Jiménez M Q, Díaz L P, Alvarez M, Moré R L, Zaldegui J R, González M G
An Esp Pediatr. 1975 Jan-Feb;8(1):24-41.
The clinical findings of 2 patients are studied with an association of malformations not previously reported: double outlet right ventricle and total anomalous pulmonary venous connection to the innominate vein. The following features are worth while to be underlined: 1. Both patients showed a discordance between viscero-atrial situs and direction of the apex; 2. In both cases, particularly in case 2, there existed a very unusual spacial relationship between ventricles, the right ventricle being superior with respect to the left ventricular cavity; 3. From the embryological viewpoint, this ventricular arrangement is supposed to be due to an arrest of the development of the heart in the straight tube stage, there being, however, an upwards expansion of the atrioventricular canal and common atrium; 4. An accurate diagnosis of the common or divided nature of the atrioventricular valves has been impossible in these cases; 5. These combination of lesions constitutes a challenge to cardiologists and surgeons as far as the surgical indication is concerned. In that sense, the simple anastomosis of the common pulmonary vein to the left atrium, although is not a radical cure, may be a feasible and beneficial procedure.
对2例患者的临床发现进行了研究，发现了以前未报告过的畸形组合：右心室双出口和肺静脉完全异常连接至无名静脉。以下特征值得强调：1. 两名患者均表现出内脏-心房位与心尖方向不一致；2. 在这两个病例中，特别是病例2，心室之间存在非常不寻常的空间关系，右心室相对于左心室腔位于上方；3. 从胚胎学角度来看，这种心室排列被认为是由于心脏在直管阶段发育停滞所致，然而，房室管和共同心房向上扩张；4. 在这些病例中，不可能准确诊断房室瓣是共同的还是分开的性质；5. 就手术指征而言，这些病变的组合对心脏病专家和外科医生构成了挑战。从这个意义上说，将共同肺静脉简单吻合至左心房，虽然不是根治性治疗，但可能是一种可行且有益的手术。
