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CLINICAL STUDIES

Eisenmenger syndrome in adults

Ventricular septal defect, truncus arteriosus, univentricular heart

^a Divisions of Cardiology, University of California, Los Angeles, California, USA
^b Department of Medicine, University of California, Los Angeles, California, USA
^c Department of Pediatrics, University of California, Los Angeles, California, USA
^d The Ahmanson/UCLA Adult Congenital Heart Disease Center, University of California, Los Angeles, California, USA

Manuscript received June 8, 1998; revised manuscript received February 17, 1999, accepted March 15, 1999.

Reprint requests and correspondence: Dr. Joseph K. Perloff, Division of Cardiology, Room 47-123 CHS, UCLA Center for the Health Sciences, Los Angeles, California 90095-1679
jperloff{at}medicine.medsch.ucla.edu

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.

Abbreviations and Acronyms   CT = computerized tomography   PVD = pulmonary vascular disease   TA = truncus arteriosus   UVH = univentricular heart   VSD = ventricular septal defect

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