Moene R J, Ottenkamp J, Oppenheimer-Dekker A, Bartelings M M
Br Heart J. 1985 Jan;53(1):58-63. doi: 10.1136/hrt.53.1.58.
Of 279 patients with transposition of the great arteries, 14(5%) had narrowing of the aorta, including local coarctation (6), isthmus hypoplasia (6), isthmus atresia (1), and kinking of the aorta (1). There were six deaths in 10 surgically treated patients; in addition four patients died before operation. Two of the four survivors had a subpulmonary malalignment ventricular septal defect with angiocardiographic narrowing of the right ventricular outflow tract, two had evidence of redundant muscle tissue obstructing the right ventricular outflow tract. Necropsy showed the presence of anatomical right ventricular outflow tract or inflow tract obstruction or both in all 10 cases. Outflow tract obstruction was represented by anterior displacement of the infundibular septum and ventriculoinfundibular fold (in hearts with a subpulmonary malalignment ventricular septal defect) or by redundant muscle tissue; inflow tract obstruction was represented by hypoplasia of the tricuspid valve. It is concluded that the combination of transposition of the great arteries and narrowing of the aorta is always accompanied by right ventricular outflow or inflow tract obstruction or both; the right ventricular abnormalities are probably responsible for the presence of the aortic arch anomalies by reducing aortic flow during morphogenesis. Successful surgical treatment of this complex anomaly is feasible in selected cases.
在279例大动脉转位患者中，14例（5%）存在主动脉缩窄，包括局部缩窄（6例）、峡部发育不全（6例）、峡部闭锁（1例）和主动脉扭曲（1例）。10例接受手术治疗的患者中有6例死亡；此外，有4例患者在手术前死亡。4例幸存者中，2例有肺动脉瓣下对位不良型室间隔缺损伴右心室流出道血管造影显示狭窄，2例有多余肌肉组织阻塞右心室流出道的证据。尸检显示，所有10例患者均存在解剖学上的右心室流出道或流入道梗阻或两者皆有。流出道梗阻表现为漏斗间隔和心室漏斗皱襞向前移位（在伴有肺动脉瓣下对位不良型室间隔缺损的心脏中）或多余肌肉组织；流入道梗阻表现为三尖瓣发育不全。结论是，大动脉转位合并主动脉缩窄总是伴有右心室流出道或流入道梗阻或两者皆有；右心室异常可能是在形态发生过程中通过减少主动脉血流导致主动脉弓异常的原因。在选定的病例中，成功手术治疗这种复杂畸形是可行的。
