Gassner I, Hammerer I, Gschmitzer F
Wien Med Wochenschr. 1979 Sep 30;129(17):476-80.
Report on two girls with an obstruction within the right ventricle beneath the infundibulum. A muscular band - probabaly the abnormally situated moderator band - emerges at right angles from the ventricular septum, crosses the lumen and ends at the anterior wall of the right ventricle, thus separating a high-pressure inflow chamber from a low-pressure out-flow chamber. The latter compartment is distinctly larger than the infundibular chamber in Fallot's tetralogy. In bt connecting the left ventricle with the hig-pressure compartment. In one patient an increase of severity of the obstruction could be documented; this child showed a subvalvular membranous aortic stenosis. The diagnosis has to be established by meticulous pressure recording between pulmonary and tricuspid valve and by dye injection into the right ventricle. Ventricular septum defect (in 80%), subvalvular aortic stenosis (in about 10%), pulmonary valvular and peripheric stenosis and open Ductus Botalli are the most important accompanying lesions. The malformation - not diagnosed prior to surgery - causes serious trouble during operation. The investigator thus has to be aware of this condition in every pulmonary valvular ahd subvalvular stenosis especially when accompanied by a ventricular septum defect.
关于两名女童右心室漏斗部下方存在梗阻的报告。一条肌束——可能是位置异常的节制索——从室间隔呈直角发出，穿过管腔并止于右心室前壁，从而将高压流入腔与低压流出腔分隔开。后一腔室明显大于法洛四联症中的漏斗部腔室。在其中一名患者中，可记录到梗阻严重程度增加；该患儿表现为瓣膜下膜性主动脉狭窄。诊断必须通过仔细记录肺动脉瓣和三尖瓣之间的压力以及向右心室注入染料来确定。室间隔缺损（80%）、瓣膜下主动脉狭窄（约10%）、肺动脉瓣及外周狭窄和动脉导管未闭是最重要的伴随病变。这种畸形——术前未被诊断——在手术期间会引发严重问题。因此，研究人员必须在每例肺动脉瓣及瓣膜下狭窄病例中，尤其是伴有室间隔缺损时，意识到这种情况。
