PULMONARY TUMOR THROMBOTIC MICROANGIOPATHY: A RARE CAUSE OF PULMONARY HYPERTENSION

Abstract

SESSION TITLE: Monday Fellow Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: We present a case of a 64 year old male who was admitted to the hospital for rapidly progressive dyspnea. Imaging revealed bilateral mosaic attenuation, septal thickening and ground glass opacities. After an extensive work-up, the patient was diagnosed with pulmonary hypertension related to pulmonary tumor emboli mircoangiopathy(PTTM). CASE PRESENTATION: A 64-year-old male with a history of atrial fibrillation and OSA presented with dyspnea on exertion associated with hemoptysis and hematuria. The patient was in his usual state of health until the summer when he was diagnosed with atrial fibrillation as a part of his work physical. After of which he was referred to a cardiologist who attempted multiple unsuccessful cardioversions while on different anti-arrhythmic agents. During this time, he was also continued on rivaroxaban for anticoagulation. After his last cardioversion he was maintained in normal sinus rhythm but this unfortunately did little to help resolve his profound dyspnea on exertion. He then underwent a right heart catheterization as an outpatient and had a right atrial pressure of 3 mmHg, pulmonary artery pressure of 67/19 and a pulmonary capillary wedge pressure of 3 mmHg. As he was quickly worsening from a respiratory stand point, he was then referred to the emergency department for admission and pulmonary was consulted. Physical exam demonstrated clear lungs bilaterally with no palpable lymphadenopathy. Chest x-ray on admission showed clear lung fields with no infiltrate, effusions, or masses. CT chest revealed multiple ground glass opacities, mosaic attenuation and septal thickening. Differential diagnosis at the time included inflammatory, vasculitis, autoimmune, and malignant etiologies. Extensive lab work that included auto-immune serologies were all negative. Given radiologic findings and right heart catheterization report there was concern of an occlusive disease and the patient underwent a VATS wedge resection. Pathology showed metastatic poorly differentiated carcinoma involving pulmonary arteries and vein with immunohistochemical findings suggest urothelial carcinoma. DISCUSSION: Pulmonary hypertension in PTTM presents with rapidly progressive dyspnea and develops due to progressive vascular fibrointimal stenosis and occlusion. A unique abnormality that can be found on lab work in patients with PTTM is a ‘compensated DIC’ in which there is a slow consumption of clotting factors and platelets. This along with progressive RV dysfunction can clue a diagnostician to preform a RHC with a pulmonary artery wedge aspiration for cytology. This has a reported sensitivity of 80-88% and 82-94% specificity respectively. CONCLUSIONS: In patients where the severity of pulmonary hypertension is otherwise not explained by the severity of heart or lung disease, PH due to pulmonary artery obstruction needs to be considered in the differential diagnosis. Reference #1: Uruga, Hironori, et al. "Pulmonary tumor thrombotic microangiopathy: a clinical analysis of 30 autopsy cases.” Internal Medicine 52.12 (2013): 1317-1323. Reference #2: Roberts, Kari E., et al. "Pulmonary tumor embolism: a review of the literature.” The American journal of medicine 115.3 (2003): 228-232. Reference #3: Price, Laura C., Athol U. Wells, and Stephen J. Wort. "Pulmonary tumour thrombotic microangiopathy.” Current opinion in pulmonary medicine 22.5 (2016): 421-428. DISCLOSURES: no disclosure on file for Eliot Friedman; No relevant relationships by Kriti Pathak, source=Web Response No relevant relationships by Noor Salam, source=Web Response