Sahulee Raj, Singh Rakesh K, Pasternack Daniel M
Division of Cardiology, Department of Pediatrics, Hackensack Meridian Medical School, 30 Prospect Ave, Hackensack, NJ, 07601, USA.
Division of Cardiology, NYU Grossman School of Medicine, New York, NY, USA.
Pediatr Cardiol. 2023 Jan;44(1):86-94. doi: 10.1007/s00246-022-02949-2. Epub 2022 Jun 22.
Infants with single ventricle physiology and congenital anomalies of the airway and/or lung are potentially poor candidates for staged palliation. The prevalence and midterm outcomes for patients with anomalies of the airway or lung with hypoplastic left heart syndrome are unknown. We performed an analysis of data in infants with hypoplastic left heart syndrome from the National Pediatric Cardiology Quality Improvement Collaborative registry. The prevalence of congenital anomalies of the airway or lung in the registry was determined. Clinical characteristics and midterm outcomes were compared between infants with hypoplastic left heart syndrome with and without anomalies of the airway or lung. Fifty-seven (2.3%) of 2467 infants with hypoplastic left heart syndrome enrolled in the registry had congenital airway or lung anomalies. Infants congenital anomalies of the airway or lung had significantly lower transplant-free survival at 1 year (49.5 vs 77.2%, p < 0.001). Infants with airway or lung anomalies had longer combined hospital length of stay for stage 1 and stage 2 palliation (102 vs 65.1 days, p < 0.001) and underwent more major procedures (2.04 vs 0.93 procedures, p < 0.001) than those without. There was no difference in the number of interstage readmissions (1.85 vs 1.89, p = 0.87) or need for non-oral feeding supplementation (71.4 vs 54.5%, p = 0.12) between groups. Infants enrolled in the National Pediatric Cardiology Quality Improvement Collaborative registry with hypoplastic left heart syndrome and anomalies of the airway or lung have increased morbidity and mortality at 1 year compared to those with hypoplastic left heart syndrome alone.
患有单心室生理结构以及气道和/或肺部先天性异常的婴儿可能不是分期姑息治疗的理想对象。气道或肺部异常合并左心发育不全综合征患者的患病率和中期结局尚不清楚。我们对国家儿科心脏病学质量改进协作登记处中左心发育不全综合征婴儿的数据进行了分析。确定了登记处中气道或肺部先天性异常的患病率。比较了有和没有气道或肺部异常的左心发育不全综合征婴儿的临床特征和中期结局。登记处登记的2467例左心发育不全综合征婴儿中，有57例（2.3%）患有先天性气道或肺部异常。患有气道或肺部先天性异常的婴儿1年时无移植生存率显著较低（49.5%对77.2%，p<0.001）。与没有气道或肺部异常的婴儿相比，患有气道或肺部异常的婴儿在1期和2期姑息治疗期间的住院总时长更长（102天对65.1天，p<0.001），接受的大手术更多（2.04次对0.93次，p<0.001）。两组间在过渡期再入院次数（1.85次对1.89次，p=0.87）或非经口喂养补充需求（71.4%对54.5%，p=0.12）方面没有差异。与仅患有左心发育不全综合征的婴儿相比，登记参加国家儿科心脏病学质量改进协作登记处的患有左心发育不全综合征且伴有气道或肺部异常的婴儿在1年时发病率和死亡率更高。
