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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.
Bonello et al. (Wed,) studied this question.