小儿特发性肺动脉扩张临床分析

Idiopathic Dilation of the Pulmonary Artery

报告小儿特发性肺动脉扩张6例,男1例,女5例,年龄5～10岁。6例均无症状,听诊胸骨左缘 2～3肋间有 2～3/6级收缩期喷射性杂音;1例有舒张期杂音;均有 P_2明显分裂;3例心底部有收缩早期喀喇音。胸片均显示肺动脉段明显隆凸。心电图正常。Doppler 和超声心动图显示肺总动脉明显增宽。4例作了右心导管检查,3例进行了数字减影心血管造影,未发现左向右分流,各腔室压力正常,4 例均显示肺总动脉显著扩张,无肺动脉瓣关闭不全。

This paper reports on 6 cases of paediatric idiopathic dilation of the pulmonary artery, of which 1 was male, and the remaining 5 were females, ranging in age from 5 to 10 years. All the 6 cases has no symptoms. At the second and the third left intercostal space we noted a grade 2-3/6 systolic ejection murmur in cardiac auscultation. One of the cases had diastolic murmur. Markedly split pulmonary second sound was heard in all cases. Three cases had systolic early click murmur. All of the plain chest X-rays exhibited that the pulmonary artery was apparently intact. The cardiogram was normal. Using the combined techniques of Doppler and echocardiogram, we found the general pulmonary artery obviously widened. Right cardiac catheterization was performed in 4 cases, and digital substraction angiocardiography (DSA) in 3 cases, No left-to-right shunt was noted. The other intracardiac pressures were normal as was the pulmonary artery pressure. 4 cases showed apparent dilation of the pulmonary artery, but no pulmonary insufficiency.