Park S S, Sanders D B, Smith B P, Ryan J, Plasencia J, Osborn M B, Wellnitz C M, Southard R N, Pierce C N, Arabia F A, Lane J, Frakes D, Velez D A, Pophal S G, Nigro J J
1Division of Cardiothoracic Surgery, Division of Cardiology, Division of Critical Care Medicine, Children's Heart Center, Division of Radiology, Phoenix Children's Hospital, Phoenix, AZ, USA.
Perfusion. 2014 Jan;29(1):82-8. doi: 10.1177/0267659113496580. Epub 2013 Jul 18.
Mechanical circulatory support emerged for the pediatric population in the late 1980s as a bridge to cardiac transplantation. The Total Artificial Heart (TAH-t) (SynCardia Systems Inc., Tuscon, AZ) has been approved for compassionate use by the Food and Drug Administration for patients with end-stage biventricular heart failure as a bridge to heart transplantation since 1985 and has had FDA approval since 2004. However, of the 1,061 patients placed on the TAH-t, only 21 (2%) were under the age 18. SynCardia Systems, Inc. recommends a minimum patient body surface area (BSA) of 1.7 m(2), thus, limiting pediatric application of this device. This unique case report shares this pediatric institution's first experience with the TAH-t. A 14-year-old male was admitted with dilated cardiomyopathy and severe biventricular heart failure. The patient rapidly decompensated, requiring extracorporeal life support. An echocardiogram revealed severe biventricular dysfunction and diffuse clot formation in the left ventricle and outflow tract. The decision was made to transition to biventricular assist device. The biventricular failure and clot formation helped guide the team to the TAH-t, in spite of a BSA (1.5 m(2)) below the recommendation of 1.7 m(2). A computed tomography (CT) scan of the thorax, in conjunction with a novel three-dimensional (3D) modeling system and team, assisted in determining appropriate fit. Chest CT and 3D modeling following implantation were utilized to determine all major vascular structures were unobstructed and the bronchi were open. The virtual 3D model confirmed appropriate device fit with no evidence of compression to the left pulmonary veins. The postoperative course was complicated by a left lung opacification. The left lung anomalies proved to be atelectasis and improved with aggressive recruitment maneuvers. The patient was supported for 11 days prior to transplantation. Chest CT and 3D modeling were crucial in assessing whether the device would fit, as well as postoperative complications in this smaller pediatric patient.
机械循环支持在20世纪80年代末开始应用于儿科患者群体,作为心脏移植的桥梁。自1985年以来,全人工心脏(TAH-t,SynCardia Systems公司,亚利桑那州图森市)已被美国食品药品监督管理局批准用于终末期双心室心力衰竭患者的同情用药,作为心脏移植的桥梁,并于2004年获得美国食品药品监督管理局的批准。然而,在接受TAH-t治疗的1061例患者中,只有21例(2%)年龄在18岁以下。SynCardia Systems公司建议患者最小体表面积(BSA)为1.7平方米,因此限制了该设备在儿科的应用。本独特病例报告分享了这家儿科机构使用TAH-t的首次经验。一名14岁男性因扩张型心肌病和严重双心室心力衰竭入院。患者病情迅速恶化,需要体外生命支持。超声心动图显示严重双心室功能障碍以及左心室和流出道弥漫性血栓形成。决定过渡到双心室辅助装置。尽管患者的体表面积(1.5平方米)低于1.7平方米的建议值,但双心室衰竭和血栓形成促使团队选择了TAH-t。胸部计算机断层扫描(CT)结合一种新型三维(3D)建模系统和团队,有助于确定合适的尺寸。植入后利用胸部CT和3D建模来确定所有主要血管结构通畅且支气管开放。虚拟3D模型证实设备尺寸合适,没有左肺静脉受压的迹象。术后病程因左肺不透明而复杂化。左肺异常经证实为肺不张,通过积极的复张手法有所改善。患者在移植前接受了11天的支持治疗。胸部CT和3D建模对于评估该设备是否合适以及在这名较小的儿科患者中的术后并发症至关重要。