Quero-Jiménez M, Raposo-Sonnenfeld I
Br Heart J. 1975 Mar;37(3):293-304. doi: 10.1136/hrt.37.3.293.
The clinical and anatomical findings in two patients with isolated ventricular inversion and situs solitus are described. The other 4 previously published cases are reviewed. The 6 patients with this malformation, all without pulmonary stenosis, presented a clinical picture of cyanotic congenital heart disease, associated with increased pulmonary blood flow (hypoxaemia and cardiac failure). The importance of different diagnostic tests is discussed and it is concluded that angiocardiography is the only definitive means of establishing the dianosis. Because the physiopathological disturbance is the same as in transposition of the great arteries, both malformations should be similarly considered with respect to diagnosis and treatment. Nevertheless, the high incidence of certain associated malformations in cases of isolated ventricular inversion adds to difficulty in diagnosis, and makes a good result from the Mustard procedure less likely than in transposition of the great arteries.
本文描述了两名孤立性心室反位合并内脏正位患者的临床及解剖学发现,并对其他4例先前发表的病例进行了回顾。这6例患有该畸形的患者均无肺动脉狭窄,表现为青紫型先天性心脏病的临床症状,伴有肺血流量增加(低氧血症和心力衰竭)。文中讨论了不同诊断检查的重要性,并得出结论:心血管造影是确诊的唯一决定性方法。由于这种生理病理紊乱与大动脉转位相同,因此在诊断和治疗方面,这两种畸形应被同样看待。然而,孤立性心室反位病例中某些相关畸形的高发生率增加了诊断难度,使得Mustard手术获得良好效果的可能性低于大动脉转位。