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目的探讨Williams-Beuren综合征合并先天性心血管疾病手术纠治的麻醉管理特点。方法 2008—2011年31例Williams-Beuren综合征合并先天性心血管疾病患儿行主动脉瓣上狭窄纠治术,术中予大剂量舒芬太尼复合咪达唑仑、依托咪酯、丙泊酚和罗库溴铵行全身麻醉。主动脉开放后静脉输注多巴胺、肾上腺素、米力农和硝酸甘油等血管活性药物。体外循环停机后调节凝血功能,根据血气分析结果补充电解质和维持酸碱平衡。结果本组病例全部顺利完成手术,体外循环的平均时间为(99.69±41.66)min,平均主动脉阻断时间为(44.38±16.25)min,平均后平行时间为(25.26±16.11)min。31例(100.0%)患儿均使用多巴胺5~10μg·kg-1·h-1,15例(48.4%)患儿使用肾上腺素0.02~0.5μg·kg-1·h-1,8例(25.8%)患儿使用硝酸甘油0.5μg·kg-1·min-1。主动脉开放后,12例患儿行非同步除颤复跳,6例复跳后出现心室颤动,2例停体外循环后再恢复体外循环。1例先天性主动脉瓣上狭窄伴冠状动脉肺动脉瘘患儿,在主动脉插管准备建立体外循环时突发心室颤动,给予除颤复跳后迅速建立体外循环。术后发生低心排和左心室流出道残余梗阻各2例(6.5%),乳糜胸和心包积液各1例(3.2%)。重症监护病房的平均逗留时间为(4.23±2.44)d,平均住院天数为(14.88±4.38)d。1例因心包积液术后2周再次住院。全组无病例死亡,无麻醉相关并发症发生。结论麻醉前全面细致的评估、选用适当的麻醉药物诱导和维持、合理应用血管活性药物、严密的围术期监测并及时处理是Williams-Beuren综合征合并先天性心血管疾病纠治术切实可行的麻醉管理方法。
Objective To investigate the characteristics of anesthesia management in Williams-Beuren syndrome with congenital cardiovascular disease. Methods Thirty-one patients with Williams-Beuren syndrome complicated with congenital cardiovascular diseases during 2008-2011 underwent aortic valve stenosis surgery. High-dose sufentanil combined with midazolam, etomidate, Propofol and rocuronium were given general anesthesia. Vasodilators such as dopamine, epinephrine, milrinone and nitroglycerin were infused intravenously after the aorta was opened. Cardiopulmonary bypass after adjustment for coagulation, blood gas analysis results based on electrolyte and maintain acid-base balance. Results All the patients completed the operation smoothly. The average time of cardiopulmonary bypass was (99.69 ± 41.66) min, the mean aortic block time was (44.38 ± 16.25) min and the mean time of parallel operation was (25.26 ± 16.11) min. In 31 cases (100.0%), dopamine 5 ~ 10μg · kg-1 · h-1 and epinephrine 0.02 ~ 0.5μg · kg-1 · h-1 in 15 children (48.4% 25.8%) children with nitroglycerin 0.5μg · kg-1 · min-1. After the aorta was opened, 12 children underwent asynchronous defibrillation and resuscitation, ventricular fibrillation occurred in 6 patients after resuscitation, and 2 patients stopped resuming cardiopulmonary bypass before resuming cardiopulmonary bypass. A case of congenital aortic stenosis with coronary artery disease in children with pulmonary arterial fistula, aortic catheterization to establish cardiopulmonary bypass when the sudden onset of ventricular fibrillation, giving defibrillation after the resumption of rapid establishment of cardiopulmonary bypass. Postoperative low cardiac output and left ventricular outflow tract obstruction in 2 cases (6.5%), chylothorax and pericardial effusion in 1 case (3.2%). The average length of stay in the intensive care unit was (4.23 ± 2.44) days and the average length of stay was (14.88 ± 4.38) days. One patient was hospitalized again 2 weeks after pericardial effusion. No case of death in the whole group, no complications related to anesthesia. Conclusions Comprehensive and meticulous evaluation before anesthesia, appropriate anesthetic induction and maintenance, rational use of vasoactive drugs, strict perioperative monitoring and prompt treatment are feasible for Williams-Beuren syndrome with congenital cardiovascular disease Anesthetic management methods.