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Journal of the American College of Cardiology ; 81(8 Supplement):3300, 2023.
Article in English | EMBASE | ID: covidwho-2251326

ABSTRACT

Background Patients with hypoplastic left heart syndrome (HLHS) undergo a Fontan procedure as part of single ventricle surgical palliation. Post-Fontan, sluggish blood flow and an imbalance in coagulant factor proteins may predispose to thrombus formation. Other risk factors may include chylothorax as well as acute and chronic inflammation. Currently, there is no standardized surveillance strategy to detect thrombus in Fontan patients. Case A 34-month old male with HLHS underwent an extracardiac non-fenestrated Fontan complicated by chylothorax treated with 5 days of IV steroids and diuretics. He was on therapeutic aspirin. After progressive worsening of right pleural effusion, a chest tube was placed three weeks post-Fontan with continued chylous output. Stool alpha 1 antitrypsin was negative. Decision-making Given persistent chylothorax, a repeat echocardiogram was performed revealing a large mass in the Fontan circuit less than one month post-op. Cardiac CT showed occlusive thrombus filling the entirety of the Fontan conduit extending into hepatic veins and bilateral pulmonary arteries. He underwent extensive surgical thrombectomy and Fontan conduit revision. Hypercoagulable work-up revealed elevated factor 8 and von Willebrand factor activity which persisted more than one month post-op. Patient's history was also significant for COVID-19 infection 6 months prior. He was initially anticoagulated with bivalirudin with tirofiban initiated for antiplatelet therapy. He was ultimately transitioned to rivaroxaban, pentoxifylline and aspirin with chylothorax resolution over one month without thrombus recurrence. Conclusion Development of risk stratification tools to identify patients at higher risk for thrombi formation post-Fontan may facilitate patient selection for more aggressive anticoagulation. Consideration of elevated factor 8 as well as persistent or recurrent chylothorax may be beneficial, as increased thrombosis risk has been reported for both conditions in Fontan patients.Copyright © 2023 American College of Cardiology Foundation

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