Journal List > Korean Circ J > v.22(4) > 1072808

Hong, Han, Kim, Kim, Sohn, Oh, Lee, Park, Choi, Seo, and Lee: Eisenmenger Syndrome in Adult - Clinical Feature and Natural History -

Abstract

Background

Eisenmenger syndrome is a complication of heart dease with left-to-right shunt in which the pulmonary arterial pressure is increased due to increased pulmonary vascular resistance and the shunt directions becomes bidirectional or reversed at the level of atria, ventricies, or great arteries. Corrective surgery is impossible unless there is substantial reactive change pulmonary vascular resistance.

Method

To identify clinical and hemodynamic features and observe natural history of adult patients with Eisenmenger syndrome, a retrospective clinical study was done on 61 patients(male:female=39:22) with Eisenmenger syndrome who were admitted to Seoul National University Hospital from September, 1979 to A piril, 1989, and were confirmed after cardiac catheterization and angiography.

Results

VSD was most freguent underlying defect(31 cases, 51%), followed by PDA(12 cases, 20%), ASD(9 cases,15%) and Combined lesion(9 cases, 15%).
The average age was 27.3 years with the peak incidence in third decades.
Effort intolerance(96.7%), palpitation(75.4%), hemoptysis(19.7%), and syncope(8.2%) were common symptoms, and cyanosis(62.3%), clubbing(63.9%) and increased heart sound were freguently(86.9%) observed.
The mean pressure of the pulmonary artery and the total pulmonary vascular resistance were markedly elevated with the mean value of 76.7±14.2mmgHg and 29.3±12.HU respectively, 4 of the 19 patients who were given 100% oxygen inhalation showed reversibility of the pulmonary vascular resistance.
During follow up(mean:40±29 months), Complications include infective endocarditis(1 case), brain abcess(2 case), atrial fibrillation(3 case), acute renal failure(1 case) and gout(2 case).
6 patients(10%) died during medical follow-up period(mean:40±29 months). Congestive heart failure and pulmonary infarction after cardiac catheterization including one postoprative death were causes of death.

Conclusion

VSD, ASD, and PDA were common underlying defects of adult Eisenmenger syndrome. Complication was not uncommon and the common cause of death was congestive failure. The prognosis of the patients with Eisenmenger syndrome may not be so dismal as has been thought, though the exact survival to be determined.

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