Abstract
BackgroundPulmonary hypertension (PH) is commonly seen in adults who have congenital heart disease (CHD). Therapy is available for pulmonary arterial hypertension (PAH) and has greatly benefitted many patients with PAH related to CHD (PAH-CHD) over the last 15 years, with evidence of improved quality of life and prognosis in those with Eisenmenger syndrome and repaired PAH-CHD.In this review, we describe the standard management and advanced therapies for PAH, which are available in specialist PH centres around the UK and Ireland, and how these are used in PAH-CHD. Decisions around the choice of therapy are governed by commissioning and available evidence.ConclusionWe explain the different pathways for action and the variety of medications now at our disposal to help this important group of patients.
Highlights
Pulmonary hypertension (PH) is commonly seen in adults who have congenital heart disease (CHD)
It represents an advanced form of pulmonary arterial hypertension (PAH) associated with CHD [1, 2] that can lead to multiorgan involvement and systemic complications related to the underlying disease, the PH and the chronic hypoxaemia
Eisenmenger syndrome (ES) or one of the other forms of PAH related to CHD (PAH-CHD), represents a severe condition that is associated with considerable morbidity and limited life expectancy
Summary
ES or one of the other forms of PAH-CHD, represents a severe condition that is associated with considerable morbidity and limited life expectancy. More children born with CHD are advancing into adulthood as a result of advancements in medical care. Current available PAH-specific treatments include a variety of agents covering different mechanisms of action: PDE5i’s, sGC stimulators, ERAs, prostacyclin analogues and prostacyclin receptor agonists. A combination therapy of ERA and PDE5i can be considered in order to improve outcomes in the former group of patients. It is pivotal to recognise the unique pharmacological characteristics of drugs within each class and the specific evidence supporting the use of each treatment. Continued research to quantify the value of earlier treatment and combined regimens of targeted PAH therapies are likely to complement current insights and potentially revolutionise the treatment of PAH-CHD
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