 - 18岁),30%为女性。大多数患者有形态学右心室(69%)、中度或更严重的心室功能障碍(83%)以及中度或更严重的房室瓣反流(65%)。大多数植入是作为移植桥梁(76%),大多数患者属于机构间机械辅助循环支持注册档案2(56%)。最常用的装置是美敦力HeartWare HVAD(56%)。共有13例患者在装置支持下出院,67%的患者发生不良事件,最常见的是神经系统不良事件(25%)。在装置植入后1年,移植率为69.5%,9.2%的患者继续接受VAD支持,21.3%的患者死亡。在血流动力学方面,VAD在一些个体中有效降低了Fontan压力和心室舒张末期压力。
VAD在支持等待心脏移植的终末期Fontan衰竭患者方面是有效的。未来的研究应集中于确定预测对VAD支持有良好反应的临床和生理特征。