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Fetal Cardiac MRI of Complex Interrupted Aortic Arch

2023; Radiological Society of North America; Volume: 307; Issue: 5 Linguagem: Inglês

10.1148/radiol.223224

1527-1315

Thomas Vollbrecht, Christopher Hart, Julian A. Luetkens,

Congenital Diaphragmatic Hernia Studies

HomeRadiologyVol. 307, No. 5 PreviousNext Reviews and CommentaryFree AccessImages in RadiologyFetal Cardiac MRI of Complex Interrupted Aortic ArchThomas M. Vollbrecht, Christopher Hart, Julian A. Luetkens Thomas M. Vollbrecht, Christopher Hart, Julian A. Luetkens Author AffiliationsFrom the Departments of Diagnostic and Interventional Radiology (T.M.V., C.H., J.A.L.) and Pediatric Cardiology (C.H.), University Hospital Bonn, Venusberg-Campus 1, 53127 Bonn, Germany; and Quantitative Imaging Laboratory Bonn (QILaB), Bonn, Germany (T.M.V., J.A.L.).Address correspondence to J.A.L. (email: [email protected]).Thomas M. VollbrechtChristopher HartJulian A. Luetkens Published Online:May 23 2023https://doi.org/10.1148/radiol.223224MoreSectionsPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked In Supplemental material is available for this article.A 32-year-old woman (gravida 2, para 1) at gestational age of 35 weeks and 2 days was referred for prenatal cardiac MRI assessment of suspected type A interrupted aortic arch with type II aortopulmonary window. Doppler US–gated fetal cardiac MRI with use of axial cine and four-dimensional flow imaging confirmed these findings and helped further detect anomalous origin of the right pulmonary artery from the ascending aorta (Figure; Movies S1, S2). This rare constellation is known as Berry syndrome and accounts for approximately 0.046% of all congenital cardiovascular anomalies (1,2). With use of the fetal cardiac MRI scan for surgical planning, a one-stage (total) repair with reconstruction of the aortic arch by using an autologous pericardial patch was successfully performed 5 days after birth. No surgery-related complications occurred, and further development of the neonate was normal after discharge on postoperative day 14. Doppler US–gated fetal cardiac MRI is a novel technique that can routinely provide information about fetal cardiovascular anatomy and hemodynamics (3).(A) Schematic of the embryologic emergent shows the underlying heart defect adopted from the Edwards double arch hypothesis. An incomplete septation of the truncus arteriosus results in an aortopulmonary window (*), which hinders the fusion of the primitive pulmonary arterial branches and results in the anomalous origin of the right pulmonary artery (RPA) from the ascending aorta (AAo). Consequently, the distal segment of the aortic arch obliterates due to reduced blood flow, resulting in a type A interrupted aortic arch. (B) Axial Doppler US–gated fetal cardiac balanced steady-state free precession cine image demonstrates the ascending aorta and the main pulmonary artery (MPA) (arrow) communicating by means of the aortopulmonary window (*). At this level, the right pulmonary artery (arrow) arises from the ascending aorta, and the left pulmonary artery (LPA) (arrow) arises from the main pulmonary artery. The descending aorta (DAo) (arrow) is connected to the main pulmonary artery by means of the arterial duct, while the aortic arch interrupts after the origin of the left subclavian artery (LSA) (not shown in this section). DA = ductus arteriosus, LCCA = left common carotid artery, RBCA = right brachiocephalic artery.Download as PowerPointOpen in Image Viewer Get the Flash Player to see this video.Movie S1: Movie of an axial Doppler ultrasound-gated fetal cardiac MRI cine stack covering the heart and the thoracic vessels. The movie demonstrates the mentioned malformations and reveals normal intracardiac anatomy with no ventricular septal defect.Download Original Video (34.6 MB)Get the Flash Player to see this video.Movie S2: Coronal rotating Doppler ultrasound-gated fetal cardiac 4D flow movie visualizing the type A interrupted aortic arch. Distal to the origin of the left subclavian artery the aortic arch ends blindly and does not communicate with the descending aorta, which is connected with the main pulmonary artery via the ductus arteriosus.Download Original Video (35.9 MB)Disclosures of conflicts of interest: T.M.V. No relevant relationships. C.H. No relevant relationships. J.A.L. Received payments for activities related to the scientific advisory board for Bayer Healthcare; received payments for lectures from Philips.References1. Berry TE, Bharati S, Muster AJ, et al. Distal aortopulmonary septal defect, aortic origin of the right pulmonary artery, intact ventricular septum, patent ductus arteriosus and hypoplasia of the aortic isthmus: a newly recognized syndrome. Am J Cardiol 1982;49(1):108–116. Crossref, Medline, Google Scholar2. Bi WJ, Xiao YJ, Liu YJ, Hou Y, Ren WD. Berry syndrome: a case report and literature review. BMC Cardiovasc Disord 2021;21(1):15. Crossref, Medline, Google Scholar3. Vollbrecht TM, Hart C, Zhang S, et al. Fetal Cardiac Cine MRI with Doppler US Gating in Complex Congenital Heart Disease. Radiol Cardiothorac Imaging. 2023 23;5(1):e220129. Link, Google ScholarArticle HistoryReceived: Dec 19 2022Revision requested: Jan 9 2023Revision received: Feb 6 2023Accepted: Feb 10 2023Published online: May 23 2023 FiguresReferencesRelatedDetailsRecommended Articles Congenital Variants and Anomalies of the Aortic ArchRadioGraphics2016Volume: 37Issue: 1pp. 32-51Isolated Right Common Carotid Artery Arising from the Right Pulmonary ArteryRadiology: Cardiothoracic Imaging2022Volume: 4Issue: 5Role of CT in the Pre- and Postoperative Assessment of Conotruncal AnomaliesRadiology: Cardiothoracic Imaging2022Volume: 4Issue: 3Subclavian Artery: Anatomic Review and Imaging Evaluation of AbnormalitiesRadioGraphics2022Volume: 42Issue: 7pp. 2149-2165Anomalous Origin of the Right Pulmonary Artery from the AortaRadiology2022Volume: 305Issue: 1pp. 35See More RSNA Education Exhibits Imaging Evaluation Of Congenital Abnormalities Of The Aorta: From Embriology To Pathology To 3d Modeling.Digital Posters2021Vascular Rings And Slings: The Jewels Of Cardiac Angiographic ImagingDigital Posters2021The Ductus Arteriosus: A Review Of Embryology, Fetal Imaging, Pathophysiology, And The Role Of Advanced Imaging In PDA Management And Intervention In Children And AdultsDigital Posters2021 RSNA Case Collection Pulmonary AtresiaRSNA Case Collection2022Takayasu arteritisRSNA Case Collection2022Aberrant left subclavian artery from right-sided aortic archRSNA Case Collection2020 Vol. 307, No. 5 Metrics Altmetric Score PDF download