Triatmojo Nicodemus N, Putri Valerinna Yogibuana Swastika, Rahimah Anna Fuji, Damanik Gracelia Ruth Elizabeth, Wardhana Koernia Kusuma
Department of Cardiovascular Medicine, Saiful Anwar Hospital, Malang, Indonesia.
Department of Cardiology at Saiful Anwar Hospital, Malang, Indonesia.
Cardiovasc Hematol Disord Drug Targets. 2025;25(2):139-142. doi: 10.2174/011871529X366103250311061025.
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.
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.
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.
自身免疫性溶血性贫血（AIHA）是血液学中的一种罕见疾病，每年发病率为十万分之一至十万分之三。目前关于AIHA患者心脏直视手术的可用数据有限。尽管围手术期存在挑战，但多学科团队的努力使得一名温反应性AIHA患者成功进行了房间隔缺损（ASD）封堵手术。
一名56岁患有大型椭圆形ASD的女性计划接受手术封堵。该患者从未接受过输血，也未曾有过任何血液学问题。在手术准备期间，患者的免疫球蛋白G库姆斯试验结果显示免疫球蛋白G呈阳性。患者被诊断为温性AIHA缓解期。当手术封堵ASD需要进行体外循环（CPB）时出现了挑战，这增加了溶血风险。患者还需要进行低温处理以降低代谢，这可能与AIHA的病理生理相互作用。采取了多种方法，手术成功进行，没有遇到重大障碍。
一名温反应性AIHA完全缓解的患者成功进行了ASD封堵手术。考虑到CPB和AIHA之间不同的溶血机制，确定AIHA是冷反应性还是温反应性对于心肺机温度管理至关重要。可以采取多种方法，如使用滚压泵、肝素涂层回路和给予类固醇来预防溶血。
