Eisenmenger syndrome and pulmonary arterial hypertension in adults with congenital heart disease

Better characterisation of patients with Eisenmenger syndrome and other forms of pulmonary arterial hypertension (PAH) and congenital heart disease (CHD), along with their varied clinical phenotypes, an improved understanding of pathophysiological mechanisms, combined with earlier detection and closer monitoring of disease and disease progression are bound to improve quality of life and long-term prospects for these patients. Avoidance of iatrogenic mistakes, such as unnecessary routine phlebotomies, and timely introduction of appropriate therapy prior to clinical decompensation should remain key objectives. Prostacyclins and lung transplantation for this group of patients, although effective, are hampered by their invasive nature and the problem of patient selection. Newer oral disease-targeting therapies such as endothelin antagonists seem to be safe and beneficial for adult patients with Eisenmenger physiology, although additional long-term data are required for this challenging patient population. Furthermore, prospective studies need to examine the potential role, type and timing of routine anticoagulation for patients with PAH and CHD.