BACKGROUND Intractable, mechanical hemolytic anemia (IMHA) is a rare catastrophic complication following mitral valve surgery. We analyzed patient characteristics and IMHA management by reoperations after mitral valve surgery. METHODS We collected medical records from mitral valve patients requiring reoperation due to IMHA. INCLUSION CRITERIA hemoglobin < 100 g/L; positive hemolysis tests and echocardiography results; and exclusion of other hemolysis causes. RESULTS Data from 25 IMHA cases included 10 (40%) early onset (1.3 (0.3,3.0) months) and 15 (60%) late onset (120 (24,204) months) cases. Early IMHA etiologies included surgical defects (6, 60%), uncontrolled infection (3, 30%) and Bechet's disease (1, 10%). Late IMHA etiologies included degeneration (13, 87%), new infection (1, 7%) and trauma (1, 7%). There were more mechanical valves (15, 88%) than bio-valves (2, 12%); the main valvular dysfunction was paravalvular leak (16, 64%). IMHA manifestations included jaundice (18, 72%), dark urine (21, 84%), heart failure (16, 64%), acute kidney injury (11, 44%), hepatomegaly (15, 60%), splenomegaly (15, 60%) and pancreatitis (1, 4%). Laboratory results showed decreased hemoglobin (70 ± 14 g/L) and increased bilirubin (72 ± 57 μmol/L), lactate dehydrogenase (2607 ± 2142 IU/L) and creatinine (136 ± 101 μmol/L) levels. Creatinine level negatively correlated with hemoglobin level (B = -3.33, S.E. B = 1.31, Exp(B) = 368.15, P = 0.018). Preoperative medications included iron supplements (20, 80%), erythropoietin (16, 64%) and beta-blocker (22, 88%). Two patients died of cardiac causes before reoperation. The other 23 underwent reoperation with long surgical times (aortic cross clamp 124 ± 50 min, cardiopulmonary bypass 182 ± 69 min) and blood transfusions (red blood cells 6 (6, 8) units, plasma 600 (400,800) ml, platelet 1(0,2) units). Postoperative complications included cardiac dysfunction (5, 22%), arrhythmia (10, 43%), sepsis (6, 26%), pulmonary infection (5, 22%), gastrointestinal bleeding (3, 13%), cerebral hemorrhage (2, 9%), chronic renal dysfunction (1, 4%) and surgical hemorrhage (1, 4%). Five (33%) patients died after reoperation from cardiac dysfunction (3, 60%), septic shock (1, 20%) and self-discharge (1, 20%). CONCLUSIONS IMHA induces severe multi-organ dysfunction, contributing to high mortality. Perioperative management should focus on etiological treatment, organ protection, and blood management.

中文翻译：

---

难治性机械性溶血性贫血并发二尖瓣手术：案例研究。

背景技术难治性机械性溶血性贫血（IMHA）是二尖瓣手术后罕见的灾难性并发症。我们通过二尖瓣手术后的再手术分析了患者的特征和IMHA管理。方法我们收集了因IMHA而需要再次手术的二尖瓣患者的病历。纳入标准血红蛋白<100 g / L; 溶血试验和超声心动图检查结果阳性；并排除其他溶血原因。结果25例IMHA病例的数据包括10例（40％）早期发作（1.3（0.3,3.0）个月）和15例（60％）晚期发作（120（24,204）个月）。IMHA的早期病因包括手术缺陷（6％，60％），不受控制的感染（3，30％）和贝切特氏病（1，10％）。IMHA的晚期病因包括变性（13，87％），新感染（1，7％）和外伤（1，7％）。机械阀（15，88％）比生物阀（2，12％）多；主要的瓣膜功能障碍是瓣周漏（16，64％）。IMHA表现包括黄疸（18，72％），尿液黑（21，84％），心力衰竭（16，64％），急性肾损伤（11，44％），肝肿大（15，60％），脾肿大（15 ，60％）和胰腺炎（1、4％）。实验室结果显示血红蛋白减少（70±14 g / L），胆红素（72±57μmol/ L），乳酸脱氢酶（2607±2142 IU / L）和肌酐（136±101μmol/ L）增加。肌酐水平与血红蛋白水平呈负相关（B = -3.33，SE B = 1.31，Exp（B）= 368.15，P = 0.018）。术前药物包括铁补充剂（20，80％），促红细胞生成素（16，64％）和β受体阻滞剂（22，88％）。两名患者在再次手术前死于心脏原因。其他23例再次手术时间较长（主动脉夹钳124±50分钟，体外循环182±69分钟）并输血（红细胞6（6，8）单位，血浆600（400,800）ml，血小板1（ 0,2）个单位）。术后并发症包括心功能不全（5，22％），心律不齐（10，43％），败血症（6，26％），肺部感染（5，22％），胃肠道出血（3，13％），脑出血（2 （9％），慢性肾功能不全（1％，4％）和手术出血（1％，4％）。再次手术后，有五名（33％）患者死于心脏功能障碍（3，60％），败血性休克（1，20％）和自放电（1，20％）。结论IMHA引起严重的多器官功能障碍，导致高死亡率。围手术期管理应注重病因治疗，器官保护和血液管理。