• Margarita Brida, Heba Nashat, and Michael A Gatzoulis.
• Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton and Harefield NHS Trust.
• Curr Opin Pulm Med. 2020 Sep 1; 26 (5): 422-428.

Purpose Of ReviewPulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a common association adversely affecting quality of life and survival in these patients. We provide herewith recent advances in the understanding and management of PAH-CHD.Recent FindingsSignificant progress has been made in disease-targeting therapy with pulmonary vasodilators for the treatment of Eisenmenger syndrome, the most severe form of PAH-CHD. Important gaps, however, still exist in the assessment and management of patients with PAH-CHD with systemic to pulmonary shunts. The choice of therapy, either interventional, medical, or both is an on-going dilemma that requires more long-term data. PAH after defect closure represents the most concerning subgroup of patients with the worst prognosis, requiring close follow-up and proactive disease-targeting therapy treatment. Small defects are not considered responsible for patients who have severe PAH and therefore, present different subgroup of patients similar to idiopathic PAH.SummaryEven with advances in diagnosis and treatment PAH-CHD remains a challenging field requiring lifelong follow-up and meticulous treatment in centres specialized in both CHD and PAH.

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