A Tale of Triumph: Overcoming Challenges in Surgical ASD Closure for a Patient with Warm AIHA.

Abstract

Background: Autoimmune hemolytic anemia (AIHA) is a rare disorder in hematology, with an incidence of 1-3 per 100,000 per year. The current data available on open-heart procedures in patients with AIHA is limited. Despite presenting periprocedural challenges, multidisciplinary efforts enabled the successful performance of surgical atrial septal defect (ASD) closure in a patient with warm-reactive AIHA.

Case presentation: A 56-year-old woman with a large elliptical ASD was planned for surgical closure. The patient has never received a blood transfusion or experienced any previous hematological issues. During the surgical preparation, the patient's immunoglobulin G Coombs test result was positive for the presence of immunoglobulin G. The patient was diagnosed with a remission state of warm AIHA. A challenge arose when surgical ASD closure needed a cardiopulmonary bypass (CPB), which increased the risk of hemolysis. The patient also needed to be hypothermic to reduce metabolism, which may interact with the pathophysiology of AIHA. Several approaches were taken, and the procedure was conducted successfully without noteworthy obstacles.

Conclusion: A successful surgical ASD closure was performed in a patient with complete remission of warm-reactive AIHA. Considering the different hemolytic mechanisms between CPB and AIHA, determining whether AIHA is cold or warm reactive is crucial for managing temperature in the heart-lung machine. Several approaches, such as utilizing a roller pump, a heparin-coated circuit, and administering steroids, can be implemented to prevent hemolysis.