The Changing Landscape of Pulmonary Arterial Hypertension in the Adult with Congenital Heart Disease
Department of Cardiology, Academic Medical Center, Meibergdreef 9, 1105 AZ Amsterdam, The Netherlands
Netherlands Heart Institute, Moreelsepark 1, 3511 EP Utrecht, The Netherlands
Author to whom correspondence should be addressed.
Academic Editor: Bernhard Rauch
J. Clin. Med. 2017, 6(4), 40; https://doi.org/10.3390/jcm6040040
Received: 31 January 2017 / Revised: 9 March 2017 / Accepted: 23 March 2017 / Published: 30 March 2017
(This article belongs to the Special Issue Novel Therapeutic Approaches for Pulmonary Arterial Hypertension)
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.