Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts.
Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts.
J Heart Lung Transplant. 2018 Jan;37(1):38-45. doi: 10.1016/j.healun.2017.06.017. Epub 2017 Jul 4.
Expanded use of pediatric ventricular assist devices (VADs) has decreased mortality in children awaiting heart transplantation. Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs), a National Heart, Lung, and Blood Institute-sponsored North American database, provides a platform to understand this emerging population.
Between September 2012 and September 2016, patients aged younger than 19 years who underwent VAD implantation were enrolled in Pedimacs. FDA approved durable devices as well as temporary support devices were included. The second annual report updates the current Pedimacs data. Patients implanted with temporary devices are included in Pedimacs and this analysis includes this group of paracorporeal continuous flow VADs.
Over the 4 years, 42 hospitals implanted 432 devices in 364 patients less than 19 yrs of age. Diagnoses included cardiomyopathy in 223 (61%), myocarditis in 41 (11%), and congenital heart disease in 77 (21%), of which 48 had single-ventricle physiology. At implant, 87% were at Intermacs patient profile 1 or 2. The age distribution of children (59% male) supported on VAD included 69 (19%) aged younger than 1 year, 66 (18%) aged 1 to 6 years, 56 (15%) aged 6 to 10 years, and 173 (48%) aged 11 to 19 years. Median follow-up was 2.2 months (range, 1 day to 41.5 months). Median (interquartile) age at implant was 1.7 (0.3-10.0) years for paracorporeal continuous-flow pumps (n = 60), 1.7 (0.4-5.3) years for paracorporeal pulsatile pumps (n = 105), and 15.0 (11.3-16.9) years for implantable continuous-flow pumps (n = 174). Support strategies included LVAD in 293 (80%), biventricular device in 55 (15%), and total artificial heart in 8 (2%). Nearly 50% of patients underwent transplantation within 6 months, with overall mortality of 19%. Adverse event burden continues to be high.
Pedimacs constitutes the largest longitudinal pediatric VAD registry. Preimplant data across centers will be helpful at creating shared protocols with which to improve outcomes. Adverse events continue to be the major challenge, especially among the young critically ill children with complex congenital disease.
儿科心室辅助装置（VAD）的广泛应用降低了等待心脏移植的儿童的死亡率。美国国立心肺血液研究所（National Heart，Lung，and Blood Institute）赞助的儿科机构间机械循环支持注册（Pediatric Interagency Registry for Mechanical Circulatory Support，Pedimacs）是一个了解这一新兴人群的平台。
2012 年 9 月至 2016 年 9 月期间，在 Pedimacs 注册了接受 VAD 植入的年龄小于 19 岁的患者。包括 FDA 批准的耐用设备和临时支持设备。第二份年度报告更新了当前的 Pedimacs 数据。接受临时设备植入的患者也包含在 Pedimacs 中，并且该分析包括这组体外连续流 VAD。
在 4 年期间，364 名小于 19 岁的患者在 42 家医院植入了 432 个设备。诊断包括心肌病 223 例（61%）、心肌炎 41 例（11%）和先天性心脏病 77 例（21%），其中 48 例为单心室生理。植入时，87%的患者处于 Intermacs 患者状况 1 或 2。接受 VAD 支持的儿童（59%为男性）的年龄分布包括：<1 岁 69 例（19%）、1-6 岁 66 例（18%）、6-10 岁 56 例（15%）和 11-19 岁 173 例（48%）。中位随访时间为 2.2 个月（范围：1 天至 41.5 个月）。体外连续流泵（n=60）植入时的中位（四分位间距）年龄为 1.7（0.3-10.0）岁，体外搏动流泵（n=105）为 1.7（0.4-5.3）岁，植入式连续流泵（n=174）为 15.0（11.3-16.9）岁。支持策略包括左心室辅助装置 293 例（80%）、双心室装置 55 例（15%）和全人工心脏 8 例（2%）。近 50%的患者在 6 个月内接受了移植，总死亡率为 19%。不良事件负担仍然很高。
Pedimacs 是最大的儿科 VAD 纵向登记处。来自各中心的植入前数据将有助于制定改善结果的共享方案。不良事件仍然是主要挑战，尤其是在患有复杂先天性疾病的年轻重症儿童中。
