Abstract
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.
Highlights
Any congenital heart disease (CHD) with intra- or extracardiac shunting that allows for persistent exposure to increased pressure and volume overload of the pulmonary circulation may lead to the development of pulmonary arterial hypertension (PAH)
Transthoracic echocardiographic echocardiographic (apical view) images images of subgroups in pulmonary arterial hypertension associated with congenital heart disease (PAH‐CHD): subgroups in pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD): (A) Eisenmenger
Note: Randomized controlled trials with beraprost and sitaxentan were not included in this table, as they have not been approved for treatment of pulmonary arterial hypertension in Western countries or have been withdrawn. ∆ = change; ↑ = improved; = = no statistically significant difference; 6MWD = 6-min walk distance; AMBITION = Ambrisentan and Tadalafil in Patients with Pulmonary Arterial Hypertension; BREATHE-5 = Bosentan Randomized Trial of Endothelin Antagonist Therapy-5; CHD = congenital heart disease; EARLY = Endothelin Antagonist Trial in Mildly Symptomatic Pulmonary Arterial Hypertension Patients; endothelin receptor antagonists (ERAs) = endothelin-receptor antagonist; GRIPHON = Prostacyclin Receptor
Summary
Any congenital heart disease (CHD) with intra- or extracardiac shunting that allows for persistent exposure to increased pressure and volume overload of the pulmonary circulation may lead to the development of pulmonary arterial hypertension (PAH). 2017, 6, 40 patients will go on to develop PAH, depending on location, size (i.e., 2 both of 22 anatomical and degree of shunting), and type of cardiac defect [5,6,7]. In the type of cardiac defectand is important, as led only percentage of major advances in addition, pediatric cardiovascular medicine surgery have toaa small marked increase in patients with unrepaired atrial septal defects develop. Having addition, the type of cardiac defect is important, as only has a small percentage though not negligible—whereas the number of adult patients with after defect closure appears of patients with unrepaired atrial septal defects develop ES, compared to higher percentages of to increase, is illustrated in Figureseptal patients withasunrepaired ventricular defects and complete atrioventricular septal defects [5,8].
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