Treatment Algorithm for Patients With von Willebrand Syndrome Type 2A and Congenital Heart Disease—A Treatment Algorithm May Reduce Perioperative Blood Loss in Children With Congenital Heart Disease

Objectives: In children with congenital heart disease (CHD), excessive perioperative bleeding is associated with increased morbidity and mortality, thus making adequate perioperative hemostasis crucial. We investigate the prevalence of acquired von Willebrand syndrome type 2A (aVWS) in CHD and develop a treatment algorithm for patients with aVWS and CHD (TAPAC) to reduce perioperative blood loss. Design: Retrospective cohort study. Setting: Single-center study. Patients: A total of 627 patients with CHD, undergoing corrective cardiac surgery between January 2008 and May 2017. Interventions: The evaluation of perioperative bleeding risk was based on the laboratory parameters von Willebrand factor (VWF) antigen, ristocetin cofactor activity, platelet function analyzer (PFA) closure time adenosine diphosphate, and PFA epinephrine. According to the bleeding risk, treatment was performed with desmopressin or VWF. Measurements and Main Results: aVWS was confirmed in 63.3 %, with a prevalence of 45.5% in the moderate and 66.3 % in the high-risk group. In addition, prevalence increased with ascending peak velocity above the stenosis (vmax) from 40.0% at less than or equal to 3 m/s to 83.3% at greater than 5 m/s. TAPAC reduced mean blood loss by 36.3% in comparison with a historical control cohort (p
Source: Pediatric Critical Care Medicine - Category: Pediatrics Tags: Cardiac Intensive Care Source Type: research