The Eisenmenger syndrome is characterized by elevated pulmonary vascular resistance and right to left shunting through a systemic to pulmonary circulation connexion. Interestingly, Eisenmenger patients have a better life expectancy than their counterparts with primary pulmonary hypertension, probably due to the gradual and better cardiovascular adaptation over longer period of time. The mean age at death of patients with Eisenmenger Syndrome is 37 years; most die from progressive cardiovascular disease and congestive heart failure or sudden ventricular arrhythmia; approximately 11 percent die from hemoptysis. However, survival of patients into their 60s has been reported. Natural history is indeed better than expected by most physicians. Improved quality of life and survival is due to prevention of complications and avoidance of mistakes in management. These patients may live longer with informed medical care and protection from special risks. Recently, the continuous infusion of prostacyclin showed beneficial effects opening new therapeutic modalities in this group of patients. In patients awaiting lung transplantation, for example, six month survival was higher in those with severe symptomatic Eisenmenger syndrome than in patients with primary pulmonary hypertension (89 versus 60 percent).
Cantor WJ, Harrisson DA, Moussadji JS, Connelly MS, webb GD, Liu P, McLaughlin PR, Siu SC. Determinants of survival and length of survival in adults with Eisenmenger syndrome. Am J Cardiol 1999; 84: 677-681
Daliento L, Somerville J, Presbitero P, Menti L, Brach-Prever S, Rizzoli G, Stone S. Eisenmenger syndrome: Factors relating to deterioration and death. Eur Heart J 1998; 19: 1845-1855
Hayden AM et al. Primary diagnosis predicts prognosis of lung transplant candidates. Transplantation 1993 May;55(5):1048-50
Rosenzweig EB, Kerstein D, Barst RJ: Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 1999; 99: 1858-65
Vongpatanasin W, Brickner E, Hillis D, Lange RA. The Eisenmenger syndrome in adults. Ann Intern Med 1998;128:745-755