DISTAL AORTOPULMONARY SEPTAL-DEFECT, AORTIC ORIGIN OF THE RIGHT PULMONARY-ARTERY, INTACT VENTRICULAR SEPTUM, PATENT DUCTUS-ARTERIOSUS AND HYPOPLASIA OF THE AORTIC ISTHMUS - A NEWLY RECOGNIZED SYNDROME
DISTAL AORTOPULMONARY SEPTAL-DEFECT, AORTIC ORIGIN OF THE RIGHT PULMONARY-ARTERY, INTACT VENTRICULAR SEPTUM, PATENT DUCTUS-ARTERIOSUS AND HYPOPLASIA OF THE AORTIC ISTHMUS - A NEWLY RECOGNIZED SYNDROME
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DOI：
10.1016/0002-9149(82)90284-3
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发表时间：
1982-01-01
影响因子：
2.8
通讯作者：
PAUL, MH
中科院分区：
文献类型：
--
作者：
BERRY, TE;BHARATI, S;PAUL, MH
远端主动脉肺间隔缺损、右肺动脉的主动脉起源、完整的室间隔和主动脉峡部中断或缩窄之间的关联此前尚未被报道为一种综合征。五名新患者遇到了这种异常组合，之前报道过三名患者出现这种情况。两名患者接受了手术并取得了成功。与矢状定向的传统近端主动脉肺间隔缺损相比，该系列中的患者具有更远端的缺损类型，可能代表总动脉干的部分持续存在。肺动脉分叉可能会附着在该未分开的躯干段上，因此，右肺动脉可能部分或完全转移到主动脉中。这种异常的右肺动脉起源可能导致胚胎发生过程中“窃取”主动脉血流并导致主动脉弓发育不全。这一概念得到了血管造影观察的支持，即右肺动脉向右移位越大，肺弓发育不全越严重。诊断性血管造影征象是右肺动脉起点极高，同时伴有主动脉弓发育不全或闭锁。推荐的手术治疗方法是在肺干内植入右肺动脉来闭合主动脉肺间隔缺损并修复主动脉弓异常。
The association of distal aortopulmonary septal defect, aortic origin of the right pulmonary artery, intact ventricular septum and Interruption or coarctation of the aortic isthmus has not previously been reported as a syndrome. This combination of anomalies was encountered in five new patients and was previously reported in three. Two patients have undergone surgery with successful results. In contrast to the sagittally oriented conventional proximal aortopulmonary septal defect, the patients in this series had a more distal type of defect, possibly representing a partial persistence of the common arterial trunk. The pulmonary arterial bifurcation may malattach to this undivided truncal segment and, as a result, the right pulmonary artery may be partially or completely shifted into the aorta. This abnormal right pulmonary arterial origin may lead to “steal” from the aortic flow during embryogenesis and to hypoplasia of the aortic arch. This concept is supported by the angiographic observation that the greater the rightward displacement of the right pulmonary artery, the greater the hypoplasia of the arch. The diagnostic angiographic sign is a strikingly high origin of the right pulmonary artery together with aortic arch hypoplasia or atresia. Closure of the aortopulmonary septal defect with implantation of the right pulmonary artery in the pulmonary trunk and repair of the aortic arch anomaly is the recommended surgical treatment.