ACUTE PULMONARY EMBOLISM AND HEMOPTYSIS IN A PATIENT WITH CYSTIC FIBROSIS

Abstract

TOPIC: Pulmonary Vascular Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Cystic fibrosis (CF) is an autosomal recessive disease that occurs due to reduced functioning of an apical membrane protein, the CF transmembrane conductance regulator (CFTR), found on the surface of epithelial cells [1]. Lung disease in CF is characterized by obstruction of the airway and bronchiectasis (King et al). We present a unique case of an unprovoked acute on chronic pulmonary embolism (PE) in a patient with CF at our institution. CASE PRESENTATION: A 58 year old male patient with a history of CF on elexacaftor-tezacaftor-ivacaftor presented to the ED with one-day history of left-sided pleuritic chest pain. Additionally, the patient endorsed one episode of hemoptysis. Of note, the patient underwent a bronchial artery embolization 10 years ago for frequent episodes of hemoptysis. Computed tomography (CT) angiography of the chest was significant for filling defects consistent with the presence of bilateral pulmonary emboli. Echocardiogram was significant for mildly elevated pulmonary artery systolic pressure. Given the history of hemoptysis, the patient was started on an intravenous (IV) unfractionated heparin drip. A ventilation-perfusion (V/Q) scan was obtained to determine the chronicity of the pulmonary emboli. The V/Q scan demonstrated multiple perfusion defects, consistent with both acute and chronic pulmonary embolism. Further imaging and bloodwork were not suggestive of occult malignancy or hypercoagulable disorder. The patient was later transitioned to weight-based enoxaparin sodium with warfarin. DISCUSSION: Previous studies have focused predominantly on venous thromboembolism (VTE) events and hypercoagulability in children with CF. There is evidence which suggests that CF results in the development of a hypercoagulable state. This is thought to be a result of vitamin K deficiency or hepatic dysfunction, and consequently, lower levels of protein S and C [2]. Due to medical advancements in managing CF, there is an increasing need for studies and guidelines regarding lifetime VTE risk, as well as choice and duration of anticoagulation in adults with CF. The presence of hemoptysis, a common feature of CF, may further complicate management of VTE. Several mechanisms have been proposed for the association of hemoptysis with CF, including the development of dilated vessels in the bronchial circulation and the presence of bronchiectasis leading to vascular erosion [3]. In this case, the risk of recurrence of major bleeding was weighed against the need for prompt anticoagulation. CONCLUSIONS: Overlap of symptoms with CF lung disease, as well as chronic lung changes on imaging, may complicate diagnosis of acute on chronic PE in patients with CF. In addition, acute PE with concurrent hemoptysis presents a therapeutic challenge. REFERENCE #1: King, C. S., Brown, A. W., Aryal, S., Ahmad, K., & Donaldson, S. (2019). Critical care of the adult patient with cystic fibrosis. Chest, 155(1), 202-214. REFERENCE #2: Raffini, L. J., Raybagkar, D., Blumenstein, M. S., Rubenstein, R. C., & Manno, C. S. (2006). Cystic fibrosis as a risk factor for recurrent venous thrombosis at a pediatric tertiary care hospital. The Journal of pediatrics, 148(5), 659-664. REFERENCE #3: Efrati, O., Harash, O., Rivlin, J., Bibi, H., Meir, M. Z., Blau, H., ... & Modan-Moses, D. (2008). Hemoptysis in Israeli CF patients—prevalence, treatment, and clinical characteristics. Journal of Cystic Fibrosis, 7(4), 301-306. DISCLOSURES: No relevant relationships by Bahadur Ali, source=Web Response No relevant relationships by Taylor Charter, source=Web Response No relevant relationships by Winnie Chu, source=Web Response No relevant relationships by Nikolaos Hatzis, source=Web Response No relevant relationships by Anita Kim, source=Web Response No relevant relationships by Sora Lee, source=Web Response No relevant relationships by Samantha Shetty, source=Web Response