PURPOSE: To assess, in a large centre for adult with congenital heart disease, Eisenmenger patients survival and identify predictors of death in this population.
METHODS: All Eisenmenger patients under follow-up at our centre since 2000 (n 51, mean age 37±16 years) were included. Data including symptoms of heart failure (HF), functional class (NYHA), medication, laboratory, six minutes walking test (6MWT) and haemodynamic parameters were considered.
RESULTS: During a median follow-up of 68±18 months, 8 patients died (survival rate 84%). The causes of death were: sudden in 4 pts and refractory heart failure in 4. Underlying cardiac anatomy was: a non-restrictive ventricular septal defect in 3, atrial septal defect in 1, complete AV canal in 2, single ventricle in 2 patients. Mean age at death was 28+12 years. At univariate analysis, higher NYHA functional class at first observation (3.4±0.5 vs 2.9±0.4; p<0.005), deteriorating NYHA functional class during the follow-up (p<0.005), HF signs (p<0.001), need for prostanoids (p<0.005), larger desaturation at 6MWT (18±5 vs 11±8%; p<0.01), shorter distance travelled at 6MWT (52±15 vs 74±24% of theoretical distance; p<0.05) and a lower reduction of pulmonary vascular resistances (ΔPVR) during acute vasoreactivity test (29±21 vs 52±14 WU/m2; p<0.05) were predictive of death. On logistic regression analysis only presence of HF signs (OR=3.2, 95% CI 1.12-10.3, p=0.042), deteriorating NYHA functional class (OR=5.1, 95% CI 1.12-23.1, p=0.032), need for prostanoids (OR=6.3, 95% CI 1.13-37.2, p = 0.039) and ΔPVR (OR=2.4, 95% CI: 1.9-8.9; p= 0.034) were found to be predictive of death. In contrast, a history of clinical arrhythmia, ECG features (QRS duration and QTc interval), previous history of pulmonary or systemic emboli, laboratory, near-syncope or syncope, were not significantly different between the two outcome groups.
CONCLUSIONS: Sudden death and refractory heart failure were the causes of death in our Eisenmenger population. An higher functional class, clinical deterioration during the follow-up, signs of heart failure and a lower ΔPVR during acute vasoreactivity test are of prognostic value in terms of mortality for Eisenmenger patients.
CLINICAL IMPLICATIONS: A careful clinical evaluation including functional class, clinical deterioration during the follow-up and signs of heart failure is relevant for the outcome of Eisenmenger patients. A lower reduction of PVR during acute vasoreactivity test could have prognostic value in terms of mortality for this population.
DISCLOSURE: The following authors have nothing to disclose: Michele D'Alto, Emanuele Romeo, Paola Argiento, Anna Correra, Berardo Sarubbi, Antonietta Caronna, Giancarlo Scognamiglio, Maria Russo, Raffaele Calabrò
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