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Evaluation of the incidence of rare diseases: difficulties and uncertainties, the example of chronic thromboembolic pulmonary hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare form of pulmonary hypertension, classified as group 4 in the present clinical classification [1, 2]. CTEPH is generally considered a late complication of one or multiple episodes of acute pulmonary embolism that have not resolved despite at least 3 months of therapeutic anticoagulation. Haemodynamically, CTEPH is characterised by a mean pulmonary arterial pressure of at least 25 mmHg at rest, measured during right heart catheterisation.


Author:Simonneau, G., Hoeper, M. M.

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