Piot O, Guillo P, Pouillart F, Lucas O, Beaucousin M C, Duval A M, Gaudeau S, Brun-Buisson C, Dubois-Randé J L, Castaigne A
Service de cardiologie, hôpital Henri-Mondor, Créteil.
Arch Mal Coeur Vaiss. 1995 Sep;88(9):1349-52.
The authors report the case of tetralogy of Fallot (TOF) associated with situs inversus, the first description of this rare association in a previously asymptomatic adult. A 32 years old chauffeur was admitted to hospital with pyrexia and convulsions due to a left temporo-parietal cerebral abscess which had a favourable outcome. The chest X-ray and Doppler echocardiographic study showed a TOF with a high infundibular stenosis and dextrocardia. Abdominal ultrasonography confirmed a complete situs inversus. The good tolerance was attributed to the equilibrated character of the TOF. The orientation of the heart and the cono-truncal septation occur at different times during embryogenesis. However, there are genetic arguments in favour of the non-fortuitous nature of this association.
作者报告了1例法洛四联症(TOF)合并内脏反位的病例,这是对一名既往无症状成年人中这种罕见关联的首次描述。一名32岁的司机因左颞顶叶脑脓肿伴发热和惊厥入院,治疗效果良好。胸部X线和多普勒超声心动图检查显示为伴有高位漏斗部狭窄和右位心的TOF。腹部超声检查证实为完全性内脏反位。良好的耐受性归因于TOF的平衡特征。心脏的定向和圆锥干分隔在胚胎发育的不同时间发生。然而,有遗传学依据支持这种关联并非偶然。