单心室是一种青紫型先天性心脏病、在临床上极少见.现将我院经手术及心血管造影证实的2例报告如下:1 病例介绍例1:女,4岁,因生后发绀,发现心脏杂音4年入院.出生后即见口唇发绀,吃奶时易呛咳,哭闹时发绀加重;平素易感冒,活动后气促.体查:发育正常,神志清,口唇及四肢末稍发绀、双肺呼吸音清晰,心尖搏动在右锁骨中线内第五肋间.心率102次/分,律齐,胸骨右缘3～5肋间可闻及Ⅳ/Ⅵ级收缩期吹风样杂音,粗糙,同左胸骨旁传导,可见杵状指、趾.Hb161g/L,心电图示右位心.左前分支阻滞.胸片示肺血减少,右位心,肝脏影在左侧.胃泡在脊柱左侧向中线移位.超声心动图示右位心,单心室伴大血管错位,房缺.内脏转位(肝脏在左侧).心血管造影示右位心伴内脏不全转位,右室型单心室伴肺动脉瓣下狭窄.转心胸外科行改良Fontan氏手术,手术所 Single ventricle is a purple type of congenital heart disease, is extremely rare in clinical. Now our hospital by surgery and cardiovascular angiography confirmed 2 cases are reported as follows: 1 case introduction Example 1: female, 4 years old, due to postnatal Cyanosis, heart murmur 4 years admitted to hospital after birth, see the lips cyanosis, easy to cough when feeding, cyanosis increased when crying; usually easy to cold, shortness of breath after exercise. Physical examination: normal development, clear-headed, lips and limbs Slightly cyanosis, clear lung breath sounds, apex beat in the right intercostal midline of the fifth intercostal heart rate 102 beats / min, law Qi, sternal right intercostal 3 ~ 5 intercostal can smell and Ⅳ / Ⅵ systolic hair style Murmur, rough, with the left side of the parasternal conduction, showing clubbing, toe .Hb161g / L, ECG right centroid. Left anterior branch block .Larxes showed reduced lung blood, right centripetal, liver shadow on the left. Left shift to the midline of the spine .Echocardiography shows the right centripetal, single ventricle with major vessel dislocation, atrial septal dislocation (liver in the left.) Cardiac angiography showed right centripetal with visceral insufficiency transposition of the right ventricle Single ventricle with pulmonary valve stenosis. Cardiothoracic surgery improved Fontan’s surgery, surgical