Original Article

Life span of patients with Eisenmenger syndrome is not superior to that of patients with other causes of pulmonary hypertension

Béatrice Bonello, Sébastien Renard, Julien Mancini, Sandrine Huber, Gilbert Habib, Alain Fraisse


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.
Aims: 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.

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