Pulmonary Hypertension Associated with Congenital Heart Disease: A Clinical Primer

Pulmonary hypertension associated with congenital heart disease represents a significant challenge for clinicians due to its complex pathophysiology and diverse presentation. This patient population exhibits a broad spectrum of anatomical and hemodynamic abnormalities, with congenital heart disease-associated pulmonary arterial hypertension (PAH-CHD) comprising a significant proportion of pediatric pulmonary hypertension (PH) cases. Although progress in diagnostic methods and treatment options has been made, PH continues to be a major contributor to illness and death among affected pediatric patients, especially when diagnosis or treatment is postponed. This review aims to equip non-specialist clinicians with a better understanding of PH associated with congenital heart disease, focusing on its pathophysiology, clinical presentation, and diagnostic criteria. Key recommendations for evaluating and managing this fragile population are presented, emphasizing the importance of early recognition and multidisciplinary collaboration. As an increasing number of congenital heart disease patients reach adulthood, understanding its lifelong impacts becomes crucial for improving outcomes and creating tailored treatment approaches.