Division of Pediatric Cardiology, National Research Center-Tuscany Region Foundation "G. Monasterio", Ospedale del Cuore, via Aurelia Sud, 54100, Massa, Italy.
Specialization School in Diseases of Cardiovascular System, University of Pisa, AOU Pisana, via Paradisa 2, 56124, Pisa, Italy.
Int J Cardiovasc Imaging. 2021 Dec;37(12):3385-3395. doi: 10.1007/s10554-021-02325-w. Epub 2021 Jul 8.
To study the different characteristics of arterial duct (AD) in a series of prenatally detected right aortic arch (RAA). Out of 832 congenital heart diseases (CHD) referred to a tertiary center, 98 cases had RAA. Based on anatomical landmarks we identified 7 types of AD: type 1 left-sided, transverse; type 2 left-sided, vertical; type 3 from the underside of aortic arch (AA), vertical; type 4 right-sided, mirror-image "V", transverse; type 5 right-sided, "H" shaped, transverse; type 6 bilateral; type 7 absent or unidentifiable. For each type of AD the incidence of associated major CHD was calculated and chi-square test was applied to verify the null hypothesis with significance level of p < 0.05. Type 1 occurred in 43% of cases including 4 with CHD and no cases with pulmonary outflow obstruction (POO). Symptoms of vascular ring were present in 41% of survivors. Type 2, 3 and 7 AD were associated with tetralogy of Fallot (TOF) or equivalents. No type 5 AD with CHD had POO and 3 isolated cases had asymptomatic hypoplasia of left pulmonary artery (LPA). Two type 6 AD had disconnection of LPA. Type 1 occurred more often as an isolated finding (p < 0.001), whereas types 2 (p = 0.0026), 3 (p = 0.0045), 4 (p = 0.0325) and 7 (p = 0.0001) were frequently associated with major CHD. In RAA, type 1 (U-shaped) is usually an isolated finding (p < 0.001) which includes all symptomatic vascular rings. POO is always present when the AD is vertical or absent but not when it lies on a transverse plane. Bilateral AD is rare and brings the risk of functionary loss of left lung if not identified.
研究一系列产前检测到的右主动脉弓(RAA)中动脉导管(AD)的不同特征。在转诊至三级中心的 832 例先天性心脏病(CHD)中,有 98 例存在 RAA。根据解剖学标志,我们确定了 7 种类型的 AD:1 型为左侧、横向;2 型为左侧、垂直;3 型为主动脉弓(AA)底部、垂直;4 型为右侧、镜像“V”形、横向;5 型为右侧、“H”形、横向;6 型为双侧;7 型为缺失或无法识别。对于每种类型的 AD,计算了相关主要 CHD 的发生率,并应用卡方检验来验证具有统计学意义的 p<0.05 的零假设。1 型发生在 43%的病例中,包括 4 例 CHD 患者和无肺流出道梗阻(POO)的患者。41%的幸存者存在血管环症状。2、3 和 7 型 AD 与法洛四联症(TOF)或等效物相关。无 CHD 的 5 型 AD 伴有 POO,3 例孤立性左肺动脉(LPA)发育不全。2 例 6 型 AD 存在 LPA 分离。1 型更常作为孤立性发现(p<0.001),而 2 型(p=0.0026)、3 型(p=0.0045)、4 型(p=0.0325)和 7 型(p=0.0001)常与主要 CHD 相关。在 RAA 中,1 型(U 形)通常为孤立性发现(p<0.001),包括所有有症状的血管环。当 AD 垂直或缺失时,POO 总是存在,但当 AD 位于横平面时则不存在。双侧 AD 罕见,如果未识别,会导致左肺功能丧失。