单心室是一种少见的先天性复杂心内畸形,发生率占先天性心脏病的0.3％。本病预后差,常由于合并其他畸形而在婴儿期死亡,偶有活至成年者。笔者诊治2例,报道如下。 例1:男性,12岁。自幼发现口唇紫绀,轻度活动后即有明显气急。体检:BP12/9.3kPa,口唇紫绀,杵状指(趾)。心率90次/分,律齐,胸骨右缘第4肋间闻及Ⅱ级全收缩期杂音,A_2亢进、单一。心电图示心房及右心室肥大,电轴右偏+244°,心房内传导阻滞。B超示肝脏发育及位置异常(中位肝),脾脏缺如。胸片见肺血略减少,主动脉右位,心腰部明显凹陷,心室均增大,拟诊法洛四联症。右心导管检查:导管在心室部活动范围较大,可达左右心缘,并极易进入肺动脉和主动脉。心室内压力增高达14/10kPa。与周围动脉压相仿。心室内各部血氧含量增高并大致接近。右心室造影:显示为单一心室,右心室肌小梁粗大,主动脉和肺动脉同时显影,均开口于右心室。主动脉起始段转位于肺动脉总干 Single ventricle is a rare congenital complex heart deformity, the incidence of congenital heart disease accounted for 0.3%. The poor prognosis of this disease, often due to the merger of other malformations in infancy and death, occasionally live to adults. The author diagnosis and treatment of 2 cases, reported as follows. Example 1: Male, 12 years old. Older lips found cyanotic, mild activity that is obviously impatient. Physical examination: BP12 / 9.3kPa, cyanotic lips, clubbing (toe). Heart rate 90 beats / min, law Qi, the right intervertebral sternal fourth intercostal smell and Ⅱ level systolic murmur, A 2 hyperthyroidism, single. ECG atrial and right ventricular hypertrophy, right axis deviation of + 244 °, atrial conduction block. B ultrasound showed abnormal liver development and location (median liver), lack of spleen. Chest radiotherapy to see a slight reduction of the lungs, aortic right, significant depression of the heart and waist, ventricles are increased, to be diagnosed with tetralogy of Fallot. Right heart catheterization: catheter in the ventricular range of motion larger, up and down around the heart, and easy access to the pulmonary artery and aorta. Intraventricular pressure increased up to 14 / 10kPa. Similar to the surrounding arterial pressure. Department of ventricular oxygen content increased and generally close. Right ventricular angiography: showed a single ventricle, right ventricular muscle trabecular thick, aortic and pulmonary simultaneously developed, are open to the right ventricle. Transposition of the aorta in the main pulmonary artery