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• W2034336094 abstract "HomeCirculationVol. 105, No. 11Premature Closure of the Foramen Ovale and Ductus Arteriosus in a Fetus With Transposition of the Great Arteries Free AccessOtherPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessOtherPDF/EPUBPremature Closure of the Foramen Ovale and Ductus Arteriosus in a Fetus With Transposition of the Great Arteries Mary T. Donofrio, MD Mary T. DonofrioMary T. Donofrio From the Children’s Heart Center of the Medical College of Virginia Hospitals, Virginia Commonwealth University, Richmond, Va. Search for more papers by this author Originally published19 Mar 2002https://doi.org/10.1161/hc1102.105232Circulation. 2002;105:e65–e66A 32-year-old woman presented for evaluation at 34 weeks gestation complaining of feeling swollen and short of breath for several days. By report, she had received good prenatal care, and a complete ultrasound at 20 weeks gestation revealed no fetal abnormalities and a normal 4-chambered heart. Imaging by the perinatologist prompted immediate referral for a fetal echocardiogram. This study revealed severe cardiomegaly with marked dilation of the left atrium and ventricle (Figure 1). There was normal visceral situs and normal atrioventricular alignment. The pulmonary artery originated from the left ventricle (Figure 2) and the aorta from the right ventricle (Figure 3), making the diagnosis transposition of the great arteries (TGA). The aortic and pulmonary valves were normal in size and architecture. The mitral valve appeared thickened and there was severe regurgitation (Figure 4). The atrial septum was intact and bowed into the right atrium (Figure 5). The ductus arteriosus was identified by 2-dimensional imaging; however, no flow could be documented (Figure 3). The rhythm was sinus at 140 bpm. There was minimal fetal movement and continuous monitoring revealed an abnormal fetal heart rate tracing. Because of the significant concern for fetal well-being, the baby was delivered by Cesarean section emergently in an operating room suite adjacent to the cardiac operating room. On delivery, the baby became immediately cyanotic, limp, and cold. Within seconds, the heart rate dropped to <30 bpm. The baby was intubated, and via a median sternotomy, placed on extracorporeal membrane oxygenation (ECMO) supportusing 2 venous cannulas (1 in each atrial chamber) and 1 aortic cannula. Cannulation was achieved within 6 minutes after delivery, with immediate decompression of the heart and return of a normal heart rate and excellent perfusion. The initial arterial blood gas on ECMO revealed a pH of 7.22 and a Po2 of 485 mm Hg. After 7 days of support, transesophageal echocardiogram revealed significant recovery of the heart. There was mild mitral regurgitation and left ventricular function was good. The findings from the neurological examination and head ultrasound were normal. Viral and metabolic studies to assess for a primary cardiomyopathic process were negative. The baby underwent an arterial switch operation and was successfully weaned from bypass. After 45 minutes, pulmonary hypertension developed and the baby was placed back on ECMO. Within 24 hours, pulmonary artery pressure returned to normal. Unfortunately, during this time, the baby sustained a significant intraventricular hemorrhage in the brain and support was electively withdrawn at the family’s request. Download figureDownload PowerPointFigure 1. Four-chambered view. Note the severe cardiomegaly and dilation of the left atrium and ventricle. LA indicates left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle; S, spine; and Ant, anterior chest.Download figureDownload PowerPointFigure 2. Short-axis view. Note that the pulmonary artery originates from the left ventricle. PA indicates pulmonary artery. See Figure 1 legend for additional abbreviations.Download figureDownload PowerPointFigure 3. Short-axis view. Note that the aorta originates from the right ventricle. A tiny ductus arteriosus may be present. Ao indicates aorta; DA, ductus arteriosus. See Figure 1 legend for additional abbreviations.Download figureDownload PowerPointFigure 4. Four-chambered view. Note the mitral regurgitation (blue jet). MR indicates mitral regurgitation. See Figure 1 legend for additional abbreviations.Download figureDownload PowerPointFigure 5. Four-chambered view. Note that there is no flow across the intact atrial septum. A indicates atrial septum. See Figure 1 legend for additional abbreviations.We believe that this case represents the hemodynamic result of premature foramen ovale and ductus arteriosus closure in a fetus with TGA. Undetected, this combination would have resulted in fetal demise. The immediate delivery of the baby and initiation of ECMO restored a circulation that was compatible with life. This case demonstrates that, in addition to the structural anatomy, a complete assessment of cardiac physiology throughout gestation is very important in the management of fetuses with congenital heart disease.The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke's Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.Circulation encourages readers to submit cardiovascular images to the Circulation Editorial Office, St Luke's Episcopal Hospital/Texas Heart Institute, 6720 Bertner Ave, MC1-267, Houston, TX 77030.FootnotesCorrespondence to Mary T. Donofrio, MD, Associate Professor of Pediatrics, Director of Perinatal Cardiology and Noninvasive Laboratory, Children’s Heart Center, Virginia Commonwealth University, Box 980342, Richmond, VA 23298. E-mail [email protected] Previous Back to top Next FiguresReferencesRelatedDetailsCited By Zhang X, Haneishi H and Liu H (2019) Impact of ductus arteriosus constriction and restrictive foramen ovale on global hemodynamics for term fetuses with d‐TGA, International Journal for Numerical Methods in Biomedical Engineering, 10.1002/cnm.3231, 37:11, Online publication date: 1-Nov-2021. Grzyb A, Koleśnik A, Gruszfeld D and Szymkiewicz-Dangel J (2020) Complete closure of the ductus arteriosus in the foetus with transposition of the great arteries, Cardiology in the Young, 10.1017/S1047951120000529, 30:4, (588-590), Online publication date: 1-Apr-2020. Mawad W, Chaturvedi R, Ryan G and Jaeggi E (2018) Percutaneous Fetal Atrial Balloon Septoplasty for Simple Transposition of the Great Arteries With an Intact Atrial Septum, Canadian Journal of Cardiology, 10.1016/j.cjca.2017.12.010, 34:3, (342.e9-342.e11), Online publication date: 1-Mar-2018. Gu X, Zhang Y, Han J, Liu X, Ge S and He Y (2018) Isolated premature restriction or closure of foramen ovale in fetuses: Echocardiographic characteristics and outcome, Echocardiography, 10.1111/echo.14009, 35:8, (1189-1195), Online publication date: 1-Aug-2018. Vaujois L, Boucoiran I, Preuss C, Brassard M, Houde C, Fouron J and Raboisson M (2017) Relationship between interatrial communication, ductus arteriosus, and pulmonary flow patterns in fetuses with transposition of the great arteries: prediction of neonatal desaturation, Cardiology in the Young, 10.1017/S1047951117000087, 27:7, (1280-1288), Online publication date: 1-Sep-2017. van Velzen C, Türkeri F, Pajkrt E, Clur S, Rijlaarsdam M, Bax C, Hruda J, de Groot C, Blom N and Haak M (2016) Pregnancy complications in singleton pregnancies with isolated fetal heart defects, Acta Obstetricia et Gynecologica Scandinavica, 10.1111/aogs.12955, 95:11, (1273-1280), Online publication date: 1-Nov-2016. Sullivan K, Lacey S, Schrum S, Freid E, Collins S, Bouchard A, Burns S, Poulos N, Walsh D, Ingyinn M, Ettedgui J, Ceithaml E, Jerabek C, Herald T, Castillo R, Trogolo F, Bleiweis M and Hudak M (2014) The Use of Veno-Venous Extracorporeal Membrane Oxygenation for Perinatal Support of an Infant with D-Transposition of the Great Arteries, Intact Atrial and Ventricular Septa, and Flow-Restricted Ductus Arteriosus, A & A Case Reports, 10.1213/XAA.0000000000000022, 2:10, (126-129), Online publication date: 1-May-2014. Donofrio M, Moon-Grady A, Hornberger L, Copel J, Sklansky M, Abuhamad A, Cuneo B, Huhta J, Jonas R, Krishnan A, Lacey S, Lee W, Michelfelder E, Rempel G, Silverman N, Spray T, Strasburger J, Tworetzky W and Rychik J (2014) Diagnosis and Treatment of Fetal Cardiac Disease, Circulation, 129:21, (2183-2242), Online publication date: 27-May-2014. Nakajima J, Kawakami T, Takeuchi K and Tsuchiya K (2012) Dextro-Transposition of the Great Arteries in a Neonate with Ventricular Septal Defect and Pulmonary Stenosis Complicated by Premature Closure of the Ductus Arteriosus and Possible Pathophysiology of Both Defects, Pediatric Cardiology, 10.1007/s00246-012-0557-4, 34:8, (2009-2012), Online publication date: 1-Dec-2013. Lowenthal A, Lal A, Tierney E and Tacy T (2012) Tricuspid Atresia With Progressive Ductal Restriction in a Fetus, Pediatric Cardiology, 10.1007/s00246-012-0391-8, 34:6, (1499-1501), Online publication date: 1-Aug-2013. Chan E and Fai To K (2013) Hydrops fetalis, hepatic centrolobular necrosis, and hypoxic-ischaemic encephalopathy in a fetus with premature closure of foramen ovale, Pathology, 10.1097/PAT.0000000000000010, 45:7, (708-710), Online publication date: 1-Dec-2013. Hernández Herrera R, Ochoa Torres M, Ramos González R and Ramírez Sánchez L (2013) Prenatal diagnosis of foramen ovale premature closure and hydrops fetalis, Diagnóstico Prenatal, 10.1016/j.diapre.2013.03.004, 24:4, (158-160), Online publication date: 1-Oct-2013. Shih J, Huang S, Lin C, Lin T, Su Y, Lin S, Wu E, Shyu M and Lee C (2012) Diagnosis of Transposition of the Great Arteries in the Fetus, Journal of Medical Ultrasound, 10.1016/j.jmu.2012.04.005, 20:2, (65-71), Online publication date: 1-Jun-2012. Punn R and Silverman N (2011) Fetal Predictors of Urgent Balloon Atrial Septostomy in Neonates with Complete Transposition, Journal of the American Society of Echocardiography, 10.1016/j.echo.2010.12.020, 24:4, (425-430), Online publication date: 1-Apr-2011. Al-Naami G and Al-Mesned A (2008) Transposition of Great Arteries with Constrictive Ductus Arteriosus Revisited, Pediatric Cardiology, 10.1007/s00246-007-9189-5, 29:4, (827-829), Online publication date: 1-Jul-2008. Chiou H, Moon-Grady A, Rodriguez R, Konia T, Parrish M and Milstein J (2008) A rare lethal combination of premature closure of the foramen ovale and d-transposition of the great arteries with intact ventricular septum, International Journal of Cardiology, 10.1016/j.ijcard.2007.11.055, 130:2, (e57-e59), Online publication date: 1-Nov-2008. Yoo S, Golding F and Jaeggi E (2008) Ventricular outflow tract anomalies: so-called conotruncal anomalies Fetal Cardiology, 10.3109/9781439807989.021, (305-328), Online publication date: 1-Dec-2008. Ashworth M, Al Adnani M and Sebire N (2006) Neonatal death due to transposition in association with premature closure of the oval foramen, Cardiology in the Young, 10.1017/S1047951106000850, 16:6, (586-589), Online publication date: 1-Dec-2006. Donofrio M, Bremer Y and Moskowitz W (2004) Diagnosis and management of restricted or closed foramen ovale in fetuses with congenital heart disease, The American Journal of Cardiology, 10.1016/j.amjcard.2004.07.133, 94:10, (1348-1351), Online publication date: 1-Nov-2004. March 19, 2002Vol 105, Issue 11 Advertisement Article InformationMetrics https://doi.org/10.1161/hc1102.105232 Originally publishedMarch 19, 2002 PDF download Advertisement" @default.
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