DiGeorge综合征合并先天性心脏病患儿的围术期管理

Perioperative management for children of DiGeorge syndrome complicated with congenital heart disease

目的 总结DiGeorge综合征合并先天性心脏病患儿心脏手术围术期的管理经验。方法 回顾性分析行心脏外科手术治疗的8例DiGeorge综合征合并先天性心脏病患儿的临床资料,收集患儿术前人口学资料、临床表型特征资料、围术期资料。结果 8例DiGeorge综合征合并先天性心脏病患儿均顺利完成心脏外科手术治疗。4例患儿术前存在不同程度低钙血症,其中1例存在低钙性抽搐;4例患儿术中出现持续性低钙血症;2例患儿术后出现低钙血症;术后随访中1例患儿反复出现低钙性抽搐,继发癫痫。8例患儿术后需延长正性肌力药物的使用时间,5例患儿术后需延长通气治疗时间,其中1例术前合并先天性喉喘鸣、气管支气管软化症的患儿需反复使用呼吸机辅助通气。4例患儿术后出现感染,以呼吸道感染为主,其中3例患儿免疫球蛋白水平偏低,需使用静注人免疫球蛋白,有1例因反复感染住院时间超过30 d。术中有8例患儿、术后有4例患儿需接受输血治疗。8例患儿均治愈出院,无再次手术及死亡病例。术后随访复查心脏彩超均提示无残余分流和心包积液,心功能正常。结论 气道畸形、低钙血症、免疫功能低下等合并症可能造成DiGeorge综合征合并先天性心脏病患儿的围术期气道管理困难;合并顽固性低钙血症可导致术中、术后血流动力学不稳定,使正性肌力药物使用时间延长;合并免疫功能缺陷可能导致术后感染率增高;采用心脏外科手术治疗均取得良好的手术效果,但仍需关注患儿的智力发育情况。

Objective To summarize the perioperative management experience of cardiac surgery in children with DiGeorge syndrome complicated with congenital heart disease.Methods The clinical data of 8 children with DiGeorge syndrome complicated with congenital heart disease undergoing cardiac surgery were retrospectively analyzed,and the preoperative demographic data,clinical phenotypic characteristic data,and perioperative data were collected.Results The cardiac surgery was completed successfully in 8 children with DiGeorge syndrome complicated with congenital heart disease.A total of 4 children had preoperative hypocalcemia in various degree,thereinto one child had hypocalcemic convulsion,continuous hypocalcemia appeared in 4 children during operation,and postoperative hypocalcemia appeared in 2 children;furthermore,during postoperative follow-up,one child had repeated hypocalcemic convulsion,and suffering from secondary epilepsy.All the 8 children needed to prolong the length of positive inotropic medication after operation,and 5 children needed to prolong ventilation time after operation,among which one child with preoperative congenital laryngeal stridor and tracheobronchomalacia needed to use repeatedly ventilator-assisted ventilation.Four children developed postoperative infection,and respiratory tract infection was the main type,among them,3 children had low immunoglobulin level and needed intravenous injection of human immunoglobulin,and one child was hospitalized for more than 30 days due to repeated infection.Transfusion therapy was required in 8 children during operation,and in 4 children after operation.All the 8 children were cured and discharged,and no reoperation or death occurred.Postoperative follow-up echocardiography revealed no residual shunt,no pericardial effusion,and normal cardiac function.Conclusion The complications with respect to airway malformation,hypocalcemia,immunodeficiency,etc.may develop difficulty of perioperative tracheal management in children with DiGeorge syndrome complicated with congenital heart disease;in addition,complication of refractory hypocalcemia can develop intra-and postoperative hemodynamic instability,and prolong the length of positive inotropic medication;moreover,complication of immunodeficiency can increase postoperative infection rate.Employing cardiac surgery for treatment can achieve satisfactory surgical results;however,attention should be paid to the intelligent development of the children.