Treatment strategies for pulmonary arterial hypertension associated with adult congenital heart diseases

INTRODUCTION: The number of patients with adult congenital heart disease (ACHD) is gradually increasing worldwide due to advances in surgical techniques and pharmacological therapies. ACHD can lead to pulmonary arterial hypertension (PAH), and treatment strategies for PAH associated with ACHD have also evolved. AREAS COVERED: Several PAH-targeted drugs including endothelin receptor antagonists, phosphodiesterase type 5 inhibitors, soluble guanylate cyclase stimulators, and prostacyclin analogs are available for treatment of PAH. In this review, we summarized the current evidence regarding the use of PAH-targeted drugs in patients with PAH associated with ACHD. We also propose a 'treat and repair' strategy, which involves initial medical treatment to improve PAH followed by surgical or interventional repair of the systemic-to-pulmonary shunt. A PubMed literature search was conducted from 2000 to 2025. EXPERT OPINION: In cases of PAH associated with a systemic-to-pulmonary cardiac shunt, advanced PAH-targeted drugs can improve hemodynamics, and reduce the risk of cardiac defect repair and further improvement in PAH. The treat and repair strategy represents a promising therapeutic approach for PAH patients associated with systemic-to-pulmonary shunts.