Eisenmenger syndrome in adults
Ventricular septal defect, truncus arteriosus, univentricular heart
Koichiro Niwa, MD, FACCa,b,c,d,
Joseph K. Perloff, MD, FACCa,b,c,d,
Samuel Kaplan, MD, FACCa,b,c,d,
John S. Child, MD, FACCa,b,c,d and
Pamela D. Miner, MN, NPa,b,c,d
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
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Figure 1 Supraventricular and ventricular arrhythmias. SVT = supraventricular tachycardia; TA = truncus arteriosus; UH = univentricular heart; VSD = ventricular septal defect; VT = ventricular tachycardia.
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Figure 2 (a) Computerized tomographic scan from a 46-year old man in group A (ventricular septal defect, VSD). There is extensive intrapulmonary hemorrhage (Hem) in the left lung, and a lesser degree in the right lung (unmarked white arrows). (b) Necropsy specimen from a 45-year-old man in group A (VSD). Sudden death was due to massive hemorrhage into the right lung as shown here. Vertical white arrows identify thick-walled intrapulmonary arteries of pulmonary vascular disease.
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Figure 3 (a) Computerized tomographic scan from a 48-year-old man in group A (ventricular septal defect, VSD). Massive in situ thrombus filled an aneurysmal right pulmonary artery (RPA). The lumen (lower arrow) was strikingly reduced, and there was mural calcification (Ca++). (b) Necropsy specimen showing massive in situ thrombus in an aneurysmal RPA of a 45-year old man in group A (VSD). The strikingly reduced lumen is identified by the lower arrow. Sudden death was due to massive intrapulmonary hemorrhage.
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Figure 4 (a) Necropsy specimen from a 35-year-old man in group A (ventricular septal defect, VSD). There is aneurysmal dilation of the pulmonary trunk (PT). The patient died at home. The cause of death was not established. RV = right ventricle. (b) Necropsy specimen from a 27-year-old man in group A (VSD). Sudden death was due to rupture (arrow) of an aneurysmal PT.
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Figure 5 Five-year actuarial survival rates (Kaplan-Meier method) after initial visit were 67% in group A, 91% in group B and 34% in group C. The survival rates were significantly different between the three groups. Log-rank statistics: chi-square value = 8.099, df = 2, p value = 0.0174. Abbreviations as in Figure 1.
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