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本文报道经心导管造影确诊右室双出口43例,着重讨论临床表现和诊断。31例二维超声检查21例诊断右室双出口。43例心导管造影按室缺部位分主动脉瓣下20例,14例合并肺动脉狭窄;肺动脉瓣下16例,8例合并肺动脉狭窄;双动脉口下1例;远离大动脉口6例4种类型。本组43例右室双出口仅10例临床诊断与心血管造影相符(23%)。主动脉瓣下室缺伴肺动脉狭窄14例中有10例诊断四联症。主动脉瓣下室缺无肺动脉狭窄很易误诊室间隔缺损合并肺高压。肺动脉瓣下室缺患儿早期出现紫绀和心衰与完全性大动脉错位相似。提示右室双出口的诊断主要依赖二维超声心动图和心导管造影检查。双圆锥可作为右室双出口的诊断依据之一。
This article reports 43 cases of right ventricular double outlet confirmed by transcatheter arterial catheterization, focusing on clinical manifestations and diagnosis. 31 cases of two-dimensional ultrasound examination of 21 cases of right ventricular double outlet. Forty-three cases of cardiac catheterization were divided into two groups according to ventricular septum defect: 20 cases under aortic valve, 14 cases with pulmonary stenosis, 16 cases under pulmonary valve, 8 cases with pulmonary stenosis, 1 case under double-artery port, 4 cases . The group of 43 cases of right ventricular double outlet only 10 cases of clinical diagnosis consistent with angiography (23%). Aortic valve room with pulmonary stenosis in 14 cases out of 10 cases diagnosed with tetralogy of Fallot. Aortic valve space without pulmonary stenosis is very easy to misdiagnose ventricular septal defect with pulmonary hypertension. Early absence of cyanosis and heart failure in children with infrarenal flaps resembled complete arterial dislocation. Prompt diagnosis of right ventricular outlet depends mainly on two-dimensional echocardiography and cardiac catheterization. Double cones can be used as a diagnosis of double outlet right ventricle.