Presentation, survival prospects, and predictors of death in Eisenmenger syndrome: a combined retrospective and case-control study.
OBJECTIVE
To characterize contemporary Eisenmenger patients at a large centre for adult congenital heart disease, assess survival prospects, and identify predictors of death in this population.


RESULTS
All Eisenmenger patients under follow-up at our centre since 2000 (n=171, mean age 37+/-12 years) were included. To identify predictors of mortality, a case-control study was performed. Data including symptoms, functional class, medication, laboratory, and electrocardiographic and echocardiographic parameters are presented. Iron deficiency was common and strongly related to phlebotomy (relative risk 4.1, P<0.0001). Haemoglobin concentration was inversely related to arterial oxygen saturations in iron-replete patients (P<0.001) but not in iron-deficient patients. During a median follow-up of 67 months, 20 patients died. Survival at 40, 50, and 60 years of age was 94, 74, and 52%, respectively. When compared with healthy individuals, median survival was reduced by approximately 20 years in Eisenmenger patients and was worst in those with complex lesions. Predictors of mortality included functional class, signs of heart failure, history of clinical arrhythmia, QRS duration and QTc interval, and low serum albumin and potassium levels.


CONCLUSIONS
Despite good short-term prognosis, life expectancy is markedly reduced in Eisenmenger patients. Markers of heart failure and parameters associated with arrhythmia are of prognostic value in terms of mortality and may guide clinicians caring for Eisenmenger patients.
Diller
Gerhard-Paul
GP
Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital, Sydney Street, London SW3 6NP, UK.
Dimopoulos
Konstantinos
K
Broberg
Craig S
CS
Kaya
Mehmet G
MG
Naghotra
Utpal Singh
US
Uebing
Anselm
A
Harries
Carl
C
Goktekin
Omer
O
Gibbs
J Simon R
JS
Gatzoulis
Michael A
MA
D000293:Adolescent; D000328:Adult; D000368:Aged; D016022:Case-Control Studies; D004541:Eisenmenger Complex / Q000401:mortality*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012044:Regression Analysis; D012189:Retrospective Studies; D012307:Risk Factors; D016019:Survival Analysis