van Dissel Alexandra C, Mulder Barbara J M, Bouma Berto J
Department of Cardiology, Academic Medical Center, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands.
Netherlands Heart Institute, Moreelsepark 1, 3511 EP Utrecht, The Netherlands.
J Clin Med. 2017 Mar 30;6(4):40. doi: 10.3390/jcm6040040.
Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a common type of pulmonary arterial hypertension (PAH) and a frequent complication of congenital heart disease (CHD). PAH-CHD represents a heterogeneous patient population and it is important to distinguish between the underlying cardiac defects considering the prognostic and therapeutic implications. Improved interventional techniques have enabled repair or palliation of most cardiac defects, though a substantial number of patients remain at high risk for PAH after closure. Traditionally, the treatment and management of PAH-CHD patients has been limited to palliative and supportive care, and based on expert opinion rather than clinical trials. Recently, however, the availability of advanced PAH-specific treatment has opened up a new field for the clinical management of this condition. Nevertheless, there is limited evidence on the optimal therapeutic approach for PAH-CHD. Herein, we discuss the current and novel therapeutic options for PAH-CHD as well as highlight several challenges in the clinical management at present.
先天性心脏病相关肺动脉高压（PAH-CHD）是肺动脉高压（PAH）的一种常见类型，也是先天性心脏病（CHD）的常见并发症。PAH-CHD患者群体具有异质性，考虑到预后和治疗意义，区分潜在的心脏缺陷很重要。尽管大多数心脏缺陷通过改进的介入技术得以修复或姑息治疗，但仍有相当数量的患者在缺损闭合后仍面临PAH的高风险。传统上，PAH-CHD患者的治疗和管理仅限于姑息和支持治疗，且基于专家意见而非临床试验。然而，近年来，先进的PAH特异性治疗方法的出现为该病的临床管理开辟了新领域。尽管如此，关于PAH-CHD最佳治疗方法的证据仍然有限。在此，我们讨论PAH-CHD的当前和新型治疗选择，并强调目前临床管理中的几个挑战。
