THE EISENMENGER-SYNDROME IN ADULTS

For this article, the literature on the pathophysiology, clinical feat ures, natural history, prognosis, and management of the Eisenmenger sy ndrome in adults was reviewed. English-language articles from 1966 to the present were identified through a search of the MEDLINE database b y using the terms Eisenmenger, congenital heart disease, and pulmonary hypertension. Selected cross-referenced articles were also included. Articles on the pathophysiology, clinical presentation, evaluation, na tural history, complications, and treatment of the Eisenmenger syndrom e in adults were selected, and descriptive and analytical data relevan t to the practicing physician were manually extracted. The Eisenmenger syndrome is characterized by elevated pulmonary vascular resistance a nd right-to-left shunting of blood through a systemic-to-pulmonary cir culation connection. Most patients with the syndrome survive for 20 to 30 years. The hemostatic changes associated with the syndrome may lea d to thromboembolic events, cerebrovascular complications, or the hype rviscosity syndrome. Erythrocytosis is present in most patients, but e xcessive phlebotomy may cause microcytosis and exacerbate the symptoms of hyperviscosity. Other complications associated with the Eisenmenge r syndrome include hemoptysis, gout, cholelithiasis, hypertrophic oste oarthropathy, and decreased renal function. Pregnancy or noncardiac su rgery is associated with a high mortality rate in patients with the Ei senmenger syndrome. Because most pediatric patients with the Eisenmeng er syndrome survive to adulthood, primary care physicians should have a thorough understanding of the syndrome; its associated complications ; and medical and surgical management, especially with regard to the a ppropriate timing of phlebotomy and lung or heart-lung transplantation . In addition, patients with the syndrome should undergo routine follo w-up at a tertiary care center that has physicians and nurses with spe cial expertise in congenital heart disease. In patients with the Eisen menger syndrome who are pregnant or require noncardiac surgery, a mult idisciplinary approach should be used to reduce the excessive mortalit y associated with these conditions.