CLOT OR NOT? AN UNUSUAL CTEPH MIMIC

Abstract

TOPIC: Pulmonary Vascular Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Chronic thromboembolic pulmonary hypertension (CTEPH) is an important cause of severe pulmonary hypertension (PH) resulting from pulmonary thromboembolic events with subsequent remodeling of pulmonary vasculature. As one of the few forms of PH with potentially definitive management via pulmonary thromboendarterectomy (PTE), it is imperative that the diagnosis of CTEPH involves careful assessment of the patient's history and available studies, including echocardiograms, ventilation-perfusion (V/Q) scans, computed tomography (CT) scans, right heart catheterizations, and pulmonary angiography. This is especially true when considering other conditions that may mimic the presentation of CTEPH. Herein, we discuss a rare instance of pulmonary artery sarcoma (PAS) mimicking CTEPH in a patient with progressive shortness of breath. CASE PRESENTATION: A 53-year-old female with past medical history remarkable for breast cancer status-post lumpectomy and radiation on adjuvant anastrazole monotherapy presented to an outside hospital for progressive shortness of breath and weight loss. CT chest angiography suggested large pulmonary embolism clot burden, and the patient subsequently underwent catheter-directed thrombolysis with tissue plasminogen activator in bilateral pulmonary artery catheters for 24 hours, with minimal change in mean pulmonary artery pressures between times of insertion and removal. The patient subsequently transferred institutions for consideration of PTE.Review of her CT scans and a new V/Q scan suggested a more lobulated, large volume appearance of the obstructing material in her pulmonary vasculature, raising concern for malignant obstruction as opposed to CTEPH. Subsequent positron emission tomography (PET) CT scan showed equivocal fluorodeoxyglucose (FDG) uptake. The patient was brought to surgery, wherein bilateral intravascular tumor involving the main pulmonary artery extending into both branches was discovered consistent with PAS. The mass was extracted, and the patient was eventually discharged with oncologic follow-up, with post discharge course complicated by metastatic disease currently undergoing her third line of therapy. DISCUSSION: This case demonstrates the complexity diagnosing CTEPH. Important mimics of CTEPH outside of PAS include fibrosing mediastinitis, sarcoidosis, large vessel vasculitis, amongst others. Early clinical clues suggesting against the patient's initial diagnosis include recent weight loss and the lobulated appearance of the pulmonary arterial mass. The prognosis of PAS is extremely poor, due to diagnostic and management delays given the stark similarity to CTEPH, with mean survival rates between 12 and 18 months. (1,2) CONCLUSIONS: Diagnosis and management of CTEPH requires expert, multidisciplinary care, especially with the complexity of properly diagnosing the condition amidst other possible mimicking conditions. REFERENCE #1: Bandyopadhyay D, et al. Primary pulmonary artery sarcoma: a close associate of pulmonary embolism-20-year observational analysis. J Thorac Dis 2016; 8: 2592–2601. REFERENCE #2: Wong HH, et al. Presentation and management of pulmonary artery sarcoma. Clin Sarcoma Res 2015; 5: 3. DISCLOSURES: No relevant relationships by Adam Custer, source=Web Response No relevant relationships by NEVEEN El-Farra, source=Web Response No relevant relationships by Jane Wang, source=Web Response No relevant relationships by Alexander Yuen, source=Web Response