An 11-year-old child, a known case of sickle cell anaemia with a history suggestive of sickling crisis in the past was scheduled for surgical pulmonary valvotomy. Pre-operative blood transfusion and hydroxyurea were administered. Pre-operative blood transfusion is indicated in sickle cell disease patients to raise the haematocrit level and lower sickle haemoglobin (HbS) levels. Before the start of cardiopulmonary bypass (CPB), exchange transfusion was performed to reduce HbS level and raise adult haemoglobin level. Hypothermia was prevented by employing normothermic CPB.

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Hypertrophic obstructive cardiomyopathy is a relatively common disorder that signifies asymmetric hypertrophy of interventricular septum causing obstruction of the left ventricular outflow tract (LVOT). However, more recent studies have shown that during ventricular systole, flow against an abnormal mitral valve apparatus results in drag forces on the part of the leaflets. The mitral leaflet is pushed into the LVOT to obstruct it. We present a case where intraoperative transesophageal echocardiography played a crucial role in defining the etiology of LVOT obstruction that subsequently helped in deciding the surgical plan.

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Cold haemagglutination is a primary or acquired autoimmune disease involving antibodies that lead to agglutination of red blood cells at low temperature followed by complement fixation and haemolysis on rewarming. This disease can lead to adverse consequences in patients undergoing cardiothoracic surgery, especially when hypothermic cardiopulmonary bypass is applied. The authors discuss the management of two patients who underwent mitral valve replacement surgery while cold agglutinins were detected in the perioperative period. In the first patient, the diagnosis was made preoperatively followed by administration of glucocorticoids to achieve acceptable level of antibody titers. The second patient experienced haemoglobinuria during her intensive care unit stay. The case report describes the pathophysiology of cold agglutination, relevant laboratory investigations such as antibody titers and thermal amplitude, identification of at-risk patients, and management strategies to avoid serious complications.