Kawahito T, Egawa Y, Matsumura C, Ooshio T, Miyauchi T, Kikutsuji T, Kirino A, Oota A, Takahashi Y
Department of Cardiovascular Surgery, National Kagawa Children's Hospital, Zentsuji, Japan.
Kyobu Geka. 1993 Sep;46(10):870-5.
A month old girl was referred to our hospital for heart murmur and congestive heart failure. After the UCG and angiography, clinical diagnosis was tetralogy of Fallot, right aortic arch, isolation of left subclavian artery (Victorica type I), and a patent ductus arteriosus that originated from left subclavian artery. The direction of blood flow in the patent ductus arteriosus was from a subclavian artery to pulmonary artery, so she was in the condition of pulmonary over flow and appeared congestive heart failure, although she associated with tetralogy of Fallot. With intraoperative and postoperative examinations, we diagnosed the association of partial DiGeorge syndrome. Isolation of subclavian artery is uncommon, but this anomaly was classified by Victorica. Embryology of this anomaly was explained with hypothesis of double arch system. We had seen no reports of DiGeorge syndrome associated with isolation of a subclavian artery. But DiGeorge Syndrome is hypoplasia of branchiogenic organs, and subclavian arteries is differentiated from brachial arteries. So we supposed that this two lesions have the possibility of combination.
一名1个月大的女婴因心脏杂音和充血性心力衰竭被转诊至我院。经超声心动图（UCG）和血管造影检查后，临床诊断为法洛四联症、右位主动脉弓、左锁骨下动脉离断（维克托里卡I型）以及起源于左锁骨下动脉的动脉导管未闭。动脉导管未闭内的血流方向是从锁骨下动脉到肺动脉，因此尽管她合并法洛四联症，但仍处于肺血流过多状态并出现充血性心力衰竭。通过术中及术后检查，我们诊断其合并部分迪格奥尔格综合征。锁骨下动脉离断并不常见，但这种异常由维克托里卡进行了分类。这种异常的胚胎学通过双弓系统假说进行了解释。我们未见过迪格奥尔格综合征与锁骨下动脉离断相关的报道。但迪格奥尔格综合征是鳃源性器官发育不全，而锁骨下动脉由肱动脉分化而来。所以我们推测这两种病变有可能合并出现。
