Longest Survivor of Pulmonary Atresia With Ventricular Septal Defect
2011; Lippincott Williams & Wilkins; Volume: 124; Issue: 19 Linguagem: Inglês
10.1161/circulationaha.111.035469
ISSN1524-4539
AutoresDaisuke Fukui, Hisashi Kai, Tomohiro Takeuchi, Takeki Gondo, Toyoharu Oba, Kazutoshi Mawatari, Tatsuo Tonai, Y. Matsuo, Shinichiro Ueda, Hiroshi Niiyama, Takafumi Ueno, Tsutomu Imaizumi,
Tópico(s)Cardiac Structural Anomalies and Repair
ResumoHomeCirculationVol. 124, No. 19Longest Survivor of Pulmonary Atresia With Ventricular Septal Defect Free AccessBrief ReportPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplemental MaterialFree AccessBrief ReportPDF/EPUBLongest Survivor of Pulmonary Atresia With Ventricular Septal DefectWell-Developed Major Aortopulmonary Collateral Arteries Demonstrated by Multidetector Computed Tomography Daisuke Fukui, MD, Hisashi Kai, MD, PhD, Tomohiro Takeuchi, MD, PhD, Takeki Gondo, MD, Toyoharu Oba, MD, Kazutoshi Mawatari, MD, Tatsuo Tonai, MD, Yu Matsuo, MD, Shin-ichiro Ueda, MD, Hiroshi Niiyama, MD, PhD, Takafumi Ueno, MD, PhD and Tsutomu Imaizumi, MD, PhD Daisuke FukuiDaisuke Fukui From the Department of Internal Medicine, Division of Cardio-Vascular Medicine, Kurume University School of Medicine (D.F., H.K., T. Takeuchi, T.G., T.O., K.M., T. Tonai, Y.M., S. U., H.N., T.I.) and Center of Cardiovascular Medicine, Kurume University Hospital, Kurume, Fukuoka, Japan (T.U.). , Hisashi KaiHisashi Kai From the Department of Internal Medicine, Division of Cardio-Vascular Medicine, Kurume University School of Medicine (D.F., H.K., T. Takeuchi, T.G., T.O., K.M., T. Tonai, Y.M., S. U., H.N., T.I.) and Center of Cardiovascular Medicine, Kurume University Hospital, Kurume, Fukuoka, Japan (T.U.). , Tomohiro TakeuchiTomohiro Takeuchi From the Department of Internal Medicine, Division of Cardio-Vascular Medicine, Kurume University School of Medicine (D.F., H.K., T. Takeuchi, T.G., T.O., K.M., T. Tonai, Y.M., S. U., H.N., T.I.) and Center of Cardiovascular Medicine, Kurume University Hospital, Kurume, Fukuoka, Japan (T.U.). , Takeki GondoTakeki Gondo From the Department of Internal Medicine, Division of Cardio-Vascular Medicine, Kurume University School of Medicine (D.F., H.K., T. Takeuchi, T.G., T.O., K.M., T. Tonai, Y.M., S. U., H.N., T.I.) and Center of Cardiovascular Medicine, Kurume University Hospital, Kurume, Fukuoka, Japan (T.U.). , Toyoharu ObaToyoharu Oba From the Department of Internal Medicine, Division of Cardio-Vascular Medicine, Kurume University School of Medicine (D.F., H.K., T. Takeuchi, T.G., T.O., K.M., T. Tonai, Y.M., S. U., H.N., T.I.) and Center of Cardiovascular Medicine, Kurume University Hospital, Kurume, Fukuoka, Japan (T.U.). , Kazutoshi MawatariKazutoshi Mawatari From the Department of Internal Medicine, Division of Cardio-Vascular Medicine, Kurume University School of Medicine (D.F., H.K., T. Takeuchi, T.G., T.O., K.M., T. Tonai, Y.M., S. U., H.N., T.I.) and Center of Cardiovascular Medicine, Kurume University Hospital, Kurume, Fukuoka, Japan (T.U.). , Tatsuo TonaiTatsuo Tonai From the Department of Internal Medicine, Division of Cardio-Vascular Medicine, Kurume University School of Medicine (D.F., H.K., T. Takeuchi, T.G., T.O., K.M., T. Tonai, Y.M., S. U., H.N., T.I.) and Center of Cardiovascular Medicine, Kurume University Hospital, Kurume, Fukuoka, Japan (T.U.). , Yu MatsuoYu Matsuo From the Department of Internal Medicine, Division of Cardio-Vascular Medicine, Kurume University School of Medicine (D.F., H.K., T. Takeuchi, T.G., T.O., K.M., T. Tonai, Y.M., S. U., H.N., T.I.) and Center of Cardiovascular Medicine, Kurume University Hospital, Kurume, Fukuoka, Japan (T.U.). , Shin-ichiro UedaShin-ichiro Ueda From the Department of Internal Medicine, Division of Cardio-Vascular Medicine, Kurume University School of Medicine (D.F., H.K., T. Takeuchi, T.G., T.O., K.M., T. Tonai, Y.M., S. U., H.N., T.I.) and Center of Cardiovascular Medicine, Kurume University Hospital, Kurume, Fukuoka, Japan (T.U.). , Hiroshi NiiyamaHiroshi Niiyama From the Department of Internal Medicine, Division of Cardio-Vascular Medicine, Kurume University School of Medicine (D.F., H.K., T. Takeuchi, T.G., T.O., K.M., T. Tonai, Y.M., S. U., H.N., T.I.) and Center of Cardiovascular Medicine, Kurume University Hospital, Kurume, Fukuoka, Japan (T.U.). , Takafumi UenoTakafumi Ueno From the Department of Internal Medicine, Division of Cardio-Vascular Medicine, Kurume University School of Medicine (D.F., H.K., T. Takeuchi, T.G., T.O., K.M., T. Tonai, Y.M., S. U., H.N., T.I.) and Center of Cardiovascular Medicine, Kurume University Hospital, Kurume, Fukuoka, Japan (T.U.). and Tsutomu ImaizumiTsutomu Imaizumi From the Department of Internal Medicine, Division of Cardio-Vascular Medicine, Kurume University School of Medicine (D.F., H.K., T. Takeuchi, T.G., T.O., K.M., T. Tonai, Y.M., S. U., H.N., T.I.) and Center of Cardiovascular Medicine, Kurume University Hospital, Kurume, Fukuoka, Japan (T.U.). Originally published8 Nov 2011https://doi.org/10.1161/CIRCULATIONAHA.111.035469Circulation. 2011;124:2155–2157A 59-year-old woman was admitted because of cyanosis and dyspnea on exertion and at rest. In her childhood, she was suspected of having ventricular septal defect (VSD), but she refused to undergo cardiac catheterization and operation. Dyspnea on exertion gradually developed after adolescence.On admission, chest roentgenography demonstrated enlarged cardiac silhouette with elevated cardiac apex, a right aortic arch, and enlargement of the main pulmonary arteries and their major branches with increased pulmonary arterial vascularity (Figure 1). Echocardiography revealed a large VSD which lay beneath the dilated aorta that overrides the interventricular septum, hypertrophied right ventricle, and the blind outflow tract of the right ventricle (Figure 2 and online-only Data Supplement Movies I and II). These findings suggested pulmonary atresia (PA) with VSD (PA-VSD).Download figureDownload PowerPointFigure 1. Chest roentgenogram in an antero-posterior projection demonstrating enlarged cardiac silhouette with elevated cardiac apex, a right aortic arch, and the enlargement of the main pulmonary arteries and their major branches with increased pulmonary arterial vascularity.Download figureDownload PowerPointFigure 2. Transthoracic echocardiographic examination. A, Parasternal long-axis image demonstrating a large VSD (arrowhead), which lay beneath the dilated aorta that overrides the interventricular septum, and hypertrophied right ventricle. Color flow Doppler image showed the right-to-left shunt flow through VSD and the turbulent flow of aortic regurgitation. B, Parasternal short-axis image at the basal level depicting the blind right ventricular outflow tract (open arrow). Ao indicates aorta; LA, left atrium; LV, left ventricle; LVOFT, left ventricular outflow tract; RA, right atrium; and RV, right ventricle.The volume-rendering 64-multidetecter computed tomography (MDCT) images visualized multiple well-developed arterial collaterals, namely major aortopulmonary collateral arteries (MAPCAs) (Figure 3A). The mediastinum was occupied by the multiple MAPCAs, mimicking the cavernous tissue (Figure 3B). Several MAPCAs originated from the descending aorta (Figure 3C).Download figureDownload PowerPointFigure 3. A, The volume-rendering 64-MDCT images of the hearts and multiple well-developed MAPCAs. B, Mutidetector computed tomography images demonstrating that multiple MAPCAs formed the cavernous tissue-like appearance in the mediastinum (open arrows). C, Representative MDCT image showing that a large MAPCA originated from the descending aorta (black arrow). Ao indicates aorta; LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle; and SVC, superior vena cava.Pulmonary atresia with VSD is the ultimate form of tetralogy of Fallot and is estimated to represent 5% to 10% of tetralogy of Fallot patients.1 The survival rate without surgical repair is as low as 50% at 1 year of age and 8% at 10 years.2 Adult survivors of PA-VSD are quite rare: Marelli et al reported that the mean life expectancy without operation did not exceed 3 decades,3 and the oldest reported survivors were 54 years old.3,4 To the best of our knowledge, this patient is the oldest reported survivor of PA-VSD.Survival of PA-VSD patients is dependent on the adequacy of pulmonary blood flow derived from direct or indirect aortopulmonary collateral vessels. The well-developed MAPCAs might have enabled this patient to survive to nearly 60 years of age. Multidetecter computed tomography is a valuable noninvasive imaging modality to evaluate the development and sources of MAPCAs in PA-VSD.DisclosuresNone.FootnotesThe online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161/CIRCULATIONAHA.111.035469/-/DC1Correspondence to Hisashi Kai, MD, PhD, FAHA, Department of Internal Medicine, Division of Cardio-Vascular Medicine, Kurume University School of Medicine, 67 Asahimachi, Kurume, Fukuoka 830-0011, Japan. E-mail [email protected]kurume-u.ac.jp.References1. Perloff J. Ventricular septal defect with pulmonary stenosis. In: , Perloff J, ed. Clinical Recognition of Congenital Heart Disease. 4th ed. Philadelphia, Pa: Saunders Co.;1994:440–482.Google Scholar2. Bertranou EG, Blackstone EH, Hazelrig JB, Turner ME, Kirklin JW. Life expectancy without surgery in tetralogy of Fallot. Am J Cardiol. 1978; 42:458–466.CrossrefMedlineGoogle Scholar3. Marelli AJ, Perloff JK, Child JS, Laks H. Pulmonary atresia with ventricular septal defect in adults. Circulation. 1994; 89:243–251.LinkGoogle Scholar4. Smiterton TC, Nimetz AA, Freidrich AL. Pulmonary atresia with ventricular septal defect: report of the oldest surviving case. Chest. 1975; 67:603–606.CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Achour A, Mnari W, Abdelali M, Zrig A, Miladi A, Ben Messaoud M, Hmida B and Maatouk M (2020) Case Report: A forty year-survivor of Tetralogy of Fallot with pulmonary atresia and chronic pediatric shunt thrombosis; findings from cardiac CT scan, F1000Research, 10.12688/f1000research.24374.1, 9, (647) Sarawagi A, Sodani V, Sodani R and Verma M (2019) Stratification of Tetralogy of Fallot and Status of Pulmonary Artery by Cardiac CT (Pulmonary Angiography), Journal of Evolution of Medical and Dental Sciences, 10.14260/jemds/2019/605, 8:36, (2787-2792), Online publication date: 9-Sep-2019. Hirst C, Jureidini S and Hauptman P (2015) Arrhythmias in a long-term adult survivor with uncorrected tetralogy of Fallot: Case report and review of the literature, Journal of Electrocardiology, 10.1016/j.jelectrocard.2015.04.006, 48:4, (734-738), Online publication date: 1-Jul-2015. Dobrocky T, Klink T, Weisstanner C, Heverhagen J and Christe A (2014) Imaging findings in uncorrected tetralogy of Fallot and pulmonary atresia with major aortopulmonary collateral arteries and septic embolism, Acta Radiologica Short Reports, 10.1177/2047981613515211, 3:1, (204798161351521), Online publication date: 1-Jan-2014. Shah A, Rhodes J and Jaquiss R (2014) Pulmonary Atresia with Ventricular Septal Defect Pediatric and Congenital Cardiology, Cardiac Surgery and Intensive Care, 10.1007/978-1-4471-4619-3_19, (1527-1542), . Burch M and Dedieu N (2013) Almanac 2012: Congenital heart disease. The national society journals present selected research that has driven recent advances in clinical cardiology, Revista Portuguesa de Cardiologia, 10.1016/j.repc.2012.10.008, 32:3, (269-277), Online publication date: 1-Mar-2013. Mazur W, Siegel M, Miszalski-Jamka T and Pelberg R (2013) Right Ventricular Outflow Tract Obstruction CT Atlas of Adult Congenital Heart Disease, 10.1007/978-1-4471-5088-6_14, (131-146), . Burch M and Dedieu N (2013) Almanac 2012: Congenital heart disease. The national society journals present selected research that has driven recent advances in clinical cardiology, The Egyptian Heart Journal, 10.1016/j.ehj.2012.10.004, 65:1, (21-29), Online publication date: 1-Mar-2013. Burch M and Dedieu N (2013) Almanac 2012: Congenital heart disease. The national society journals present selected research that has driven recent advances in clinical cardiology, Revista Portuguesa de Cardiologia (English Edition), 10.1016/j.repce.2013.04.008, 32:3, (269-277), Online publication date: 1-Mar-2013. Burch M and Dedieu N (2013) Almanac 2012: Congenital heart disease, Archivos de Cardiología de México, 10.1016/j.acmx.2013.01.004, 83:1, (55-63), Online publication date: 1-Jan-2013. November 8, 2011Vol 124, Issue 19 Advertisement Article InformationMetrics © 2011 American Heart Association, Inc.https://doi.org/10.1161/CIRCULATIONAHA.111.035469PMID: 22064959 Originally publishedNovember 8, 2011 PDF download Advertisement SubjectsComputerized Tomography (CT)Congenital Heart DiseaseEchocardiography
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