refludan and Hypoplastic-Left-Heart-Syndrome

Repeated exposure to unfractionated heparin is the rule in many congenital heart disease patients. Heparin-induced thrombocytopenia occurs in 1% to 3% of adult cardiac surgeries, and carries high thrombotic morbidity (38% to 81%) and mortality (approximately 28%). Although heparin-induced thrombocytopenia appears to be infrequent in pediatric patients, particularly neonates, our evolving experience suggests postcardiopulmonary bypass congenital heart disease patients may be at increased risk. Diagnostic and therapeutic challenges include frequency of thrombocytopenia after cardiopulmonary bypass, imperfect laboratory testing, lack of established dosing of alternative anticoagulants (such as argatroban and lepirudin), and increased anticoagulant-related bleeding in young children.

Topics: Adolescent; Anticoagulants; Arginine; Autoantibodies; Cardiopulmonary Bypass; Child; Child, Preschool; Clinical Trials as Topic; Fatal Outcome; Female; Heart Defects, Congenital; Heparin; Hirudins; Hospitals, University; Humans; Hypoplastic Left Heart Syndrome; Infant; Infant, Newborn; Male; Multicenter Studies as Topic; Pipecolic Acids; Platelet Factor 4; Postoperative Complications; Postoperative Hemorrhage; Recombinant Proteins; Retrospective Studies; Sulfonamides; Thrombocytopenia; Thromboembolism; Treatment Outcome

A 21-month-old boy received anticoagulation with lepirudin for heparin-induced thrombocytopenia (HIT) after undergoing cardiac surgery and receiving extracorporeal membrane oxygenation (ECMO). This report illustrates the significance of HIT in pediatric patients after cardiac surgery and the successful administration of lepirudin in this setting. To our knowledge, this is the first published report of lepirudin administered to treat HIT in a child after cardiac surgery and ECMO. Although guidelines exist that suggest the potential administration of lepirudin as treatment for children with HIT, further studies are needed to determine the safest yet most effective dosage for this population.

Topics: Anticoagulants; Cardiac Surgical Procedures; Child, Preschool; Echocardiography; Extracorporeal Membrane Oxygenation; Fontan Procedure; Heparin; Hirudins; Humans; Hypoplastic Left Heart Syndrome; Male; Platelet Count; Postoperative Complications; Recombinant Proteins; Thrombocytopenia

There are few reports of the management of pediatric patients with heparin-induced thrombocytopenia (HIT) requiring cardiac surgery using currently available anticoagulants. We report a case of an infant with HIT requiring a bidirectional Glenn shunt who was successfully managed using lepirudin (r-hirudin, Refludan; Aventis, Bridgewater, NJ). Dosing and monitoring of anticoagulation were difficult, and we suggest caution in the use of lepirudin for cardiac surgery unless reliable monitoring of the degree of anticoagulation becomes available.

Topics: Anticoagulants; Blood Coagulation Tests; Cardiac Catheterization; Cardiac Surgical Procedures; Hemodynamics; Heparin; Hirudins; Humans; Hypoplastic Left Heart Syndrome; Infant; Male; Monitoring, Intraoperative; Partial Thromboplastin Time; Recombinant Proteins; Thrombocytopenia; Thrombosis