Eisenmenger syndrome is the most severe form of pulmonary arterial hypertension and arises on the basis of congenital heart disease with a systemic-to-pulmonary shunt. Due to the chronic slow progressive hypoxemia with central cyanosis, adult patients with the Eisenmenger syndrome suffer from a complex and multisystemic disorder including coagulation disorders (bleeding complications and paradoxical embolisms), renal dysfunction, hypertrophic osteoarthropathy, heart failure, reduced quality of life and premature death. For a long time, therapy has been limited to symptomatic options or lung or combined heart-lung transplantation. As new selective pulmonary vasodilators have become available and proven to be beneficial in various forms of pulmonary arterial hypertension, this targeted medical treatment has been expected to show promising effects with a delay of deterioration also in Eisenmenger patients. Unfortunately, data in Eisenmenger patients suffer from small patient numbers and a lack of randomized controlled studies. To optimize the quality of life and the outcome, referral of Eisenmenger patients to spezialized centers is required. In such centers, specific interdisciplinary management strategies of physicians specialized on congenital heart diseases and PAH should be warranted. This medical update emphasizes the current diagnostic and therapeutic options for Eisenmenger patients with particularly focussing on the medical treatment and corresponding study results.
Keywords: Cardiovascular diseases, adult congenital heart defects, pulmonary hypertension, Eisenmenger syndrome, followup studies, Competence Network for Congenital Heart Defects, vasodilators, multivarious haemostatic abnormalities, anticoagulation, supplementation, inhalation, Bosentan, paediatric formulation, BREATHE-5, deterioration, Sitaxsentan, Hemodynamics, CHD, Primary Endpoints, Sildenafil, Tadalafil, Epoprostenol, Iloprost, Treprostinil, Beraprost, combination
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