ACUTE COR PULMONALE AS THE INITIAL PRESENTATION OF LIMITED SYSTEMIC SCLEROSIS

Abstract

SESSION TITLE: Medical Student/Resident Pulmonary Vascular Disease 2 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/09/2018 01:15 pm - 02:15 pm INTRODUCTION: Acute Cor pulmonale is a common presentation of severe pulmonary arterial hypertension (PAH). PAH affects up to 12% of patients with systemic sclerosis (SS) and associated with a poor prognosis. We present a case of a patient presenting with acute RV failure from severe pulmonary hypertension secondary to limited SS. CASE PRESENTATION: A 63-year-old African American female presented with worsening dyspnea. Past medical history was significant for COPD, severe pulmonary hypertension (PASP of 86 mmHg on previous echocardiogram), obesity and OSA. On arrival to the hospital, patient was tachypneic to 22 breaths/minute with a blood pressure of 92/70. Her oxygen saturation was noted to be 95% on 6L via nasal cannula. She suffered a syncopal episode while walking to the bathroom after being admitted to the hospital, which prompted a rapid response team to be alerted. Her blood pressure was 70/40 mmHg on arrival of the rapid response team (RRT). Pulmonary artery catheterization (PAC) was performed after point-of-care ultrasound suggested significant RV failure. PAC revealed RA 21mmHg, RV 109/20mmHg, PA 115/31 (mean of 62), PCWP 12 mmHg. TTE was performed revealing normal LV function, EF 60-65%. Abnormal septal motion and flat intraventricular septum consistent with RV overload, with an estimated PASP 103mmHg. While in the ICU, patient had continued hypotension requiring vasopressor support with norepinephrine. She was started on furosemide infusion to achieve diuresis. She was also started on intravenous epoprostenol as a pulmonary arterial vasodilator. Given her persistent hypotension and decreased cardiac output, a milrinone infusion was started as well. Despite these efforts, patient continued to deteriorate. The etiology for her decompensation remained unclear, she was noted to have significant worsening of her PASP on review of her previous echocardiography. She was started on a heparin infusion due to concerns for possible pulmonary embolism causing the acute decompensation. Further workup was pursued to evaluate for possible causes of PAH, given the decreased likelihood that her acute worsening was secondary to her history of OSA & COPD. Anti-centromere titers > 8 were revealed suggesting a diagnosis of limited SS. Unfortunately, patient’s clinical status continued to decline and patient expired. DISCUSSION: Acute cor pulmonale from PAH has an in-hospital mortality of 50% when requiring vasopressor and/or inotropic support. PAH affects up to 12% of patients with systemic sclerosis and is the leading cause of death in this patient population. Currently there are no guidelines on appropriate screening tools to identify patients with limited SS complicated by PAH. CONCLUSIONS: Acute cor pulmonale in the setting of SS portends a poor prognosis with associated 50% mortality. More studies are needed to identify strategies for early identification that will improve outcomes in this population. Reference #1: Chaisson et al. Systemic Sclerosis-Associated Pulmonary Arterial Hypertension. Chest. 2013;144(4):1346-1356. doi:10.1378/chest.12-2396. Reference #2: Jentzer et al. Pulmonary Hypertension in the Intensive Care Unit. J Intensive Care Med. 2016 Jul;31(6):369-85. doi: 10.1177/0885066615583652. DISCLOSURES: No relevant relationships by Navneet Kaur, source=Web Response No relevant relationships by Adnan Khalif, source=Web Response