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

OBJECTIVES Morbidity and mortality patterns were characterized in adults wi th the Eisenmenger syndrome when two ventricles with a ventricular septal d efect (VSD) joined two great arteries or one great artery, or when one vent ricle joined two great arteries. BACKGROUND Although afterload in these disorders differs, clinical differen ces 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). Echocardiog raphy established the diagnoses and anatomic and hemodynamic features. Data were compiled on tachyarrhythmias, pregnancy, infective endocarditis, nonc ardiac surgery and the multisystem disorders of cyanotic adults. RESULTS Thirty-five percent of the patients died. Sixty-three percent of de aths were sudden, and resulted from intrapulmonary hemorrhage, rupture of e ither the pulmonary trunk, ascending aorta or a bronchial artery, or vasosp astic cerebral infarction, or the cause was unestablished. There were no do cumented tachyarrhythmic sudden deaths. CONCLUSIONS Medical management of coexisting cardiac disease, multisystem s ystemic disorders, noncardiac surgery and pregnancy has reduced morbidity. Increased longevity exposed patients to proximal pulmonary arterial aneurys ms, thromboses and calcification; to truncal valve stenosis and regurgitati on; to semilunar and atrioventricular valve regurgitation, and to major ris ks of nontachyarrhythmic sudden death. (C) 1999 by the American College of Cardiology.