Complex Case of Melena and Epistaxis in a Patient With Advanced Pulmonary Sarcoidosis and Pulmonary Hypertension: A Multidisciplinary Approach to Management

Document Type

Conference Proceeding

Publication Date

5-1-2025

Publication Title

Am J Respir Crit Care Med

Keywords

angiotensin receptor antagonist, antihypertensive agent, apixaban, beta adrenergic receptor blocking agent, dipeptidyl carboxypeptidase inhibitor, diuretic agent, guanylate cyclase, mineralocorticoid antagonist, riociguat, adult, anticoagulation, blood transfusion, case report, central nervous system vasculitis, chronic thromboembolic pulmonary hypertension, clinical article, complication, conference abstract, diagnosis, drug combination, drug therapy, duodenum ulcer, dyspnea, endarterectomy, epistaxis, esophagogastroduodenoscopy, gastrointestinal hemorrhage, granulomatous vasculitis, heart catheterization, heart failure, heart failure with reduced ejection fraction, hemoglobin blood level, human, hypotension, lung artery pressure, lung embolism, lung fibrosis, lung sarcoidosis, lymphadenopathy, male, melena, oral drug administration, percutaneous transluminal angioplasty, pulmonary artery systolic pressure, pulmonary hypertension, right ventricular dilatation, sarcoidosis, surgery, tachycardia, therapy, thromboembolism, transthoracic echocardiography, tricuspid valve regurgitation, vascular remodeling, vasodilatation, vein occlusion, venous pressure, ventilation-perfusion scan

Abstract

Introduction: Pulmonary hypertension (PH) is a multifactorial condition associated with elevated pulmonary arterial pressures. Treatment focuses on identification and treatment of underlying causes. Here we present the case of a 50-year-old male with advanced pulmonary sarcoidosis, recent pulmonary embolisms, and heart failure with reduced ejection fraction (HFrEF) who was found to have severe pulmonary hypertension. Case Description:A 50-year-old male with a history of advanced pulmonary sarcoidosis, right upper lobe cavitation with fungal ball, and HFrEF presented with 2 days of tarry-black stools suggestive of gastrointestinal bleeding. Two weeks prior to admission he was initiated on apixaban for pulmonary emboli. On presentation, the patient was tachycardic, dyspneic, hypotensive, and had a hemoglobin level of 7.3 g/dL. Following blood transfusion he underwent esophagogastroduodenoscopy revealing duodenal ulcers. During his admission a transthoracic echocardiography was performed demonstrating pulmonary arterial systolic pressure > 130 mmHg, a dilated right ventricle, and moderate tricuspid regurgitation. A right heart catheterization confirmed mixed pulmonary hypertension, he had a pulmonary arterial (PA) mean pressure of 71 mmHg, PA wedge mean pressure of 17mmHg, transpulmonary gradient of 54 mmHg, and diastolic gradient of 37 mmHg. Given recent pulmonary embolism history a ventilation-perfusion scan was performed to confirm CTEPH. Discussion:This case illustrates multifactorial pulmonary hypertension involving Group 2, Group 4 and Group 5:Group 2 PH: HFrEF elevates pulmonary venous pressures and pulmonary vascular remodeling. Management focuses on optimizing heart failure therapy with diuretics and goal-directed medical therapy, which includes angiotensin converting enzyme inhibitors, angiotensin receptor blockers, beta-blockers, mineralocorticoid receptor antagonists, and sodium glucose cotransporter 2 transport inhibitors.Group 4 PH: Chronic pulmonary emboli contributes to CTEPH, where persistent thrombi obstruct pulmonary vessels. Anticoagulation is used to prevent propagation of present emboli and reduce risk of further thromboembolic events. CTEPH warrants evaluation for surgical endarterectomy or pulmonary arterial balloon angioplasty, or if patient is not a candidate for either, utilization of riociguat to stimulate soluble guanylate cyclase and subsequent vasorelaxation. Group 5 PH: Due to the complex nature of sarcoidosis it is classified under group 5, typically resulting in multifactorial pathophysiology including pulmonary fibrosis, granulomatous angiitis, veno-occlusive disease, and vascular compression by enlarged lymph nodes. Management focuses on controlling the underlying inflammatory condition. This case highlights the multifactorial nature of pulmonary hypertension in some patients. As such, a tailored approach to therapy is essential to address the underlying causes, and in refractory cases lung transplant may be necessary.

Volume

211

Issue

Supplement 1

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