PDF(Pubmed)
Abstract：
Published data estimate the prevalence of the vascular ring at approximately 7 per 10,000 live births. The association of a double aortic arch with a D-transposition of the great arteries has been rarely described in the literature. In this study, we report the prenatal diagnosis of a 28-year-old woman. A fetal echocardiography at a gestational age of 24 weeks + 6 days showed a D-transposition of the great arteries and a double aortic arch with a ventricular septal defect and pulmonary stenosis. On the first night after birth, the baby experienced an increase in lactate levels, with the rate of oxygen saturation consistently below 80%. A few hours after birth, the patient underwent a Rashkind procedure. An echocardiography, CT chest x-ray, and CT angiogram confirmed a diagnosis with a severe reduction of the tracheal lumen (>85%) and bronchomalacia. Then, the patient underwent posterior tracheopexy and aortopexy and later an arterial switch operation, ventricular septal defect closure, and resection of a part of the infundibular septum, accepting the risk of potential neoaortic obstruction. The literature has reported only two cases of patients with a fetal echocardiogram diagnosis. Therefore, our patient is only the third one with a fetal diagnosis and the second one with a complex intracardiac anatomy, characterized not only by a ventricular septal defect but also by two separate components of the obstruction (a bicuspid valve and a dysplastic valve with a posterior deviation of the infundibular septum). In conclusion, a D-transposition of the great arteries with a double aortic arch remains an extremely unusual association. The clinical outcome of these patients presents a high degree of variability and is entirely unpredictable in prenatal life. Our greatest aim as fetal and perinatal cardiologists is to improve the management and outcome of these patients through a fetal diagnosis, recognizing types of congenital heart disease in newborns who require early neonatal invasive procedures.
摘要：
已发布的数据估计血管环的患病率约为每10,000例活产7。文献中很少描述双主动脉弓与大动脉D转位的关联。在这项研究中,我们报告了一名28岁女性的产前诊断.胎龄为24周6天的胎儿超声心动图检查显示大动脉D移位和双主动脉弓伴室间隔缺损和肺动脉狭窄。出生后的第一个晚上，婴儿经历了乳酸水平的增加，氧饱和度始终低于80%。出生后几个小时，患者接受了Rashkind手术.超声心动图,胸部X线CT,CT血管造影证实诊断为气管腔严重缩小（>85％）和支气管软化。然后，患者接受了后气管固定术和主动脉固定术，随后进行了动脉转换手术，室间隔缺损闭合术,切除漏斗状隔膜的一部分，接受潜在的新主动脉阻塞的风险。文献仅报道了2例胎儿超声心动图诊断的患者。因此,我们的病人只有第三个有胎儿诊断，第二个有复杂的心内解剖，不仅表现为室间隔缺损，而且表现为阻塞的两个独立部分（二尖瓣和发育不良瓣膜，漏斗状隔膜后偏）。总之,大动脉的D转位与双主动脉弓仍然是一个非常不寻常的关联。这些患者的临床结果表现出高度的变异性，并且在产前生活中是完全不可预测的。作为胎儿和围产期心脏病专家，我们的最大目标是通过胎儿诊断来改善这些患者的管理和预后。识别需要早期新生儿侵入性手术的新生儿先天性心脏病的类型。
