Bacgorund:

1. Bacgorund: This 44-year-old, 60kg, male patient was transferred from other hospital under the impression of 1) Congestive heart failure (CHF) with Dopamine infusion and IABP support, 2) Respiratory failure under mechanical ventilation. Cardiac echo revealed 1)Af with RVR, dilated LA, RA, LV and RV 2)Global hypokinesis with poor LV and RV systolic function (LVEF：17.4%), 3)Severe MR, moderate to severe TR. Dilated cardiomyopathy with stage IV CHF made the weaning of IABP failed. Due to family history and proximal muscle weakness, muscular biopsy was done and revealed Becker muscular dystrophy (BMD) (Exon 45-53 deletion). Mitral and tricuspid valve annuloplasty as an alternative to heart transplantation was indicated. Case report: General anesthesia was induced with Midazolam 5mg, Fnetanyl 150mcg, and Cisatracurium 10mg intravenously. After intra-tracheal block with 2% Xylocaine 2ml, the lungs were intubated and mechanically ventilated. Regarding the high risk for malignant hyperthermia like syndrome in such case, total intravenous infusion (TIVA) with target control infusion (TCI) of Propofol and Cis-atracurium infusion was used. Anesthetic depth was maintained with BIS range of 40 to 60. The initial PA pressure and the CCO were 12/6 mmHg and 2.2L/min. Pre-procedrue TEE showed dilated four chambers and severe MR (Figure 1.). To prevent the intrafat occluding the micropore of bypass membrane filter, TIVA with Propofol infusion was shifted to bolus Midazola, Fentanyl, and Cis-atracurium immediately before cardiopulmonary bypass. Extra-boluses were done in accordance to BIS value. The surgical procedures were uneventful. After re-gain heart beat, TEE revealed hypokinesis of LV. Primacor and Dobutamine infusion was administrated. 5 hours latter, improved LV wall motion was noted and the patient was successfully weaned off from cardiopulmonary bypass. IABP was re-on to support heart function. Post-procedure TEE showed an artifcial ring over mitral valve with improved mitral regurgitation (Figure 2.) and tricuspid valve as well. The CCO values were 4.0 to 4.9 L/min. There was no hyperthermia nor hyperkalemia happened during this operation. Primacor and dobutamine were tapered and IABP was weaned off smoothly on the post-operation day 4. No post-operative rhabdomyolysis occurred, but mild fever was noted since post-operative day 4. Leukocytosis and sputum culture showed Enterobacter, Fever subsided after antibiotics treatment. Cardiac echo before discharge revealed 1) severely abnormal LV systolic function with global hypokinesia, LVEF: 26%. 2) residual mild MR, no MS 3) mild to moderate TR, mild PR 4) impaired RV systolic function. Steroid therapy was used for BDM. He was discharged and followed up at CVS OPD. Conclusion: Successful anesthetic management with TIVA was performed in a patient with BMD underwent mitral and tricuspid valve annuloplasty. Possible complications including malignant hyperthermia were thus avoided. Anesthesia Management Of Mitral And Tricuspid Valve Annuloplasty In A Becker’s Muscular Dystropy Patient.佛教慈濟醫療財團法人台北慈濟醫院周威志, 陳虹君, 高銘章, 黃俊仁 Figure 1. Dilated four chamber with thin myocardial thickness, pericardial effusion and mitral regurgitation secondery to dilatation annulus. Figure 2. Artificial ring over mitral valve with residual MR.