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• W2894981999 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: Vasoreactivity testing is done in right heart catheterizations to evaluate patients with pulmonary hypertension who may benefit from therapy with calcium channel blockers. It is important to identify those who respond because the use of vasodilators in nonresponders can lead to unintended consequences. Patients with connective tissue diseases and lung disease do not typically show an acute vasodilator response. We present a case of primary Sjogren’s syndrome with pulmonary hypertension secondary to lung disease and acute hypoxemic vasoconstriction with a partial response to nitric oxide. CASE PRESENTATION: A 53 year-old male with a history of pulmonary fibrosis, pulmonary hypertension and COPD on 6 L of oxygen per NC presented from an outside hospital with worsening dyspnea and peripheral edema. Home medications included prednisone 20 mg and sildenafil 20 mg daily, empirically started for pulmonary hypertension based on TTE due to suspicion of SLE vs Sjogren’s from his previous provider. He presented with acute hypoxemic respiratory failure and severe hypotension warranting intubation and vasopressors with stress dose steroids. He underwent rheumatological evaluation only demonstrating positive antinuclear antibodies (ANA) and SS-A antibodies consistent with Sjogren’s disease. He was treated with Tamiflu for Influenza B and antimicrobial agents for possible superimposed infection. CT of chest showed increased interstitial markings with diffuse ground glass opacity and pulmonary fibrosis. He was extubated after 10 days of mechanical ventilation to high flow NC. Stress dose steroids were transitioned to prednisone 40 mg daily, and then tapered to his home dose. A right heart catheterization was done and demonstrated pulmonary artery pressures of 105/33 (59 mmHg) with positive response to nitric oxide of 76/23 (41 mmHg). The response was attributed to hypoxic vasoconstriction secondary to acute hypoxemia related to Influenza pneumonia in the setting of pre-existing lung disease and possible group III pulmonary hypertension. No treatment was initiated for pulmonary hypertension due to concern it would further worsen ventilation-perfusion mismatch and hypoxemia. DISCUSSION: Patients with pulmonary hypertension due to exacerbation of underlying lung disease and acute hypoxemic vasoconstriction may have a partial response to nitric oxide. The response is caused by vasodilation of affected areas of the lung which can worsen hypoxemia. Vasodilator therapy in these patients may improve hemodynamics but may be associated with worsening hypoxemia. CONCLUSIONS: Responders to vasoreactivity testing in patients with severe pulmonary hypertension exacerbated by underlying lung disease need to be further evaluated. Typical screening tests in these patients are unreliable and there is little evidence for treatment in this population. Reference #1: Ruggiero RM, Bartolome S, Torres F. Pulmonary Hypertension in Parenchymal Lung Disease. Heart Fail Clin. 2012;8(3):461-474. https://doi.org/10.1016/J.HFC.2012.04.010. DISCLOSURES: My spouse/partner as a Speaker/Speaker's Bureau relationship with Salix Please note: $1001 - $5000 Added 03/02/2018 by Ebtesam Islam, source=Web Response, value=Consulting fee No relevant relationships by Sarah Jaroudi, source=Web Response No relevant relationships by Paloma Sanchez, source=Web Response No relevant relationships by Victor Test, source=Web Response No relevant relationships by Andres Yepes, source=Web Response" @default.
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• W2894981999 date "2018-10-01" @default.
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• W2894981999 title "PULMONARY HYPERTENSION SECONDARY TO LUNG DISEASE AND SJOGREN’S SYNDROME PRESENTING WITH PARTIAL RESPONSE TO NO" @default.
• W2894981999 doi "https://doi.org/10.1016/j.chest.2018.08.925" @default.
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