Lee M L, Wang J K, Wu M H, Lue H C, Chiu I S, Chang C I
Department of Pediatrics, Changhua Christian Hospital, Taiwan.
Int J Cardiol. 1998 Feb 28;63(3):205-10. doi: 10.1016/s0167-5273(97)00325-2.
From July 1, 1989 to June 30, 1996, a total of six patients, four male and two female, with double aortic arch (DAA) were registered. They aged 16 days to 6.5 years. There were two patients with isolated DAA, and four complicated with intracardiac anomalies. All six patients presented respiratory distress and feeding problem early in life. However, cyanosis, owing to decreased pulmonary blood flow, predominated the clinical picture in each patient having DAA intricated with tetralogy of Fallot (TOF) and pulmonary stenosis, TOF with absent pulmonary valve syndrome, and type II-A tricuspid atresia. DiGeorge syndrome was documented in one patient with TOF and pulmonary stenosis. Expiratory wheezing was remarkable in the patient with TOF and absent pulmonary valve syndrome. Mild cyanosis and heart failure was noted in one patient with ventricular septal defect (VSD), and bicuspid pulmonary valve with mild valvular stenosis. Barium esophagogram showed indentations on esophagus in five patients. Echocardiography was yielding in three infants. Four patients, two isolated and two complex DAA, survived the surgical division of the smaller left aortic arch. One-stage and two-stage operation was performed in patients having DAA intricated with VSD and TOF, respectively. The other two patients with complex DAA remained in close observation owing to the tolerance of symptoms related to intracardiac anomalies.
1989年7月1日至1996年6月30日，共登记了6例双主动脉弓（DAA）患者，其中4例男性，2例女性。年龄从16天至6.5岁。有2例孤立性DAA患者，4例合并心内畸形。所有6例患者在生命早期均出现呼吸窘迫和喂养问题。然而，由于肺血流量减少导致的青紫在每例合并法洛四联症（TOF）和肺动脉狭窄、TOF合并肺动脉瓣缺如综合征以及II - A型三尖瓣闭锁的DAA患者的临床表现中占主导地位。1例合并TOF和肺动脉狭窄的患者记录有DiGeorge综合征。合并TOF和肺动脉瓣缺如综合征的患者呼气性哮鸣明显。1例合并室间隔缺损（VSD）以及二尖瓣肺动脉瓣伴轻度瓣膜狭窄的患者有轻度青紫和心力衰竭。钡餐食管造影显示5例患者食管有压迹。3例婴儿经超声心动图检查确诊。4例患者（2例孤立性DAA和2例复杂性DAA）在手术切断较小的左主动脉弓后存活。合并VSD和TOF的DAA患者分别进行了一期和二期手术。另外2例复杂性DAA患者由于对心内畸形相关症状的耐受性而仍在密切观察中。
