Surgical Management of Aortic Stenosis and Regurgitation and Ascending Aortic Aneurysm in a Patient with Thalassemia / 日本心臓血管外科学会雑誌

ABSTRACT

Thalassemia is an inherited hemoglobin disorder characterized by hemolytic anemia. Reportedly, cardiopulmonary bypass (CPB) causes hemolysis; therefore, extreme caution is warranted during CPB. However, few studies have reported open heart surgery in patients with thalassemia. We report successful surgery for aortic stenosis and regurgitation (ASR) and an ascending aortic aneurysm (AsAA) in a patient with thalassemia. A 69-year-old woman was referred to our hospital for surgical management of ASR and AsAA. Comprehensive evaluation of microcytic anemia led to diagnosis of beta-thalassemia minor. We performed aortic valve and ascending aorta replacement; we used a biologic valve and performed open distal anastomosis under hypothermic circulatory arrest (25°) combined with retrograde cerebral perfusion. Non-pulsatile flow circulation was maintained using a centrifugal pump during CPB. The suction and ventilatory pressures were decreased, and we performed dilutional ultrafiltration. A spare artificial lung was connected to the CPB to avoid complications in the event of artificial lung blockage. We did not observe any hemolysis-induced adverse event during the clinical course, and the patient was discharged 20 days postoperatively. Careful preoperative evaluation is essential to confirm thalassemia before cardiovascular surgery to establish an optimal surgical strategy and avoid the risk of CPB-induced hemolysis in patients with the hematological disorder.