目的:与其他心肌病相比,限制性心肌病很少见,通常与更差的临床结局相关。很少为这些儿童提供心室辅助装置支持,并且经常受到手术困难的阻碍。
方法:纳入EUROMACS数据库中所有患有由心室辅助装置支持的限制性心肌病的儿科(<19岁)患者,并与扩张型心肌病患者进行比较(回顾性数据库分析)。回顾性联系参与中心以提供更详细的回声和SwanGanz测量结果,以分析心室辅助装置支持对肺动脉压和右心室功能的影响。
结果:纳入了诊断为限制性心肌病的44例小儿心室辅助装置支持的患者,植入时的中位年龄为5.0岁。44例限制性心肌病患者中有26例存活下来(59.1%),16人死亡(36.4%),2人仍在持续的VAD支持(4.5%),中位支持时间为95.5天(IQR33.3-217.8)。在1年和2年的心室辅助装置支持后,限制性心肌病患者的移植概率与扩张型心肌病患者相当(52.3%vs51.4%和59.5%vs60.1%,p=0.868)。然而,限制性心肌病队列的死亡率较高(35.8%vs17.0%和35.8%vs19.0%,p=0.005)。不良事件发生率高(CVA为31.8%,泵血栓形成29.5%,大出血25.0%,最终的双心室支持为59.1%)。在心房插管组中,CVA和泵血栓形成的发生率是患者的两倍(21.1%vs40.0%,p=0.595和15.8%vs40.0%,p=0.464;由于数字少,可能不显著)。植入心室辅助装置后肺动脉压改善,6名最初因肺动脉高压被标记为不合格的患者最终可以移植。
结论:在患有限制性心肌病的儿童中很少进行心室辅助装置支持。死亡率和不良事件发生率很高。另一方面,心脏移植存活率为59.1%,所有患者在心脏移植后的前30天存活.在支持下肺动脉压改善,可能使心脏移植成为以前不合格儿童的可行选择。
OBJECTIVE: Restrictive cardiomyopathy is rare and is generally associated with worse clinical outcomes compared to other cardiomyopathies. Ventricular assist device (VAD) support for these children is seldom applied and often hampered by the surgical difficulties.
METHODS: All paediatric (<19 years) patients with a restricted cardiomyopathy supported by a VAD from the EUROMACS database were included and compared to patients with a dilated cardiomyopathy (retrospective database analyses). Participating centres were retrospectively contacted to provide additional detailed echo and Swan Ganz measurements to analyse the effect of VAD support on pulmonary artery pressure and right ventricular function.
RESULTS: Forty-four paediatric VAD-supported patients diagnosed with restricted cardiomyopathy were included, with a median age at implantation of 5.0 years. Twenty-six of the 44 patient with a restricted cardiomyopathy survived to transplantation (59.1%), 16 died (36.4%) and 2 are still on ongoing VAD support (4.5%) after a median duration of support of 95.5 days (interquartile range 33.3-217.8). Transplantation probability after 1 and 2 years of VAD support in patients with a restricted cardiomyopathy were comparable to patients with a dilated cardiomyopathy (52.3% vs 51.4% and 59.5% vs 60.1%, P = 0.868). However, mortality probability was higher in the restricted cardiomyopathy cohort (35.8% vs 17.0% and 35.8% vs 19.0%, P = 0.005). Adverse event rates were high (cerebrovascular accident in 31.8%, pump thrombosis in 29.5%, major bleeding 25.0%, eventual biventricular support in 59.1%). In the atrially cannulated group, cerebrovascular accident and pump thrombosis occurred in twice as much patients (21.1% vs 40.0%, P = 0.595 and 15.8% vs 40.0%, P = 0.464; probably non-significant due to the small numbers). Pulmonary arterial pressures improved after implantation of a VAD, and 6 patients who were initially labelled as ineligible due to pulmonary hypertension could eventually be transplanted.
CONCLUSIONS: VAD support in children with a restricted cardiomyopathy is rarely performed. Mortality and adverse event rates are high. On the other hand, survival to cardiac transplantation was 59.1% with all patients surviving the 1st 30 days after cardiac transplantation. Pulmonary arterial pressures improved while on support, potentially making cardiac transplantation a viable option for previously ineligible children.