孤立右心室发育不全5例临床分析

SOLITARY RIGHT VENTRICULAR HYPOPLASIA:A REPORT OF 5 CASES

孤立右心室发育不全系指右心室腔缩小,除开放的卵圆孔或房间隔缺损外,不合并任何其他心脏畸形的一种罕见紫绀型先天性心脏病。根据5例临床表现,我们认为掌握如下几点不难诊断:(1)女性多见。(2)生后或儿童期发生紫绀,卧位颈静脉充盈波动,而心脏听诊基本正常。(3)心电图电轴正常或轻度左偏。(4)超声心动图显示右心室腔小,流出道正常而左心室腔扩大。(5)导管检查右心房压升高,右心室舒张末期压高,而右心室和肺动脉收缩压正常或轻度升高。(6)右心室造影显示右心室腔小,流出道正常。

Solitary right ventricular hypoplasia is a rare cyanotic congenital heart disease. The main feature of this anomaly is the diminutive size of the right ventricle and atrial septal defect without other cardiac defect. Owing to lack of diagnostic experience, the diagnosis might be delayed. According to the clinical deta of five patients, we noted that following points are helpful to diagnosis. (l)Females predominate in having solitary right ventricular hypoplasia; (2) Cyanosis is present appear in infancy and early childhood. The neck veins are engorged and pulsating with the patient in prone position, while the heart is normal on auscultation; (3) Electrocardiogram exhibits a nomal frontal electrical axis or a slight shift to the left; (4)Echocardiogram shows a diminutive right ventricular chamber and normal outflow tract, but the left ventricular chamber is enlarged; (5)Cardiac catheterization date demonstrate elevation of right atrial and right ventricular end-diastolic pressure, but systolic pressures of right ventricular and pulmonary arteries are normal or mildly elevated. All 5 cases are shown to have left right shunt through septal defects, which causes unsaturation of arterial oxygen; (6)Right ventriculogra-phy reveals a diminutive right ventricular chamber and a normal outflow tract.