先天性心脏病在肛门直肠畸形患儿中的发生情况及其对治疗的影响

目的: 探讨肛门直肠畸形患儿合并先天性心脏病情况及分层治疗的意义。方法: 回顾性分析2016年1月至2019年10月郑州大学第三附属医院收治的155例肛门直肠畸形患儿的临床资料和超声心动图检查结果。对照手术结果将患儿分为肛门直肠畸形中高位组和低位组。根据心脏结构畸形程度分为微小先天性心脏病和严重先天性心脏病。采用多元Logistic回归分析患儿先天性心脏病的严重程度与wingspread分型、合并心外畸形的相关性。结果: 155例肛门直肠畸形患儿中，47例（30.3%）患儿存在不同类型的心脏结构畸形，其中18例（11.6%）为微小先天性心脏病，29例（18.7%）为严重先天性心脏病。60例（38.7%）合并心外畸形，其中38例（24.5%）合并1种心外畸形，15例（9.7%）合并两种及以上心外畸形，6例唐氏综合征，1例VATER联合征。wingspread分型及合并心外畸形是肛门直肠畸形患儿合并严重先天性心脏病的独立影响因素，肛门直肠畸形中高位组合并严重先天性心脏病的概率是低位组的4.709倍（OR=4.709，95%CI：1.651~13.432，P < 0.01），心外畸形每增加一个等级（无、1种、2种及以上）合并严重先天性心脏病的概率是原来的3.850倍（OR=3.850，95%CI：2.065~7.175，P < 0.01）。治疗上，无先天性心脏病患儿和合并微小先天性心脏病患儿可遵循原计划进行肛门直肠畸形的治疗与管理，但合并微小先天性心脏病患儿术后需要心脏随访和观察，合并严重先天性心脏病患儿须依据急症优先救治的原则，制订个性化治疗方案。与无先天性心脏病患儿和合并微小先天性心脏病患儿比较，合并严重先天性心脏病患儿围手术期感染增加（P < 0.05）、住院时间延长（P < 0.01）、治愈率降低（P < 0.05）、病死率增加（P < 0.05）。结论: 患有中高位肛门直肠畸形的疑难重症患儿、不合并瘘管及瘘管不畅的急重症患儿或合并多系统异常的患儿，严重先天性心脏病的发病率较高。超声心动图检查明确合并先天性心脏病的类型和严重程度，有助于为肛门直肠畸形患儿制订最优治疗方案。

Morbidity of congenital heart disease in children with anorectal malformations and related treatment

Objective: To investigate the morbidity of congenital heart defects(CHDs) in children with anorectal malformation, and to summarize appropriate treatment. Methods: The clinical data and echocardiographic findings of 155 children with congenital anorectal malformations from the Third Affiliated Hospital of Zhengzhou University during January 2016 and October 2019 were reviewed. According to the surgical findings of anorectal malformations, the patients were categorized as the high/intermediate group and the low group; the CHDs were classified as minor CHDs and major CHDs. Multiple logistic regression was used to analyze the correlation of wingspread classification, and extracardiac malformations with the severity of CHDs. Results: Out of 155 children with anorectal malformations, 47 (30.3%) had different types of cardiac structural malformations, including 18 cases of minor CHDs (11.6%) and 29 cases of major CHDs (18.7%). Sixty children (38.7%) had extracardiac malformations, of which 38 cases (24.5%) had a single extracardiac malformation, 15 cases (9.7%) had multiple extracardiac malformations, 6 had trisomy 21 syndrome, and 1 had VATER syndrome. Multivariate logistic regression analysis showed that wingspread classification of anorectal malformation and extracardiac disorders were independent risk factors for major CHDs. The probability of major CHDs in children with high/intermediate anorectal malformation was 4.709 times higher than that with low anorectal malformation (OR=4.709, 95% CI: 1.651-13.432, P < 0.01). The probability of major CHDs was 3.85 times higher for increasing each additional grade of extracardiac malformations(without, with single, or multiple malformations) (OR=3.850, 95% CI: 2.065-7.175, P < 0.01). According to the presence and severity of CHDs, children with anorectal malformations were classified into three categories: without CHDs, with minor CHDs and with major CHDs, for differential treatment and management. Anorectal malformations would be treated and managed in children without CHDs and with minor CHDs following the original plan; however, children with minor CHDs may require cardiac follow-up after surgery. In children with major CHDs, the personalization tactics were developed based on the principle of emergency first. There were increased perioperative infection rate (P < 0.05), longer hospital days (P < 0.01), reduced cure rate (P < 0.05) and increased mortality (P < 0.05) in children with major, compared with those without CHDs and minor CHDs. Conclusions: The morbidity of major CHDs is higher in severe cases with high/intermediate anorectal malformation and acute cases without fistula or with obstructed fistula and cases with multiple congenital disorders. Echocardiography can define the type and severity of CHDs, which are useful to develop the optimal diagnosis and treatment plan for children with anorectal malformation.