Biventricular Assist Device Support for Intractable Arrhythmias From Histiocytoid Cardiomyopathy.
Biventricular Assist Device Support for Intractable Arrhythmias From Histiocytoid Cardiomyopathy.
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DOI：
10.1097/mat.0000000000001715
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发表时间：
2022-11-01
期刊：
影响因子：
4.2
通讯作者：
Joong, Anna
中科院分区：
文献类型：
--
作者：
Magnetta, Defne A.;Reichhold, Allison;Thrush, Philip T.;Monge, Michael;Webster, Gregory;Joong, Anna
组织细胞样心肌病（HICMP）是一种罕见的线粒体心肌病，与复发性危及生命的心律失常和不同程度的收缩功能障碍有关。已有成功的心脏移植治疗HICMP的报道，但没有发表的双心室辅助装置（BiVAD）支持治疗HICMP难治性心律失常的经验。我们报告了一个13个月大的女婴，左心室不紧实和收缩功能保留，谁提出了心源性休克继发于不间断室性心律失常。在化学和电复心失败后，她接受了BiVAD植入作为移植的桥梁。她的BiVAD过程因窦性心律期间的机械流入阻塞而复杂化，需要从根尖到心房的左侧流入插管修正。该操作解决了阻塞，并将患者转移到Berlin EXCOR®BiVADs。在Berlin®泵上，她在窦性心律时出现间歇性停顿（无填充/无喷射），感觉是由于内在喷射的竞争。尽管有这些暂停，患者在BiVAD疗程的剩余时间（总共205天）中经历了平静的过程，纤维蛋白沉积最少，无器械相关并发症。BiVAD可以支持有明显血流动力学心律失常的儿童患者进行移植。心房插管策略可能是首选的情况下，保留收缩功能，心室不压实，和频繁的心律变化。
Histiocytoid cardiomyopathy (HICMP) is a rare mitochondrial cardiomyopathy associated with recurrent life-threatening arrhythmias and variable degrees of systolic dysfunction. Successful heart transplantation for HICMP has been described, but there has been no published experience with biventricular assist device (BiVAD) support for intractable arrhythmias in HICMP. We report a 13-month-old girl with left ventricular noncompaction and preserved systolic function who presented in cardiogenic shock secondary to incessant ventricular arrhythmias. After failed attempts at chemical and electrical cardioversion, she underwent BiVAD implantation as bridge to transplantation. Her BiVAD course was complicated by mechanical inflow obstruction during sinus rhythm, necessitating left sided cannulation revision from an apical to atrial inflow cannula. This maneuver resolved the obstruction and the patient was transitioned to Berlin EXCOR® BiVADs. On Berlin® pumps, she had intermittent pauses (no fill/no eject) while in sinus rhythm, felt to be due to competition from intrinsic ejection. Despite these pauses, the patient experienced an uneventful remainder of her BiVAD course (205 days total) with minimal fibrin deposition and no device related complications. BiVAD can support pediatric patients with hemodynamically significant arrhythmias to transplantation. Atrial cannulation strategy may be preferred in cases of preserved systolic function, ventricular noncompaction, and frequent rhythm changes.