Fetal Cardiology Unit, Department of Congenital Heart Disease, Evelina London Children's Healthcare, Guy's and St Thomas' NHS Foundation Trust, London, UK.
Harris Birthright Centre, Fetal Medicine Research Institute, King's College Hospital, NHS Foundation Trust, London, UK.
Prenat Diagn. 2021 Aug;41(9):1127-1133. doi: 10.1002/pd.6007. Epub 2021 Jul 8.
Prenatal recognition of dilated aortic root is extremely rare and there are significant challenges in counselling these patients. The primary aim of this case series is to describe the prevalence, associations and outcome of dilated ascending aorta diagnosed during fetal life.
This is a retrospective cohort study from two tertiary fetal cardiology centres. Dilated ascending aorta was defined as gestation-specific standard deviation > 1.96 at some point during gestation.
Sixteen infants were live born and underwent postnatal echocardiography. Prenatally suspected bicuspid aortic valve (BAV) (n = 6) was confirmed in 5 cases (83%) postnatally. Thirteen children have been followed up for a period of minimum one year. No connective tissue disease was found.
Prenatal dilated ascending aorta is a rare finding (0.06%). It is associated with BAV in 37% of cases and extracardiac abnormalities in 15.7%. Nuchal translucency measurement was >3.5 in 13% of cases. Connective tissue disease was not diagnosed postnatally. This is the largest prenatal cohort with dilated ascending aorta and postnatal outcomes to date. We showed a postnatal persistence of ascending aortic dilatation in 43% of babies. In the absence of extra-cardiac abnormalities, medium term outcome appears good but postnatal surveillance of aortic dilation is required.
产前发现扩张性主动脉根部极为罕见,对这些患者进行咨询存在重大挑战。本病例系列的主要目的是描述在胎儿期诊断的升主动脉扩张的患病率、相关性和结局。
这是来自两个三级胎儿心脏病学中心的回顾性队列研究。在妊娠期间的某个时间点,升主动脉扩张定义为特定于妊娠的标准偏差>1.96。
16 名婴儿存活并接受了产后超声心动图检查。产前怀疑二叶式主动脉瓣(BAV)(n=6)在 5 例(83%)中得到了产后证实。13 名儿童已随访至少一年。未发现结缔组织疾病。
产前升主动脉扩张是一种罕见发现(0.06%)。它与 37%的 BAV 病例和 15.7%的心脏外异常相关。13%的病例中颈后透明带厚度>3.5。产后未诊断出结缔组织疾病。这是迄今为止最大的产前伴有升主动脉扩张的队列和产后结局。我们显示 43%的婴儿在产后存在升主动脉扩张持续存在。在没有心脏外异常的情况下,中期结局似乎良好,但需要对主动脉扩张进行产后监测。