Yazdanyar A, Safavian M H, Nazarian I, Shakibi J G
Jpn Heart J. 1978 Jan;19(1):167-73. doi: 10.1536/ihj.19.167.
A 9-year-old boy is reported who had tetralogy of Fallot, rudimentary pulmonic valve, and supravalvar pulmonic stenosis. The patient lacked almost all of the clinical findings commonly encountered in this syndrome. Thus he never had signs of a large left-to-right shunt, or congestive heart failure. He had no murmur of pulmonic regurgitation and his pulmonary artery was not large on chest roentgenogram. Angiocardiography revealed moderate main pulmonary arterial dilation. The lack of the usual manifestations of tetralogy of Fallot with absent pulmonic valve was due to supravalvar pulmonic stenosis, acting as a natural pulmonary artery band. A quantitative angiocardiographic study was undertaken in order to find a clue for the diagnosis of these atypical cases. The ratio of the transverse diameters of the main pulmonary artery and the aortic root as measured on lateral angiocardiograms in 31 children with uncomplicated tetralogy of Fallot was 0.70 +/- 0.22, whereas this ratio was 1.70 in this particular patient (p less than 0.001). It is concluded that a quantitative evaluation of the ratio of the transverse diameter of the main pulmonary artery to the aortic root on lateral angiocardiogram allows differentiation of tetralogy of Fallot with absent pulmonic valve associated with supravalvular pulmonic stenosis from uncomplicated forms of tetralogy of Fallot.
据报道，一名9岁男孩患有法洛四联症、肺动脉瓣发育不全和肺动脉瓣上狭窄。该患者几乎没有该综合征常见的所有临床表现。因此，他从未出现过大量左向右分流或充血性心力衰竭的体征。他没有肺动脉反流杂音，胸部X线片显示其肺动脉不大。心血管造影显示主肺动脉中度扩张。法洛四联症合并肺动脉瓣缺如却缺乏常见表现是由于肺动脉瓣上狭窄，起到了天然肺动脉束带的作用。为了找到诊断这些非典型病例的线索，进行了一项定量心血管造影研究。在31例单纯性法洛四联症患儿中，侧位心血管造影测量的主肺动脉与主动脉根部横径之比为0.70±0.22，而该特定患者的这一比例为1.70（p<0.001）。结论是，通过侧位心血管造影对主肺动脉与主动脉根部横径之比进行定量评估，可以将法洛四联症合并肺动脉瓣缺如及肺动脉瓣上狭窄与单纯性法洛四联症区分开来。
