title: Life span of patients with Eisenmenger syndrome is not superior to that of patients with other causes of pulmonary hypertension.
abstract: BACKGROUND
  Patients with Eisenmenger syndrome (ES) carry a better prognosis from diagnosis than patients with other causes of pulmonary hypertension (PH), but their life span has not yet been clarified.
  
  
  OBJECTIVE
  To clarify both survival from diagnosis and life span in ES, and in closed shunt with pulmonary arterial hypertension (PAH), as compared with other causes of PH.
  
  
  METHODS
  Data on all adult patients with PH attending our centre over the past decade was collected. Outcome was defined as death or transplantation.
  
  
  RESULTS
  We studied 149 patients, including 30 (20%) patients with ES and 12 (8%) patients with closed shunt with PAH. Median age at diagnosis was lower for patients with ES and closed-shunt with PAH compared to patients with other causes of PH (P<0.001 and P=0.008 respectively). Median follow-up was 4.25 years. Survival from diagnosis was longer in ES compared to other causes of PH (logrank; P=0.02) and similar between closed-shunt with PAH and other causes of PH (logrank; P=0.3). Survival rates at 3, 6 and 9 years from diagnosis were: 73%, 50% and 47% for ES, 75%, 25% and 0% for closed-shunt with PAH, 65%, 23% and 9% for other causes of PH. Life span was similar in those three groups (logrank; P=0.2 and P=0.7, respectively).
  
  
  CONCLUSIONS
  Life span is similar in patients with ES, with a closed-shunt associated with PAH, and in patients with other causes of PH.
authors.affiliation: 1 Congenital heart disease unit, 2 Cardiologie Unit B, 3 Statistics department, La Timone University hospital, 13385 Marseille, France.
authors.forename: Béatrice
authors.initials: B
authors.lastname: Bonello
authors.affiliation: 1 Congenital heart disease unit, 2 Cardiologie Unit B, 3 Statistics department, La Timone University hospital, 13385 Marseille, France.
authors.forename: Sébastien
authors.initials: S
authors.lastname: Renard
authors.affiliation: 1 Congenital heart disease unit, 2 Cardiologie Unit B, 3 Statistics department, La Timone University hospital, 13385 Marseille, France.
authors.forename: Julien
authors.initials: J
authors.lastname: Mancini
authors.affiliation: 1 Congenital heart disease unit, 2 Cardiologie Unit B, 3 Statistics department, La Timone University hospital, 13385 Marseille, France.
authors.forename: Sandrine
authors.initials: S
authors.lastname: Hubert
authors.affiliation: 1 Congenital heart disease unit, 2 Cardiologie Unit B, 3 Statistics department, La Timone University hospital, 13385 Marseille, France.
authors.forename: Gilbert
authors.initials: G
authors.lastname: Habib
authors.affiliation: 1 Congenital heart disease unit, 2 Cardiologie Unit B, 3 Statistics department, La Timone University hospital, 13385 Marseille, France.
authors.forename: Alain
authors.initials: A
authors.lastname: Fraisse
keywords: Eisenmenger syndrome (ES); Pulmonary hypertension (PH); congenital heart disease; survival