Eisenmenger syndrome and other types of pulmonary arterial hypertension related to congenital heart disease

Abstract

This book chapter summarizes the pathophysiology, clinical presentation, and management of adult patients with congenital heart disease and pulmonary arterial hypertension with an emphasis on differences between congenital heart disease-related and other types of pulmonary arterial hypertension. Eisenmenger syndrome, the extreme manifestation of pulmonary vascular disease in congenital heart disease, is a progressive multi-organ entity which impacts the quality of life and survival of affected patients. Over the past few years, disease-targeting pulmonary arterial hypertension therapies have become available and can be administered to patients with congenital heart disease, despite limited available evidence. Supportive measures remain key to the management of Eisenmenger patients with an aim of reducing symptoms and treating or preventing related complications. Regular follow-up of these patients is essential in specialist centers with expertise in both congenital heart disease and pulmonary arterial hypertension due to the syndrome’s complex pathophysiology.