The spectrum of pulmonary arterial hypertension in adults with congenital heart disease: management from a physician and nurse specialist perspective

Congenital heart disease (CHD) is one of the most common causes of pulmonary arterial hypertension (PAH), with a prevalence nearing that of idiopathic PAH in registries. Despite similarities in pulmonary vascular pathology, PAH-CHD differs from idiopathic PAH and other types of PAH. In fact, PAH-CHD is a term that includes a wide spectrum of conditions and pathophysiologies, ranging from Eisenmenger syndrome to milder forms of PAH with systemic-to-pulmonary shunts, to those with previously repaired defects. Significant expertise in both CHD and PAH is required to manage these patients. We provide an overview of the modern management of the wide spectrum of PAH-CHD in adults, both from a physician and clinical nurse specialist perspective.