Key Points
- To evaluate the natural history and identify risk factors linked to mortality and clinical deterioration in Eisenmenger syndrome patients.
- Retrospective analysis of 188 patients from three cardiac centers over a median period of 31 years.
- Patients categorized by congenital heart defect complexity with assessed events including complications and mortality rates.
- Data on ability index, complications, medical treatments, and outcomes collected and analyzed.
- Eighty-four percent had a satisfactory ability index at first attendance with a mean age of 25 years.
- Complex heart disease patients experienced earlier clinical deterioration (mean age 26.7 years) and shorter survival (25.8 years).
- Sixty-one patients died, mainly from sudden death (29.5%) and heart failure (22.9%), with notable maternal mortality linked to pregnancy.
Structured PICO
PPopulation188 patients with Eisenmenger Syndrome (64 males, 124 females, mean age at last follow-up 33.02+/-12.8 years) from three specialized adult congenital heart defect centers, divided into simple (n=128) and complex (n=60) congenital heart disease.
OOutcomeDeath from all causes and clinical deterioration (time variation of Ability Index)hard clinical
Patients with Eisenmenger syndrome can survive into their seventh decade, but complex defects, worsening symptoms, right ventricular dysfunction, and non-cardiac surgery are associated with adverse prognosis.