Fate of the patient with the eisenmenger syndrome

The presence of the Eisenmenger syndrome renders any underlying shunting defect inoperable. However, little attention has been paid to the later events in the natural history of patients with this syndrome. The clinical course, features and fate of 57 such patients ranging from 21 months to 58 years of age are reviewed. Seventeen died of direct cardiac causes, 10 suddenly, and most commonly with ventricular septal defect. Heart failure, often a later event, developed in 22 patients; it was not severely disabling, was usually readily controlled, but was responsible for 5 deaths. Arrhythmias were not clinically significant. Pregnancy was well tolerated in a small number of patients. Pulmonary infarction was infrequent. Hyperuricemia was common in adults, but gouty arthritis occurred in only 2 male patients. Although all had functional limitations, many were able to lead active lives with little risk of death or other complications before the third decade of life.