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一名患有右位心、房间隔缺损和艾森曼格综合征的年轻患者采用静脉-静脉体外膜肺氧合和心肺移植的治疗

Management of a young patient with dextrocardia, atrial septal defect, and Eisenmenger syndrome with venous-venous extracorporeal membrane oxygenation and heart-lung transplantation.

作者信息

Rajab Taufiek Konrad, Kim Taehoon, Keller Steven, Mallidi Hari

机构信息

Division of Cardiac Surgery, Brigham and Women's Hospital, Boston, Massachusetts.

Division of Pulmonary and Critical Care Medicine, Brigham and Women's Hospital, Boston, Massachusetts.

出版信息

J Card Surg. 2019 Oct;34(10):1114-1116. doi: 10.1111/jocs.14168. Epub 2019 Jul 23.

Abstract

Dextrocardia is a rare congenital condition which presents important challenges for surgical management. We discuss a patient with dextrocardia, atrial septal defect, and Eisenmenger syndrome, which ultimately led to decompensated end-stage lung disease and heart-lung transplant. Venous-venous extracorporeal membrane oxygenation was an important strategy to bridge the patient until donor organs became available. Transplantation of a heart-lung block allowed for the treatment of the patient's underlying congenital heart defect, anatomic reversal of dextrocardia with appropriate venous and arterial connections, and management of pulmonary damage from pulmonary hypertension.

摘要

右位心是一种罕见的先天性疾病,给外科治疗带来了重大挑战。我们讨论了一名患有右位心、房间隔缺损和艾森曼格综合征的患者,该疾病最终导致了失代偿性终末期肺病并进行了心肺移植。静脉-静脉体外膜肺氧合是在供体器官可用之前维持患者生命的重要策略。心肺联合移植能够治疗患者潜在的先天性心脏缺陷,通过适当的动静脉连接实现右位心的解剖学反转,并处理肺动脉高压导致的肺损伤。

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本文引用的文献

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Heart-lung transplantation in situs inversus totalis.全内脏转位患者的心肺移植。
Ann Thorac Surg. 2009 Sep;88(3):1002-3. doi: 10.1016/j.athoracsur.2009.01.060.
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Technique of combined heart-lung transplantation.心肺联合移植技术
J Card Surg. 1992 Dec;7(4):313-23. doi: 10.1111/j.1540-8191.1992.tb01021.x.

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