Pulmonary Vascular Disease: Student/Resident Case Report Poster - Pulmonary Vascular Disease II |

Consider What’s Below the Diaphragm When Evaluating Pulmonary Hypertension FREE TO VIEW

Benjamin Kurth, MD; Katharine Modisett, MD
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Walter Reed National Military Medical Center, Columbia, MD

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):1222A. doi:10.1016/j.chest.2016.08.1331
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SESSION TITLE: Student/Resident Case Report Poster - Pulmonary Vascular Disease II

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: The World Health Organization has divided the etiologies of pulmonary hypertension into five groups for purposes of research and clinical investigation. Elevated pulmonary arterial pressure is indeed the final common pathway in a broad assortment of acquired and inherited disorders. The case below illustrates how the investigation can lead you in unexpected directions.

CASE PRESENTATION: A 24-year-old hispanic male male is transferred to our hospital with a three month history of multiple evaluations by different physicians for his concerns of hemoptysis and night sweats. After multiple normal CT angiograms and multiple rounds of steroids and levofloxacin, his symptoms had progressed. He was admitted for new onset dyspnea and chest pain upon exertion and incidentally reported an “intentional” 200 pound weight-loss over the past two years. Examination revealed a rapid heart beat and jugular venous distension with normal lung auscultation. Chest radiography showed hilar lymphadenopathy and mild focal interstitial opacities (see image 1). Electrocardiogram revealed sinus tachycardia with right-axis deviation and poor R-wave progression (see image 2). Laboratory results were significant for a normocytic, normochromic anemia, thrombocytopenia and elevation of indirect bilirubin. Peripheral smear demonstrated several schistocytes. Concerns for thrombotic thrombocytopenic purpura prompted the initiation of emergent plasma exchange. Evaluation of symptomatic hypotension with bedside echocardiography revealed a “D-shaped” septum, concerning for severe pulmonary hypertension. Before right-heart catheterization could be performed, patient had a witnessed cardiac arrest and died despite resuscitative efforts. Post-mortem examination revealed pulmonary tumor thrombotic microangiopathy secondary to metastatic poorly differentiated gastric adenocarcinoma with pulmonary lymphangitic carcinomatosis.

DISCUSSION: The pathogenesis of pulmonary tumor thrombotic microangiopathy is thought to involve a showering of neoplastic microemboli into the pulmonary vasculature leading to coagulation activation and endothelial dysfunction. Eventually, this clinically manifests as the unique combination of pulmonary hypertension (PH) and microangiopathic hemolytic anemia (MAHA)1. The simultaneous development of both PH and MAHA should prompt immediate evaluation of underlying malignancy.

CONCLUSIONS: Our case demonstrates an extreme outcome of unrecognized metastatic disease. Experience has demonstrated that only early recognition of this disease can prevent “diagnosis at autopsy.2

Reference #1: Gainza E, Fernández S, Martínez D, et al. Pulmonary tumor thrombotic microangiopathy: report of 3 cases and review of the literature. Medicine (Baltimore). 2014;93(24):359-63.

Reference #2: Miyano S, Izumi S, Takeda Y, et al. Pulmonary tumor thrombotic microangiopathy. J Clin Oncol. 2007;25(5):597-9.

DISCLOSURE: The following authors have nothing to disclose: Benjamin Kurth, Katharine Modisett

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