This book provides a sound overview of the current state of the art in the pathobiology, pathophysiology, diagnosis and treatment of severe pulmonary hypertension and Eisenmenger's syndrome in patients with congenital heart defects. This is particularly important since for some years now the quality of life and probably also the long-term prognosis of affected patients can be favourably influenced through pharmacotherapy. In addition to a management algorithm, clinically relevant features of the multiple organ involvement in patients with Eisenmenger's syndrome are presented in a comprehensive and understandable fashion. Relevant pitfalls in the diagnosis and therapy are clearly shown. The book is intended for everyone responsible for the clinical care of patients with pulmonary hypertension within the context of congenital heart defects.