Gyoten Takayuki, Amiya Eisuke, Kinoshita Osamu, Tsuji Masaki, Kimura Mitsutoshi, Hatano Masaru, Ono Minoru
Department of Cardiac Surgery, The University of Tokyo, 7-3-1 Hongo, Bunkyo-Ku, Tokyo, 113-8655, Japan.
Department of Cardiovascular Medicine, The University of Tokyo, Tokyo, Japan.
Gen Thorac Cardiovasc Surg. 2023 Jun;71(6):347-353. doi: 10.1007/s11748-022-01889-1. Epub 2022 Nov 8.
In muscular dystrophies (MD) patients with end-stage heart failure (HF), continuous flow left ventricular assist device (cf-LVAD) therapy is still controversial due to a progressive nature of MD-associated muscle weakness.
All the MD patients who had cf- VAD implants between March 2013 and August 2019 in our hospital were retrospectively studied. Study end points were death, major LVAD-associated complications or respiratory dysfunction caused by muscular weakness.
A total of 11 MD patients (Becker type: n = 6; Emery-Dreifuss Myodystrophy: n = 2; Fukuyama subtype: n = 1; Limb-girdle 1B: n = 2) were enrolled.
median age 41 years (IQR; 29-47); median Japanese Registry for Mechanically Assisted Circulatory Support: level 3 (2-3); a median interval between MD diagnosis and LVAD implantation 9 years (6-18). The pulmonary function test at LVAD implantation showed a median of %VC; 62% (45-82), FEV, 82% (81-88). Survival to discharge was 100% without pulmonary complication and early VAD-related complications. During a median follow-up of 38 months (27-53), re-admissions were needed due to device infection (n = 2), cerebrovascular accidents (disabling, n = 2 and non-disabling, n = 2), ventricular tachycardia (n = 4), and right HF (n = 3), respectively. 7 patients received successful heart transplant after a median waiting time of 44 months (34-61); 3 patients are still on the waiting list (waiting time: 21, 38, and 39 months). One patient died of right HF 15 months after VAD implantation. No one had overt pulmonary dysfunction during LVAD support.
In selected MD patients with end-stage HF, cf-LVAD therapy is a viable therapeutic option as bridge to heart transplant.
在患有终末期心力衰竭（HF）的肌营养不良症（MD）患者中，由于MD相关肌肉无力的进行性特点，连续血流左心室辅助装置（cf-LVAD）治疗仍存在争议。
对2013年3月至2019年8月期间在我院植入cf-VAD的所有MD患者进行回顾性研究。研究终点为死亡、与LVAD相关的主要并发症或由肌肉无力引起的呼吸功能障碍。
共纳入11例MD患者（贝克尔型：n = 6；埃默里-德赖富斯肌营养不良症：n = 2；福山亚型：n = 1；肢带型1B：n = 2）。
中位年龄41岁（四分位间距；29 - 47岁）；日本机械辅助循环支持注册中心中位级别：3级（2 - 3级）；MD诊断与LVAD植入之间的中位间隔时间为9年（6 - 18年）。LVAD植入时的肺功能测试显示，中位%VC为62%（45 - 82%），FEV为82%（81 - 88%）。出院生存率为100%，无肺部并发症和早期VAD相关并发症。在中位随访38个月（27 - 53个月）期间，分别因装置感染（n = 2）、脑血管意外（致残，n = 2；非致残，n = 2）、室性心动过速（n = 4）和右心衰竭（n = 3）需要再次入院。7例患者在中位等待时间44个月（34 - 61个月）后成功接受心脏移植；3例患者仍在等待名单上（等待时间：21、38和39个月）。1例患者在VAD植入后15个月死于右心衰竭。在LVAD支持期间，无人出现明显的肺功能障碍。
在选定的患有终末期HF的MD患者中，cf-LVAD治疗作为心脏移植的桥梁是一种可行的治疗选择。
