Recurrent hemoptysis: an emerging life-threatening complication in idiopathic pulmonary arterial hypertension

Hemoptysis is a rarely reported complication of idiopathic pulmonary arterial hypertension (IPAH). We present the case of a 27-year-old woman with IPAH, who suffered from recurrent hemoptysis and who was treated with sitaxsentan and treprostinil and remained stable in World Health Organization functional class II. During several episodes of active hemoptysis, the patient underwent bronchial artery embolization (BAE), always with good immediate results. She developed severe respiratory insufficiency and died of electromechanical dissociation 2 days after another episode, despite effective bleeding control. Recurrent hemoptysis in patients with IPAH emerges as a potential indication for urgent placement on the lung transplant list, independent from the classic prognostic factors of functional class and indices of right-sided ventricular function. Repeated BAE should not be considered as a definitive treatment in patients with pulmonary arterial hypertension with recurrent bleeding, although it may help in bridging patients to lung transplant.