92例右心室双出口临床表现及其诊断  

Clinical findings and diagnosis of 92 cases of double outlet right ventricle

作　　者：张玉威[1] 朱鲜阳[1] 邓东安[1] 李军[1] 汪曾炜[1] 李莉[1] 师绿江[1] 严子才 

机构地区：[1]解放军心血管内外科研究所沈阳军区总医院

出　　处：《中华心血管病杂志》1995年第2期116-118,共3页Chinese Journal of Cardiology

摘　　要：９２例右室双出口分无肺动脉狭窄（１３例）和有肺动脉狭窄（７９例）两组。无肺动脉狭窄诊断线索：（１）严重肺高压体征伴大量左向右分流症状和体征；（２）心电图呈Ｓ＿ⅠＳ＿ⅡＳ＿Ⅲ形伴不同程度心室肥厚；（３）Ｘ线胸片心胸比值＞０．６０伴肺血增多和肺动脉段突出；（４）二维超声心动图主动脉骑跨＞５０％；（５）右心导管显示高压、高阻、大分流和动脉血氧饱和度低于正常。有肺动脉狭窄诊断线索：（１）心尖位置和杂音最响部位不一致；（２）结性心律，ＱＲＳ电轴左偏，左心负荷过重之一或联合存在；（３）二维超声心动图主动脉完全起于右心室；（４）Ｘ胸片有左旋心，右旋心，左位升主动脉，并列心耳，奇静脉开放当中一种可排除四联症。右心造影是临床诊断主要手段，主要征象为：（１）主动脉口完全起于右心室；（２）主动脉后壁与二尖瓣无纤维连续；（３）主动脉瓣口平面侧位前倾和肺动脉后壁位于主动脉后壁的后面；（４）两组半月瓣位于同一水平；（５）逆行主动脉插管导管尖端不易进入左心室；（６）远离或靠近两大血管的室间隔缺损或有大动脉转位。Abstract The clinical diagnosis was studied in 13 cases of double outlet right ventricle (DORV) without pulmonary stenosis and 79 cases of DORV with pulmonary stenosis. The results showed that: (A) in DORV without pulmonary stenosis, the clinical findings were (1 )large left to right shunts through ventricular septal defect (VSD), (2) EGG with S wave in lead Ⅰ, Ⅱ and Ⅲassociated with ventricular hypertrophy, (3) cardiothoracic ratio >0. 6 by roentgenograms with increased pulmonary vasculature and striking prominence of the pulmonary trunk, (4) more than 50 % aortic overriding on the septum by echocardiography, (5) increased pulmonary hypertension, pulmonary vascular resistance and large left to right shunts through VSD together with subnormal arterial oxygen saturation by cardiac catheterization.(B) in DORV with pulmonary stenosis,the findings were ( 1 ) inconsistent location of the cardiac apex with that of the cardiac murmur, (2) presence of nodal arrhythmia, left axis deviation in the frontal plane and / or overload of the left ventricle,(3) total arising of the aorta from the right ventricle by echocardiography.In the presence of any one of the followings,such as levoversion or dextroversion of the heart,ascending aorta on the left side, left-sided juxtaposition of the atrial appendage or open vena azygous, tetralogy of Fallot might be excluded. A main means of clinical diagnosis is selective right ventriculography, the diagnostic features of which are, (1) aortic orific completely on the right ventricle, (2) discontinuity between posterior wall of the aorta and mitral valves, (3) anteverted aortic valves with posterior wall of the pulmonary trunk located behind posterior wall of the aorta, (4) aortic and pulmonary valves at approximately the same level, (5) difficult to catheter into the left ventricle in retrograde aortography,(6) ventricular septal defect remote or close to both the pulmonary valve and the transposed aortic valve or transposition of the great arteries.

关 键 词：右室双出口 诊断 先天性心脏病 症状 

分 类 号：R541.104[医药卫生—心血管疾病]