Calder L, Van Praagh R, Van Praagh S, Sears W P, Corwin R, Levy A, Keith J D, Paul M H
Am Heart J. 1976 Jul;92(1):23-38. doi: 10.1016/s0002-8703(76)80400-0.
Salient clinical, hemodynamic, angiocardiographic, and pathologic findings are presented in 100 patients with truncus arteriosus communis, 79 of whom were studied at autopsy. In this study of typical truncus, all had a ventricular septal defect (type A). Truncus with a partially formed aorticopulmonary septum (type A1) was much the commonest form (50%). Cases with no remnant of aorticopulmonary septum (type A2) were second in frequency (21%). The distinction between types A1 and A2 could not be made with certainty in 9%, because these types merge into one another. Cases with absence of either pulmonary artery branch (type A3) were the least frequent form (8%). Truncus with interruption, atresia, preductal coarctation, or severe hypoplasia of the aortic arch (type A4) constituted 12%. The diagnosis of truncus is primarily angiocardiographic. The plane of the truncal valve in the lateral projection is distinctive. It tilts anteriorly, facing the patient's toes, which can be of assistance in differential diagnosis. The angiocardiographic features of type A4, although unfamiliar, are pathognomonic. Since the median age at death was only 5 weeks, and in view of the difficulties associated with pulmonary artery banding, our goal should be the surgical correction of truncus during the first and second months of life.
本文呈现了100例共同动脉干患者显著的临床、血流动力学、心血管造影及病理表现，其中79例进行了尸检研究。在这项关于典型共同动脉干的研究中，所有患者均有室间隔缺损（A型）。具有部分形成的主肺动脉间隔的共同动脉干（A1型）是最常见的类型（50%）。无主肺动脉间隔残余的病例（A2型）在发生率上位居第二（21%）。9%的病例无法明确区分A1型和A2型，因为这两种类型相互融合。无肺动脉分支的病例（A3型）是最不常见的类型（8%）。伴有主动脉弓中断、闭锁、导管前缩窄或严重发育不全的共同动脉干（A4型）占12%。共同动脉干的诊断主要依靠心血管造影。共同动脉干瓣膜在侧位投影中的平面具有特征性。它向前倾斜，朝向患者脚趾方向，这有助于鉴别诊断。A4型的心血管造影特征虽然不常见，但具有诊断特异性。由于死亡的中位年龄仅为5周，且考虑到肺动脉环扎术存在的困难，我们的目标应该是在出生后的第一个月和第二个月对共同动脉干进行手术矫正。
