Congenital heart disease and sudden death in the young
Identifieur interne : 002259 ( Main/Exploration ); précédent : 002258; suivant : 002260Congenital heart disease and sudden death in the young
Auteurs : Cristina Basso [Italie] ; Carla Frescura [Italie] ; Domenico Corrado [Italie] ; Michela Muriago [Italie] ; Annalisa Angelini [Italie] ; Luciano Daliento [Italie] ; Gaetano Thiene [Italie]Source :
- Human Pathology [ 0046-8177 ] ; 1995.
Abstract
Sudden death is a frequent mode of fatal outcome in cardiac disease and does not exclude young people. The aim of this investigation was to establish whether and to what extent sudden death in the young may be ascribable to the substrate of underlying congenital heart disease. Among 182 young people (≤ 35 years) who died of cardiac sudden death and underwent postmortem examination, 58 (32%) had congenital heart disease. Seven showed an intrapericardial rupture of aortic dissection, in the setting of Marfan syndrome in two, isolated bicuspid aortic valve in two, and bicuspid aortic valve and isthmic coarctation in three; all exhibited equally severe degeneration of the aortic wall. Sixteen cases had conduction system anomalies, mostly bypass tracts; 15 coronary artery anomalies (three ostial valve-like stenosis, five origin from the wrong aortic sinus, and seven deep intramyocardial course); 12 hypertrophic cardiomyopathy; five postoperative congenital heart disease including scar following ventriculotomy, conduction system injury, and defects left unrepaired; and three congenital aortic valve stenosis. One third of sudden deaths in the young was ascribable to structural defects present since birth. A large spectrum of congenital heart disease involves the risk of sudden death, but most structural defects are usually not considered to be life threatening. Some of these concealed defects are potentially detectable in life by clinical imaging techniques.
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DOI: 10.1016/0046-8177(95)90267-8
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The aim of this investigation was to establish whether and to what extent sudden death in the young may be ascribable to the substrate of underlying congenital heart disease. Among 182 young people (≤ 35 years) who died of cardiac sudden death and underwent postmortem examination, 58 (32%) had congenital heart disease. Seven showed an intrapericardial rupture of aortic dissection, in the setting of Marfan syndrome in two, isolated bicuspid aortic valve in two, and bicuspid aortic valve and isthmic coarctation in three; all exhibited equally severe degeneration of the aortic wall. Sixteen cases had conduction system anomalies, mostly bypass tracts; 15 coronary artery anomalies (three ostial valve-like stenosis, five origin from the wrong aortic sinus, and seven deep intramyocardial course); 12 hypertrophic cardiomyopathy; five postoperative congenital heart disease including scar following ventriculotomy, conduction system injury, and defects left unrepaired; and three congenital aortic valve stenosis. One third of sudden deaths in the young was ascribable to structural defects present since birth. A large spectrum of congenital heart disease involves the risk of sudden death, but most structural defects are usually not considered to be life threatening. Some of these concealed defects are potentially detectable in life by clinical imaging techniques.</div></front></TEI><affiliations><list><country><li>Italie</li></country></list><tree><country name="Italie"><noRegion><name sortKey="Basso, Cristina" sort="Basso, Cristina" uniqKey="Basso C" first="Cristina" last="Basso">Cristina Basso</name></noRegion><name sortKey="Angelini, Annalisa" sort="Angelini, Annalisa" uniqKey="Angelini A" first="Annalisa" last="Angelini">Annalisa Angelini</name><name sortKey="Corrado, Domenico" sort="Corrado, Domenico" uniqKey="Corrado D" first="Domenico" last="Corrado">Domenico Corrado</name><name sortKey="Daliento, Luciano" sort="Daliento, Luciano" uniqKey="Daliento L" first="Luciano" last="Daliento">Luciano Daliento</name><name sortKey="Frescura, Carla" sort="Frescura, Carla" uniqKey="Frescura C" first="Carla" last="Frescura">Carla Frescura</name><name sortKey="Muriago, Michela" sort="Muriago, Michela" uniqKey="Muriago M" first="Michela" last="Muriago">Michela Muriago</name><name sortKey="Thiene, Gaetano" sort="Thiene, Gaetano" uniqKey="Thiene G" first="Gaetano" last="Thiene">Gaetano Thiene</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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