Scott Bennett, Lisa K Hornberger, Deborah Fruitman, Timothy J Bradley, Gitanjali P Mansukhani
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引用次数: 0
摘要
目的:肺动脉斜坡是一种罕见的先天性畸形,占导致气道阻塞的血管畸形患者总数的 2%。在正常心脏中,左肺动脉(LPA)和右肺动脉(RPA)发源于心包内间隙。然而,在肺动脉吊带中,左肺动脉干从 RPA 中段的后方发源于心包外间隙,并向气管后方延伸,导致气管受压,有时还会导致支气管受压。虽然先天性心脏病的所有病变都可以在出生前发现,但只有少数病例报告记录了左肺动脉吊带(LPAS)的产前诊断:方法:我们回顾性地鉴定了加拿大三家胎儿心脏病学中心(2015-2022年)的所有产前LPAS病例:结果:通过胎儿超声心动图(FE)的三血管-气管视图,三胎中的四名胎儿显示出异常的LPA起源于RPA和回声气管。在两个受影响的单绒毛膜双胎中,有一个的冠状造影显示大气管明显狭窄,与明显的气道阻塞一致:结论:通过 FE 可以在产前检测到 LPAS,并应及时评估冠状切面的气道阻塞情况。建议对相关病变进行调查并进行基因检测,以做出知情的共同决策。
Fetal diagnosis and management of pulmonary artery sling: A case series.
Objective: Pulmonary artery sling is a rare congenital anomaly accounting for 2% of all patients with vascular anomalies that cause airway obstruction. In the normal heart, the left (LPA) and right (RPA) pulmonary arteries arise in the intrapericardial space. However, in the pulmonary artery sling, the LPA trunk arises in the extrapericardial space from the posterior aspect of the mid RPA and courses posterior to the trachea causing tracheal compression and, at times, bronchial compression. While a full spectrum of congenital cardiac pathology can be identified before birth, only a few case reports document the prenatal diagnosis of an Left pulmonary artery sling (LPAS).
Method: We retrospectively identified all cases of prenatal LPAS from three Canadian fetal cardiology centers (2015-2022).
Results: Using the 3-vessel-tracheal view via fetal echocardiography (FE), four fetuses from three pregnancies demonstrated abnormal origin of the LPA from RPA and echogenic trachea. In one of two affected monochorionic twins coronal imaging demonstrated a significant narrowing of the large airways consistent with significant airway obstruction.
Conclusion: Prenatal detection of LPAS by FE is possible and should prompt an evaluation for airway obstruction in the coronal view. Investigating associated lesions and genetic testing are recommended for informed shared decision making.
期刊介绍:
Prenatal Diagnosis welcomes submissions in all aspects of prenatal diagnosis with a particular focus on areas in which molecular biology and genetics interface with prenatal care and therapy, encompassing: all aspects of fetal imaging, including sonography and magnetic resonance imaging; prenatal cytogenetics, including molecular studies and array CGH; prenatal screening studies; fetal cells and cell-free nucleic acids in maternal blood and other fluids; preimplantation genetic diagnosis (PGD); prenatal diagnosis of single gene disorders, including metabolic disorders; fetal therapy; fetal and placental development and pathology; development and evaluation of laboratory services for prenatal diagnosis; psychosocial, legal, ethical and economic aspects of prenatal diagnosis; prenatal genetic counseling