Effects of continued use of targeted therapy on patients with pulmonary arterial hypertension and complicated by hemoptysis

emoptysis is a severe complication of pulmonary hypertension (PH) with a low incidence of 6%-11%. [1][2][3][4] Although occurring in all forms of PH, it is more commonly seen in pulmonary arterial hypertension (PAH), associated with congenital heart disease (PAH-CHD). [5,6]Since enlarged bronchial arteries are a frequent source of pulmonary bleeding, the primary treatment focuses on bronchial artery embolization (BAE), especially for chronic thromboembolic pulmonary hypertension (CTEPH) patients [7,8] .However, there is disagreement regarding medical therapy, which has received little attention in the recently published PH guidelines. [5,6]ccording to the EXPERT study, [9] which examined the exposure registry of riociguat in PH patients, hemoptysis is a major safety concern associated with riociguat.This may be because of the vasodilatory property of riociguat, which is the primary pharmacological mechanism of all the PH-targeted medicines.Since the PH-targeted drug therapy (TDT) has vasodilatory effects and might aggravate hemoptysis in the context of pulmonary or bronchial vascular rupture, its appropriate usage remains debatable.Thus, this study aimed to investigate the temporary effects of long-term TDT on clinical outcomes of PAH-CHD patients who had hemoptysis.We reviewed the medical records of PAH-CHD patients who had hemoptysis and were admitted to the General Hospital of Northern Theater Command from March 2011 to August 2022.All patients needed to have a previous conclusive diagnosis of PAH-CHD and continuous usage of TDT for at least one month before ad-