Effort Syncope as the Initial Presentation of Pulmonary Hypertension: An Abstract

Abstract

SESSION TITLE: Student/Resident Case Report Poster - Pulmonary Vascular Disease I SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Cases of effort syncope have been reported due to aortic stenosis, hypertrophic cardiomyopathy, and congenital heart diseases. Patients with pulmonary artery hypertension (PAH) typically present with dyspnea, fatigue or chest pain. We present a case of effort syncope as the initial manifestation of PAH. CASE PRESENTATION: A 50 year old male with past medical history of adult onset asthma, history of unprovoked deep vein thrombosis (DVT) on Warfarin, dyslipidemia, and hypertension presented as a transfer from an outside hospital for evaluation by ophthalmology for right orbital fracture after having two syncopal episodes on the morning of hospital admission. After running 15 feet at full sprint, he began to experience sudden onset chest tightness, shortness of breath, confusion, dizziness and subsequent collapse with loss of consciousness for 1 minute. He reports similar experiences in the past where he passed out from exertion over the last 10 years. Basic laboratory findings were within normal limits (WNL). Electrocardiogram showed sinus tachycardia, incomplete right bundle branch block, and T wave inversions in anterior and inferior leads. Serial cardiac biomarkers were WNL. proBNP was 373. There was no disease on chest xray. An echocardiogram with bubble study revealed an ejection fraction of 55-60%, paradoxical septal motion, right ventricle (RV) enlargement, thickened RV wall, impaired RV function, moderate-severe PA pressures. There was no pericardial effusion or intracardiac shunt. CTA thorax revealed dilated pulmonary artery. Given a history of DVT, ventilation - perfusion (VQ) scan was performed to evaluate for chronic thromboembolic disease. The VQ scan revealed two perfusion mismatch defects in the right upper lobe and intermediate probability of pulmonary embolus. Dobutamine stress echocardiography was negative for ischemia. Cardiac catheterization showed normal coronaries, normal left ventricular systolic function with ejection fraction 60% with normal wall motion, elevated left ventricular end diastolic pressure, and severe pulmonary hypertension with mean PA pressure 52 mmHG and no response to Flolan challenge. Pulmonary function tests showed normal spirometry, lung volumes and diffusion capacity. Liver function test, HIV, vasculitis panel, cardiolipin antibodies, Factor V Leiden, prothrombin gene, and homocysteine levels were unremarkable. Management included chronic anticoagulation, supplemental oxygen of 4 L NC, and anti-hypertensives. He was referred to specialized center for thrombo-endarterectomy for chronic thromboembolic pulmonary hypertension. DISCUSSION: It is important to recognize the signs and symptoms of PAH which are often subtle and nonspecific. Earlier recognition of the disease can lead to rapid steps toward management of the underlying cause of pulmonary hypertension. CONCLUSIONS: Reference #1: Lowe, MD & Petch, MC. (2000). Syncope after effort. Postgrad Med J, 76: 164-165. DISCLOSURE: The following authors have nothing to disclose: Ezinnaya Ubagharaji, Aiswerya Sampath, Kegan Jessamy, Alka Lamsal-Ghimire, Matthew Hess No Product/Research Disclosure Information