Eisenmenger syndrome in adults: Ventricular septal defect, truncus arteriosus, univentricular heart

OBJECTIVES Morbidity and mortality patterns were characterized in adults with the Eisenmenger syndrome when two ventricles with a ventricular septal defect (VSD) joined two great arteries or one great artery, or when one ventricle joined two great arteries. BACKGROUND Although afterload in these disorders differs, clinical differences have not been defined. METHODS Seventy-seven patients were studied. Group A comprised 47 patients with VSD, aged 23 to 69 years (mean 39.5 +/- 10.2), follow-up 5 to 18 years (mean 7.2 +/- 4.9); group B, 14 patients with truncus arteriosus, aged 27 to 50 years (mean 33.7 +/- 7.3), follow-up 6 to 18 years (mean 7.7 +/- 5.1), and group C, 16 patients with univentricular heart, aged 18 to 44 years (mean 30.6 +/- 8.4), follow-up 5 to 15 years (mean 4.4 +/- 4.2). Echocardiography established the diagnoses and anatomic and hemodynamic features. Data were compiled on tachyarrhythmias, pregnancy, infective endocarditis, noncardiac surgery and the multisystem disorders of cyanotic adults. RESULTS Thirty-five percent of the patients died. Sixty-three percent of deaths were sudden, and resulted from intrapulmonary hemorrhage, rupture of either the pulmonary trunk, ascending aorta or a bronchial artery, or vasospastic cerebral infarction, or the cause was unestablished. There were no documented tachyarrhythmic sudden deaths. CONCLUSIONS Medical management of coexisting cardiac disease, multisystem systemic disorders, noncardiac surgery and pregnancy has reduced morbidity. Increased longevity exposed patients to proximal pulmonary arterial aneurysms, thromboses and calcification; to truncal valve stenosis and regurgitation; to semilunar and atrioventricular valve regurgitation, and to major risks of nontachyarrhythmic sudden death. (C) 1999 by the American College of Cardiology.