Until recently there was believed that PPH and CTEPH have the same unfavorable prognosis. Newer data show a better outcome for CTEPH. One prospective study from Japan for the period prior to the introduction of prostacyclin treatment  investigated the survival of 32 patients with PPH and 48 CTEPH patients. Both groups had equally elevated pulmonary arterial pressures. Mean survival was calculated to be 3.6 years for PPH and 6.8 years for CTEPH. Patients with CTEPH had a longer survival than those with PPH, despite the fact that the age at diagnosis of PPH was younger by 17 years on average than that of CTEPH (1).

Pulmonary thrombendarterectomy ameliorates the prognosis of proximal CTEPH, when performed in specialized centers.

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