HEMOPTYSIS IN EISENMENGER SYNDROME-ASSOCIATED PULMONARY ARTERIAL HYPERTENSION (PAH): FORGOT ABOUT BRONCHIAL ARTERY EMBOLIZATION

Abstract

SESSION TITLE: Pulmonary Vascular Disease SESSION TYPE: Med Student/Res Case Report PRESENTED ON: 10/23/2019 8:45 AM - 9:45 AM INTRODUCTION: Anticoagulation in Eisenmenger syndrome (ES) associated PAH (ES-PAH) presents a clinical challenge, as there is both an increased prevalence of pulmonary artery thrombosis and hemoptysis. We present a case of ES-PAH complicated by hemoptysis and treated successfully with multi-vessel bronchial artery embolization (BAE). CASE PRESENTATION: A 52-year-old female with an atrial septal defect complicated by ES-PAH treated with continuous epoprostenol infusion, ambrisentan, supplemental oxygen, and thrombophilia previously managed with warfarin; presented with a one-week history with multiple episodes of hemoptysis and chest tightness. She reported a similar episode of hemoptysis one-year previously with no specific source of bleeding identified. At the time of her hemoptysis had resolved with discontinuation of warfarin.On this presentation, her vital signs were unremarkable on 3 LPM nasal cannula. Physical examination demonstrated a right ventricular heave, a loud and prominent P2, and a third heart sound. Electrocardiography revealed normal sinus rhythm and bifascicular block (RBBB and LPFB). CT angiography (CTA) of the chest demonstrated dilated bronchial arteries and patchy ground glass opacities within the left upper and lower lobes, consistent with alveolar hemorrhage.Otorhinolaryngology and interventional pulmonology found no discrete bleeding in the upper or lower respiratory tract. After discussion, prophylactic embolization of the dilated right and left bronchial arteries was performed successfully. No further episodes of hemoptysis were reported post embolization. On outpatient follow-up the patient has remain hemoptysis free. DISCUSSION: Eisenmenger physiology results from chronic left-to-right shunting of blood, leading to increased right-sided pressures, and reversal of shunt flow. Patients with ES-PAH are at increased risk of pulmonary artery thrombosis with concurrent increased risk of hemoptysis and pulmonary hemorrhage(1). Thrombo-embolic risks include atrial fibrillation and indwelling central catheters. Hemoptysis is often due to bronchial artery dilatation and/or rupture. The use of anticoagulation in ES-PAH patients is controversial due to balancing risk between thrombosis and hemoptysis, and survival benefits are unclear(2). BAE in PAH patients is more complex than in patients without PAH, due to frequent higher numbers of culprit arteries and increased anatomical/morphological variability(3). However, BAE has been demonstrated to be a safe and reliable intervention for hemoptysis in PAH3. There is no definitive treatment and repeated BAE for reoccurrence is common. CONCLUSIONS: Anticoagulation in ES-PAH should be used conservatively in an individualized approach. Hemoptysis is an uncommon in ES-PAH and may result from bronchial artery dilatation and rupture. We demonstrated the safety of multi-vessel BAE in ES-PAH and encourage its consideration within this cohort. Reference #1: Wan D, Tsui C, Kiess M, et al. Anticoagulation for Thromboembolic Risk Reduction in Adults With Congenital Heart Disease. Canadian Journal of Cardiology 2017; 33: 1597–1603. Reference #2: Sandoval J, Santos LE, Córdova J, et al. Does Anticoagulation in Eisenmenger Syndrome Impact Long-term Survival?: Anticoagulation in Eisenmenger Syndrome. Congenital Heart Disease 2012; 7: 268–276. Reference #3: Rasciti E, Sverzellati N, Silva M, et al. Bronchial artery embolization for the treatment of haemoptysis in pulmonary hypertension. La radiologia medica 2017; 122: 257–264. DISCLOSURES: No relevant relationships by Olayiwola Amoran, source=Web Response no disclosure on file for Christine Archer-Chicko; Advisory Committee Member relationship with Actelion/Janssen Please note: $5001 - $20000 Added 02/07/2019 by Harold Palevsky, source=Web Response, value=Honoraria Advisory Committee Member relationship with United Therapeutics Please note: $1001 - $5000 Added 02/07/2019 by Harold Palevsky, source=Web Response, value=Honoraria DSMB relationship with Eiger Please note: $1001 - $5000 Added 02/07/2019 by Harold Palevsky, source=Web Response, value=Honoraria No relevant relationships by cyrus vahdatpour, source=Web Response