PROGNOSIS FOR PATIENTS WITH EISENMENGER SYNDROME OF VARIOUS ETIOLOGY

The objective of this study was to determine the long-term survival pa ttern and variables affecting long-term survival and complications occ urring during follow-up of patients with Eisenmenger syndrome. A retro spective study of patients diagnosed with Eisenmenger syndrome were fo llowed up. A tertiary care centre was used and it provided superspecia lity services in various disciplines. The subjects included 201 patien ts with Eisenmenger syndrome - diagnosed by a combination of echocardi ography and a peripheral arterial oxygen saturation study and/or cardi ac catheterisation with or without angiocardiography - worked up and f ollowed up for variable duration over a period of 16 years from 1976 t o 1992. One hundred nine patients were females and 92 were males - age of presentation varied from 3 months to 62 years (mean +/- standard d eviation 19.23 +/- 12.62 years). A total of 12 different anatomic lesi ons were seen - the most common three being ventricular septal defect (33.33%), atrail septal defect (29.85%), and patent ductus arteriosus (14.23%). History, physical examination, chest skiagram and electrocar diogram established only the presence of pulmonary arterial hypertensi on except where differential cyanosis indicating ductus was discernibl e or the degree of splitting of second heart sound provided some clue to the level of shunt. Contrast echocardiography, completed in 25.4% e stablished the level of shunt in all patients. In others the diagnosis was confirmed by cardiac catheterisation. Twenty patients died during a mean follow-up period of 54.6 +/- 54.47 months. Sudden cardiac deat hs (30%), congestive heart failure (25%) and haemoptysis (15%) were th e most predominant causes of death. Only one patient died during puerp erium. The acturial survival for the entire patient population at 5 ye ars, 10 years and 15 years was 86.95%, 79.64% and 76.98%, respectively . Level of shunt (atrial, ventricular or aortopulmonary) did not influ ence the survival (P > 0.5). Of all the variables tested in a univaria te analysis, history of syncope at presentation (P < 0.005), elevated mean right atrial pressure (8 mmHg or above) (P < 0.05) and systemic a rterial desaturation below 85% (P < 0.05) were found to be important i ndicators of a poor prognosis. Eisenmenger syndrome is compatible with a fair intermediate term survival. History of syncope, elevated right sided filling pressure and systemic arterial oxygen saturation less t han 85% indicated a poorer outcome.