Anesthesia for mitral valve replacement in a patient with beta thalassemia major

case report

Abstract

Post-spinal Thalassemias constitute a heterogeneous group of inherited genetic disorders characterized by deficient synthesis of hemoglobin polypeptide chains, with beta-thalassemia being the most prevalent autosomal recessive hereditary hematologic disease. Caused by mutations in the beta-globin gene on chromosome 11, it leads to anemia with symptoms such as fatigue, dyspnea, weakness, headaches, and hepatosplenomegaly. Peripheral blood smears typically reveal anisocytosis, poikilocytosis, and numerous nucleated erythrocytes. The standard treatment involves lifelong regular blood transfusions and chelation therapy to manage iron overload. This report details the anesthetic and surgical management of a complex patient with beta-thalassemia major undergoing mitral valve replacement. The patient, who also presented with hypertension, diabetes, hypothyroidism, pulmonary hypertension, gastroesophageal reflux disease, and generalized anxiety disorder, received multimodal balanced general anesthesia combined with interfascial blockade of the serratus and deep pectoralis muscles. A minimally invasive biological mitral valve replacement was successfully performed. Postoperatively, the patient exhibited stable hemodynamic and ventilatory parameters and was extubated in the operating room without complications. The discussion emphasizes the importance of regular serum ferritin monitoring in transfusion-dependent patients and the necessity of meticulous perioperative care to prevent complications such as hypoxemia and hypovolemia, particularly given the risk of erythrocyte sickling in this population.