Central venous line-related thrombosis in children with congenital heart disease: Diagnosis, prevalence, outcomes and prevention

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Thrombosis is a newly emerging clinical entity in children who are surviving previously lethal underlying disorders such as congenital heart disease. Children with congenital heart disease are the largest identifiable pediatric patient group with thrombosis accounting for 1/3 of children with thrombosis. In children, the most important risk factor for venous thromboembolic events is the use of central venous lines. Therefore, the authors reviewed the literature in what is known about the diagnosis, prevalence, outcomes and prevention of central venous line-related thrombosis in children with congenital heart disease. Ultrasound and venography must be performed to diagnose or exclude the presence of venous thrombosis in venous system in the upper body. In the studies to date in children with congenital heart disease, only echocardiograms, linograms and ultrasounds have been performed to assess thrombosis. Therefore, the prevalences of thrombosis are an underestimate. Also, both prevalences of thrombosis and prevalences of outcomes are influenced by the study design with retrospective reviews being the weakest study design as compared to prospective studies. The prevalences of thrombosis where as low as 1.1% in retrospective studies. In the two prospective studies, the prevalences were 20% and 42.5%. The reported mortality in children with thrombosis was substantial, between 40% and 50% in two retrospective reviews. In a majority of the deaths, the thrombotic event contributed or was causal to mortality. In 20% of the surviving children, there was varying relatively mild to severe debilitating sequela directly related to the thrombotic event. There is mounting evidence that children with congenital heart disease are at an increased for thrombotic events that result in significant mortality and morbidity. However, the true prevalence of thrombosis and the associated mortality and morbidity related to thrombosis needs to be determined in adequately powered prospective studies using sensitive radiographic tests.