Roth Todd S, Aboulhosn Jamil A
Memorial Cardiac and Vascular Institute, Joe DiMaggio Children's Hospital Adult Congenital Heart Disease Center, 3501 Johnson Street, Hollywood, FL 33021, USA.
Ahmanson/UCLA Adult Congenital Heart Disease Center, Medicine and Pediatrics David Geffen School of Medicine at UCLA, 100 UCLA Medical Plaza, Suite 630, Los Angeles, CA 90095, USA.
Cardiol Clin. 2016 Aug;34(3):391-400. doi: 10.1016/j.ccl.2016.04.002.
Pulmonary arterial hypertension in congenital heart disease (PAH-CHD) is a frequent complication in adults with congenital heart disease. Regardless of etiology, the optimal treatment strategy for this difficult population is challenging. The new frontier of targeted PAH therapies has demonstrated improved functional capacity in the various phenotypes of PAH-CHD, with work currently in progress scrutinizing outcomes. In those who fail conventional medical therapy, heart and heart-lung (block) transplantation become the final therapeutic options, with the role of ventricular assist devices and the total artificial heart still under investigation in this group.
先天性心脏病相关肺动脉高压（PAH-CHD）是先天性心脏病成人患者常见的并发症。无论病因如何，针对这一特殊人群的最佳治疗策略都颇具挑战性。PAH靶向治疗的新进展已显示，在PAH-CHD的各种表型中，患者的功能能力有所改善，目前仍在对治疗结果进行仔细研究。对于常规药物治疗无效的患者，心脏和心肺（联合）移植成为最终的治疗选择，而心室辅助装置和全人工心脏在该群体中的作用仍在研究中。
