Zamora C, Portos J M, de los Ríos M, Mata L A, Molina B, Attié F
Arch Inst Cardiol Mex. 1976 Sep-Oct;46(5):543-53.
Due to the lack of uniformity in the criteria for formulating the diagnosis of the syndrome of heterotaxy, 12 cases with this abnormality were reviewed. The patients were selected on the following basis: symmetrical liver, changeable P waves in consecutive electrocardiograms, bronchial isomerism, hematological disturbances, anomalous relationship of the inferior vena cava and abdominal aorta, anomalous systemic and pulmonary venous drainage and complex cardiac malformations. The most frequent findings were: symmetrical liver, changeable P waves, anomalous systemic venous return, anomalies of the atrio-ventricular valves, particularly atrioventricular canal, aorto-cava juxtaposition, single atrium, anomalous pulmonary venous return, transposition of the great arteries and pulmonary stenosis. Less frequent anomalies were: atrial and ventricular septal defects, atrial isomerism, truncus arteriosus and partial distortion of the great arteries. The hematological disturbances as well as the radioisotope scanning of the liver and the spleen were of little help. Suggestions are given for the diagnosis of the syndrome and for the evaluation of present diagnostic procedures making necessary to use the data gathered with more precision.
由于制定内脏异位综合征诊断标准缺乏统一性，对12例存在这种异常情况的病例进行了回顾。入选患者基于以下标准：肝脏对称、连续心电图中P波多变、支气管异构、血液学紊乱、下腔静脉与腹主动脉关系异常、体循环和肺静脉引流异常以及复杂心脏畸形。最常见的表现为：肝脏对称、P波多变、体循环静脉回流异常、房室瓣异常，尤其是房室通道、主动脉-腔静脉并列、单心房、肺静脉回流异常、大动脉转位和肺动脉狭窄。较少见的异常有：房间隔和室间隔缺损、心房异构、动脉干和大动脉部分扭曲。血液学紊乱以及肝脏和脾脏的放射性核素扫描帮助不大。针对该综合征的诊断以及对当前诊断程序的评估给出了建议，强调有必要更精确地收集数据。
