Abstract

INTRODUCTION: Pulmonary bleeding is a possible complication of the enlarged bronchial arteries commonly found in patients with proximal vascular obstructive lesions. Therefore, we hypothesize that the incidence of haemoptysis in patients with operable CTEPH is higher than in non-operable CTEPH or PAH. METHODS: A cohort of 175 patients with PAH and 96 patients with CTEPH were followed up from 2010 to 2015. RESULTS: During a median follow-up of 2.4 yrs (2.6-3.1), clinically relevant haemoptysis occurred in 3/175 (1.7%) patients with PAH (1 idiopathic and 2 associated with rheumatic disease), in 1/41 (2.4%) patient with non-operable CTEPH and 4/56 (7%) patients with operable CTEPH. The event rate was 0.5, 0.9 and 7.7 per 100 patient-years, respectively (p CONCLUSIONS: Haemoptysis is more frequent in patients with operable than non-operable CTEPH or IPAH. Despite its rarity, it can present with life-threatening respiratory failure. Mechanisms are uncertain, but may involve bronchial and pulmonary arteries aneurysmatic dilatations.

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