Untreated Tetralogy of Fallot With Pulmonary Atresia in a 55-Year-Old Woman

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HomeCirculationVol. 110, No. 18Untreated Tetralogy of Fallot With Pulmonary Atresia in a 55-Year-Old Woman Free AccessReview ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessReview ArticlePDF/EPUBUntreated Tetralogy of Fallot With Pulmonary Atresia in a 55-Year-Old WomanFindings From Magnetic Resonance Imaging Michael Puderbach, Joachim Eichhorn, Christian Fink and Hans-Ulrich Kauczor Michael PuderbachMichael Puderbach From Forschungsschwerpunkt E, Innovative Krebsdiagnostik und -therapie E010, Deutsches Krebsforschungszentrum (DKFZ), (M.P., C.F., H.-U.K.), and the Department of Cardiology, Childrens’ Hospital, University of Heidelberg (J.E.), Heidelberg, Germany. Search for more papers by this author , Joachim EichhornJoachim Eichhorn From Forschungsschwerpunkt E, Innovative Krebsdiagnostik und -therapie E010, Deutsches Krebsforschungszentrum (DKFZ), (M.P., C.F., H.-U.K.), and the Department of Cardiology, Childrens’ Hospital, University of Heidelberg (J.E.), Heidelberg, Germany. Search for more papers by this author , Christian FinkChristian Fink From Forschungsschwerpunkt E, Innovative Krebsdiagnostik und -therapie E010, Deutsches Krebsforschungszentrum (DKFZ), (M.P., C.F., H.-U.K.), and the Department of Cardiology, Childrens’ Hospital, University of Heidelberg (J.E.), Heidelberg, Germany. Search for more papers by this author and Hans-Ulrich KauczorHans-Ulrich Kauczor From Forschungsschwerpunkt E, Innovative Krebsdiagnostik und -therapie E010, Deutsches Krebsforschungszentrum (DKFZ), (M.P., C.F., H.-U.K.), and the Department of Cardiology, Childrens’ Hospital, University of Heidelberg (J.E.), Heidelberg, Germany. Search for more papers by this author Originally published2 Nov 2004https://doi.org/10.1161/01.CIR.0000146789.79192.08Circulation. 2004;110:e461–e462A 55-year-old woman with congenital heart disease and increasing dyspnea at low levels of exertion was referred to our department for imaging. Clinical findings were cyanosis of the lips and clubbing of the fingers. The past medical history included several hospitalizations for dyspnea, tachycardia, recurrent pulmonary embolism, and suspected cerebral embolism.Despite several conventional angiographic studies, the exact classification of the congenital heart disease and the vascular abnormalities had not been made because of the complex cardiovascular morphology. The presumptive diag- nosis was truncus arteriosus type IV. Differential diagnosis included tetralogy of Fallot with pulmonary atresia.To reevaluate the morphology and to assess acute pulmonary, cardiac, and thoracic vessel problems, MRI was performed (Figures 1 to 4). It revealed a ventricular septal defect with overriding aorta, pulmonal atresia with collateral blood supply by major aortopulmonary collateral arteries, and collateral arteries originating from the right phrenic arteries and intercostal arteries. These findings are consistent with tetralogy of Fallot with pulmonary atresia. Download figureDownload PowerPointFigure 1. Coronal-targeted maximum intensity projection (MIP) of a contrast-enhanced 3D magnetic resonance angiography. Lack of pulmonary arteries in pulmonary atresia. Visualization of collateral blood supply of the lungs: Major aortopulmonary collateral arteries (MAPCA) (black arrow) and additional collateral arteries originating from the right phrenic arteries (white arrow) and intercostal arteries (curved arrow).Download figureDownload PowerPointFigure 2. Coronal-targeted maximum intensity projection (MIP) of a contrast-enhanced 3D magnetic resonance angiography. Visualization of predominantly right-sided dilated intercostal arteries representing collateral blood supply to the right lung in pulmonary atresia.Download figureDownload PowerPointFigure 3. Parasagittal-targeted maximum intensity projection (MIP) of a contrast-enhanced 3D magnetic resonance angiography. Visualization of the ventricular septal defect (arrow) with overriding aorta in tetralogy of Fallot.Download figureDownload PowerPointFigure 4. Selected images of 4-chamber view from cardiac cine MRI over the cardiac cycle. Visualization of the ventricular septal defect with overriding aorta. Jet over the ventricular septal defect from the right to the left ventricle representing Eisenmenger’s reaction. LA indicates left atrium; RA, right atrium; LV, left ventricle; RV, right ventricle; Ao, aorta; arrow, ventricular septal defect; and open arrow, jet over the ventricular septal defect).The online-only Data Supplement, which contains Movies I and II, is available with this article at http://www.circulationaha.org.FootnotesCorrespondence to Michael Puderbach, MD, Forschungsschwerpunkt E, Innovative Krebsdiagnostik und -therapie E010, Deutsches Krebsforschungszentrum (DKFZ), Im Neuenheimer Feld 280, 69120 Heidelberg, Germany. E-mail [email protected] Previous Back to top Next FiguresReferencesRelatedDetailsCited By (2009) Tetralogy of Fallot Congenital Heart Defects, 10.1007/978-3-7985-1719-6_8, (56-67), . November 2, 2004Vol 110, Issue 18 Advertisement Article InformationMetrics https://doi.org/10.1161/01.CIR.0000146789.79192.08PMID: 15520329 Originally publishedNovember 2, 2004 PDF download Advertisement SubjectsAngiographyComputerized Tomography (CT)Heart Failure