Risk Factors, Prophylaxis, and Treatment of Venous Thromboembolism in Congenital Heart Disease Patients

Abstract

Congenital heart disease (CHD) is a common condition in the pediatric population, affecting up to 1% of all live births (i.e., around 40,000 newborns/year in the United States). Although CHD does have a wide range of severity, by the age of 5 years approximately 80% of patients will require at least one surgical intervention to achieve a complete/palliative cardiac repair. Today, in light of their much-improved surgical survival, the care of these patients focuses on morbidity prevention and/or treatment. One such morbidity has been the increased frequency of thrombotic occlusions [e.g., cardioembolic arterial ischemic strokes; arterial, cardiac, and/or newly created shunt thrombosis; venous thromboembolism (VTE)]. Patients with CHD are at high risk of developing thrombosis due to the disruption of blood flow, CHD-related coagulopathy, inflammation, and/or platelet activation secondary to extracorporeal circulation support required during open-heart surgery or as a bridge to recovery, which can increase thrombus formation. In this article, we will discuss how the coagulation system is altered in patients with CHD in regard to the patient’s anatomy, procedures they undergo to correct their congenital heart defect, and other risk factors that may increase their thrombotic risk, focusing on VTE. We will also discuss the most recently published reports pertaining to guidelines on prophylaxis and treatment of VTE in this population. Finally, we will briefly address the long-term VTE outcomes for patients with CHD.

Incidence, Risk Factors, and Outcomes of Neonatal Renal Vein Thrombosis in Ontario: Population-Based Cohort Study

“…Both respiratory distress syndrome and congenital heart disease are associated with increased risk of thrombosis through alterations in coagulation systems (22,23). Additionally, congenital heart disease predisposes neonates to thrombosis due to alterations in blood flow (23). We found that central venous catheterization conferred a threefold increased risk of developing nRVT.…”

“…The most contributory neonatal risk factors in our study were respiratory distress syndrome, congenital heart disease, and presence of central venous catheters. Both respiratory distress syndrome and congenital heart disease are associated with increased risk of thrombosis through alterations in coagulation systems (22,23). Additionally, congenital heart disease predisposes neonates to thrombosis due to alterations in blood flow (23).…”

Venous Thromboembolism in Children: From Diagnosis to Management

“…The hepatic hypo perfusion, as a result of the impaired heart function, causes anticoagulant deficiencies. Furthermore, many patients with CHD undergo open-heart surgery and require placement of a CVC [ 47 ]. In addition, it has been proven that vascular anomalies and malformations, in particular venous malformations (VMs), can increase the risk of developing thrombosis [ 48 ].…”

Intensive Care Unit Management of the Adult with Congenital Heart Disease

“…They should also be adequately fluid repleted to prevent aggravation of hyperviscosity due to dehydration. In the ICU setting, ACHD patients are at high risk of developing thrombosis due to CHD-related coagulopathy, inflammation, and/or platelet activation secondary to extracorporeal circulation support required during open-heart surgery or as a bridge to recovery [65]. One study found 37% of VTEs in critically ill CHD patients were related to central venous catheter (CVC) use and were associated with single-ventricle physiology and number of days of CVC placement [66].…”