Brancaccio G, Filippelli S, Michielon G, Iacobelli R, Alfieri S, Gandolfo F, Pongiglione G, Albanese S, Perri G, Parisi F, Carotti A, Amodeo A
Department of Cardiac Surgery, Ospedale Pediatrico Bambino Gesù, Rome, Italy.
Transplant Proc. 2012 Sep;44(7):2007-12. doi: 10.1016/j.transproceed.2012.06.034.
Despite the remarkable advances with the use of ventricular assist devices (VAD) in adults, pneumatic pulsatile support in children is still limited. We report on our experience in the pediatric population.
Retrospective review of 27 consecutive children offered mechanical support with Berlin Heart as a bridge to heart transplant, and Jarvik 2000 as a destination therapy from February 2002 to October 2011.
The median patient age was 4.8 years (range = 75 days to 20.5 years). The median patient weight was 18.6 kg (range = 2.9-63 kg). We divided the patients in two groups, including in group I patients assisted for bridging to heart transplantation and in group II patients with Duchenne's dystrophy assisted as destination therapy. In the group I, 11 patients required biventricular mechanical support (BVAD), but in all other cases, a single left VAD proved sufficient (56%). The median duration of VAD support was 48 days (1 to 192 days). The median pre-VAD pulmonary vascular resistance index (Rpi) was 5.7 WU/m(2) (3.5 to 14.4 WU/m(2)). Twelve patients (48%) were successfully bridged to heart transplantation after a median duration of mechanical support of 63 days (range = 2-168 days). Ten deaths occurred (40%), three for neurological complications, two for sepsis, two for multiorgan failure, and three other for device malfunctioning. Since 2007, the survival rate of our patients has increased from 33% to 75%, and the need for BVAD has decreased from 89% to 23%. In the group II, two patients with mean age of 15.3 years were assisted with Jarvik 2000, and both of them are alive in a follow-up of 10.4 months. In two patients with Rpi > 10 WU/m(2), unresponsive to pulmonary vasodilatator therapy, Rpi dropped to 2.2 and 2 WU/m(2) after 40 and 23 days of BVAD support, respectively. Six patients (32%) required at least one pump change. Of 12 patients undergoing heart transplantation, five developed an extremely elevated (>60%) panel-reactive antibody by enzyme-linked immunosorbent assay, confirmed by Luminex. All of them experienced at least one acute episode of rejection in the first month after heart transplant, needing plasmapheresis. The survival rate after heart transplantation was 100% with a median follow-up of 34.4 months (45 days to 8.7 years).
Mechanical support in children with end-stage heart failure is an effective strategy as a bridge to heart transplantation with a reasonable morbidity and mortality. BVAD support may offer an additional means to reverse extremely elevated pulmonary vascular resistance. The total implantable system opens a future scenarios for patients not eligible for heart transplantation.
尽管成人使用心室辅助装置（VAD）取得了显著进展，但儿童的气动搏动支持仍然有限。我们报告我们在儿科患者中的经验。
回顾性分析2002年2月至2011年10月期间连续27例接受柏林心脏机械支持作为心脏移植桥梁以及接受Jarvik 2000作为终末期治疗的儿童患者。
患者中位年龄为4.8岁（范围=75天至20.5岁）。患者中位体重为18.6 kg（范围=2.9 - 63 kg）。我们将患者分为两组，第一组为接受辅助以过渡到心脏移植的患者，第二组为患有杜氏肌营养不良症接受终末期治疗的患者。在第一组中，11例患者需要双心室机械支持（BVAD），但在所有其他情况下，单个左心室辅助装置已足够（56%）。VAD支持的中位持续时间为48天（1至192天）。VAD支持前的中位肺血管阻力指数（Rpi）为5.7 WU/m²（3.5至14.4 WU/m²）。12例患者（48%）在中位机械支持63天（范围=2至168天）后成功过渡到心脏移植。发生10例死亡（40%），3例死于神经并发症，2例死于败血症，2例死于多器官功能衰竭，3例死于装置故障。自2007年以来，我们患者的生存率从33%提高到75%，BVAD的需求从89%降至23%。在第二组中，2例平均年龄为15.3岁的患者接受了Jarvik 水平，并在10.4个月的随访中均存活。在2例Rpi>10 WU/m²且对肺血管扩张剂治疗无反应的患者中，分别在BVAD支持4 和23天后，Rpi降至2.2和2 WU/m²。6例患者（32%）至少需要更换一次泵。在12例接受心脏移植的患者中，5例通过酶联免疫吸附测定法检测到群体反应性抗体极度升高（>60%），经Luminex确认。他们所有人在心脏移植后的第一个月内至少经历了一次急性排斥反应发作，需要进行血浆置换。心脏移植后的生存率为100%，中位随访时间为34.4个月（45天至8.7年）。
终末期心力衰竭儿童的机械支持作为心脏移植的桥梁是一种有效的策略，发病率和死亡率合理。BVAD支持可能提供一种额外的方法来逆转极度升高的肺血管阻力。全植入式系统为不符合心脏移植条件的患者开辟了未来前景。
