Abstract

IntroductionTotal cavopulmonary connection/Fontan circulation patients are at a higher risk of developing systemic and non-systemic thrombosis. A variety of prophylactic agents and treatment options such as drugs and interventional procedures are available for the prevention and treatment of thrombus. We hypothesized a huge variation in these practices due to a lack of robust pediatric evidence. We investigated the current thromboprophylaxis, thrombosis surveillance and treatment practices in these children across the pediatric cardiology and cardiothoracic network centers in the United Kingdom (UK). Materials and methodAn online questionnaire was sent to a cardiologist routinely involved in the management of children on single ventricular pathways from each of the 16 peditaric network centers in the UK. ResultsA total of 12 out of 16 centers (75%) responded to the survey. There was a widespread variation in practice regarding the choice of thromboprophylaxis agent, drug monitoring range(s), and treatment options for symptomatic thromboembolic complications. Warfarin was more commonly prescribed (58%) as the first line agent for thromboprophylaxis. Systemic tissue plasminogen activator (tPA) was the most preferred treatment option (42%) for symptomatic thromboembolism. None of the centers performed routine surveillance for coagulation abnormalities following the Fontan operation. ConclusionThere was a wide variation in practice regarding prophylaxis and treatment of thromboembolism. This is probably based upon institutional preference or experience due to the paucity of robust scientific evidence. Larger prospective multi-center studies with standardized surveillance and prophylaxis pathways are required to truly investigate the preferred agent for thromboprophylaxis and treatment.

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