Paediatric Cardiac Surgery in a Patient with Cold Agglutinins

Cold agglutinins (CAs) lead to organ thrombosis or haemolysis due to increased blood viscosity and red blood cell clumping when blood temperature drops below the thermal amplitude for haemagglutination. Although it is well known that CAs are particularly relevant to adult cardiac surgery with hypothermic cardiopulmonary bypass (CPB), paediatric cardiac surgery with congenital heart disease and with CAs has been reported very rarely. We present here a case of paediatric cardiac surgery to repair atrial septal defect with pulmonary stenosis in an 11-month old infant with a family history of CAs. She was detected to have a high titre of CAs preoperatively, and underwent an intracardiac repair with normothermic CPB using temporary electrical fibrillation for added safety. Her post-operative course was uneventful without any complications.