Pulmonary Hypertension in Adults with Congenital Heart Disease

Pulmonary hypertension (PH) complicates congenital heart disease (CHD) in up to 10% of cases and impacts quality of life and survival of these patients. Different forms of PH related to CHD arise due to the complex and diverse range of underlying cardiac anatomy and physiology observed in this patient group. Distinguishing between different types of PH is important to guide diagnosis and management, and PH related to CHD is best diagnosed and managed in centers specializing in both CHD and PH. This chapter provides an overview of the current classification of PH, pathophysiology, diagnosis, and optimal management in patients with CHD. This includes the spectrum of pulmonary arterial hypertension (PAH) related to CHD, from Eisenmenger syndrome to PAH after defect repair. Evidence-based treatment strategies include supportive measures, avoidance of potentially harmful practices, use of pulmonary arterial hypertension (PAH) therapy and heart failure therapy, and management of emergencies including hemoptysis and infective endocarditis. Decisions around operability in patients with unrepaired shunts and PAH and the role of “treat-and-repair” strategies are mainly based on expert opinion and require individual specialist assessment. Expert input is also required in special instances of PH related to CHD, such as pulmonary vasculopathy after Fontan-type repair, segmental PH, disease related to peripheral pulmonary stenoses, and PH complicating Down syndrome.