目的:明确先天性心脏病(CHDs)合并先天性心脏病(CHDs)的患病率和特征。
方法:将2010年1月至2013年12月我院收治的所有组织学诊断为HSCR的患者纳入本前瞻性观察性研究,并进行心血管筛查。通过分段超声心动图方法评估心脏解剖结构。主动脉根部和左心室尺寸的测量,壁厚,并获得了函数。需要经皮或手术干预的CHD被描述为主要的心脏病。
结果:共纳入130名连续患者,中位年龄为2.3岁。11名患者(8.3%)出现相关心脏病。此外,5例患者主动脉根部轻度扩张。11名患者中有6名(4.5%)患有需要手术修复的严重CHD。
结论:相关冠心病的患病率略高于以前的论文,主要表现为间隔缺损。六位患有严重心脏病的患者中有四位也有染色体异常。如果我们不考虑染色体异常患者的亚群,3.8%的患者存在心脏缺陷.基于这些结果,我们建议对所有Hirschsprung患者进行常规超声心动图检查,有或没有相关的染色体综合征。
OBJECTIVE: To define the prevalence and characteristics of associated congenital heart diseases (CHDs) in patients with Hirschsprung\'s disease (HSCR).
METHODS: All patients with a histological diagnosis of HSCR admitted to our hospital between January 2010 and December 2013 were included in this prospective observational
study and underwent cardiovascular screening. Cardiac anatomy was assessed by a segmental echocardiographic approach. Measurements of aortic root and left ventricular dimensions, wall thickness, and function were obtained. CHDs requiring a percutaneous or surgical intervention were described as major heart diseases.
RESULTS: One hundred thirty-three consecutive patients were enrolled at median age of 2.3 years. Eleven patients (8.3%) presented an associated heart disease. Moreover, five patients had mild dilatation of aortic root. Six out of 11 (4.5%) patients had a major CHDs requiring surgical repair.
CONCLUSIONS: Prevalence of associated CHDs was slightly higher than in previous papers, and mostly represented by septal defects. Four out of six patients with major heart disease had also a chromosomal anomaly. If we do not consider the subpopulation of patients with a chromosomal anomaly, cardiac defects were present in 3.8% of the patients. Based on these results, we suggest to perform routine echocardiogram in all Hirschsprung patients, with or without associated chromosomal syndromes.