Treatment of pulmonary hypertension associated with congenital heart disease

An increase of blood pressure in the pulmonary artery (pulmonary hypertension) is often associated with congenital heart defects, particularly in childhood, which is usually due to shear stress-induced pulmonary arterial changes. In addition, pulmonary diseases or pathologies of left-sided heart structures may also contribute to the remodeling of pulmonary vessels. Pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) represents a very heterogenous subgroup of PH with very different prognoses depending on the underlying disease pattern and thus requires a sophisticated specific treatment strategy. This strategy includes general measures, supportive therapies and PAH-specific drug treatment. Before the initiation of PAH medication, the operability with open shunts has to be assessed in PAH-CHD. Where appropriate, alternative treatment options, such as the treat-to-close concept or a fenestrated shunt closure can be considered. Recent developments in interventional/surgical treatment of drug refractory PAH might be able to reduce the need for lung transplantation.