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三尖瓣闭锁综合征(Tricuspid Atresia Syndrome,简写TAS)是一种极为少见的紫绀型先天性心脏病(CHD)。我所收治并经心导管检查的CHD共637例,发现2例TAS,现报道如下并略加讨论。例1、患儿女,8岁,自幼发现心脏杂音,喜蹲踞,4岁后出现紫绀并逐渐加重。查体:BP70/40mmHg,P80次/分。身长113cm,体重18kg。重度紫绀。杵状指、趾明显,两肺无罗音。扣诊左心缘略向左扩大,胸左第二、三肋间可闻及Ⅱ级收缩期杂音,P_2不亢进。ECG:左室肥厚并劳损;UCG:左室腔增大,三尖瓣条索状回声,未见瓣叶活动,房间隔回声失落,室间隔部份回声失落。Doppler超声检查,室间隔处探及收缩期湍流;X线胸片左心缘饱满,心腰凹陷,左前斜位心前间隙增宽。右心导管及造影检查:三尖瓣闭锁(TA);房间隔缺损(ASD);室间隔缺损(VSD)并右室发育不良;右室流出道狭窄;二尖瓣返流。例2、患儿,女,9岁。自幼紫绀伴心脏杂音,活动后气促,无明显蹲踞现象。查体:BP110/60mmHg,
Tricuspid Atresia Syndrome (TAS) is a rare cyanotic congenital heart disease (CHD). A total of 637 CHDs that I had undergone and had undergone catheterization were found. Two cases of TAS were found and are reported as follows and briefly discussed. Example 1, children with children, 8 years old, childhood heart murmur, hi squat, cyanosis after 4 years of age and gradually increased. Physical examination: BP70 / 40mmHg, P80 beats / min. Length 113cm, weight 18kg. Severe cyanosis. Clubbing refers to the obvious toe, lungs without rales. Withholding the left heart margin slightly left to enlarge, chest left second, the intercostal can be heard and Ⅱ systolic murmur, P_2 not hyperthyroidism. ECG: left ventricular hypertrophy and strain; UCG: left ventricular cavity increased, tricuspid cord echo, no leaflet activity, atrial septal echo loss, echo loss of septal part. Doppler ultrasonography, ventricular septal exploration and systolic turbulence; X-ray chest left heart full, waist depression, anterior left anterior oblique space widened. Right heart catheterization and angiography: tricuspid atresia (TA); atrial septal defect (ASD); ventricular septal defect (VSD) with right ventricular dysplasia; right ventricular outflow tract stricture; mitral regurgitation. Example 2, children, female, 9 years old. His childhood cyanosis with heart murmur, shortness of breath after activities, no obvious squat phenomenon. Physical examination: BP110 / 60mmHg,