婴儿先天性半心畸形的临床病理分析(附15例尸检报告)

The clinical and pathological analysis of the congenital hypoplastic left or right heart syndrome in infants (Report from 15 cases of autopsy )

目的探讨婴儿半心畸形的临床、病理特点及其在先天性心脏病中的意义。方法在复查我院先心病标本的基础上,对15例先天性半心畸形资料进行回顾分析。结果左心发育不良12例,主要特点为左室腔明显狭窄,左房及左心耳不发育,二尖瓣重度狭窄或闭锁,主动脉瓣闭锁,主动脉弓重度狭窄或闭锁。动脉导管均呈开放状(粗大3例)。卵圆孔未闭8例,房缺2例。右房、右室及肺动脉干显著增大。右心发育不良3例,主要改变为右室腔明显狭窄,三尖瓣明显狭窄或发育不良,肺动脉瓣闭锁,肺动脉狭窄或闭锁,动脉导管也呈开放状。左室肥大,主动脉弓粗大。结论半心畸形是婴儿时期较常见的一种致死性先心病,迄今缺乏有效的治疗手段,应引起足够重视。

Objective To explore the clinical and pathological characteristics and the significance of the hypoplastie left or right heart syndrome in the congenital heart disease of infants. Methods The 15 cases of hypoplastic left or right heart syndrome were collected and reviewed. Results The half heart abnormality was divided into two types- the hypoplastic left heart syndrome （HLHS） and the hypoplastic right heart syndrome （HRHS）.There were 12 cases of HLHS and three cases of HRHS. The pathological characteristics of HLHS included obvious or exceeding narrow left ventricular chamber, dysplasia of left atrium and left atrial appendage, the mitral stenosis or mitral atresia, atresia of the aortic valve, stenosis of aorta and atresia of aorta, patent ductus arteriosus （three cases were wide）, eight cases of patent foramen ovale, two cases of atrial septal defect, increasing right atrial and ventricular chember and wide pulmonary arterial trunk. The characteristics of HRHS included obvious narrow ventricular chamber, dysplasia of tricuspid valves or tricuspid stenosis and pulmonary valve atresia, stenosis of pulmonary artery or atresia of pulmonary artery, patent ductus arteriosus, the hypertrophy of left ventricular chamber and wide aortic arch. Conclusions The half heart abnormality is a very common fatal heart malformation of the infants. Because there is short of effective methods to treat it, we should pay close attention to it.