{"id":2891,"date":"2022-05-11T15:45:25","date_gmt":"2022-05-11T13:45:25","guid":{"rendered":"https:\/\/dev.ircpss.com\/quest-ce-quun-shunt-porto-systemique-congenital\/"},"modified":"2025-11-18T09:53:44","modified_gmt":"2025-11-18T08:53:44","slug":"quest-ce-quun-shunt-porto-systemique-congenital","status":"publish","type":"page","link":"https:\/\/dev.ircpss.com\/fr\/quest-ce-quun-shunt-porto-systemique-congenital\/","title":{"rendered":"Qu’est-ce qu’un shunt porto-syst\u00e9mique cong\u00e9nital ?"},"content":{"rendered":"

[vc_row full_width=\u00a0\u00bbstretch_row\u00a0\u00bb css=\u00a0\u00bb.vc_custom_1658395068807{background-position: center !important;background-repeat: no-repeat !important;background-size: cover !important;}\u00a0\u00bb el_class=\u00a0\u00bbheader-bg\u00a0\u00bb][vc_column offset=\u00a0\u00bbvc_hidden-xs\u00a0\u00bb][vc_empty_space height=\u00a0\u00bb100px\u00a0\u00bb][\/vc_column][\/vc_row][vc_row full_width=\u00a0\u00bbstretch_row\u00a0\u00bb css=\u00a0\u00bb.vc_custom_1658395180859{background-position: center !important;background-repeat: no-repeat !important;background-size: cover !important;}\u00a0\u00bb el_class=\u00a0\u00bbheader-bg\u00a0\u00bb][vc_column width=\u00a0\u00bb1\/12″][\/vc_column][vc_column width=\u00a0\u00bb10\/12″][vc_column_text]<\/p>\n

Qu’est-ce qu’un shunt porto-syst\u00e9mique cong\u00e9nital
\n ?<\/span><\/h1>\n

[\/vc_column_text][vc_column_text]<\/p>\n

Un shunt porto-syst\u00e9mique cong\u00e9nital (CPSS)<\/strong> est une malformation vasculaire rare<\/strong> o\u00f9 le sang passe directement des intestins au c\u0153ur sans traverser le foie pour \u00eatre filtr\u00e9. Aujourd’hui, nous savons que si le sang n’est pas filtr\u00e9 par le foie, cela peut entra\u00eener des complications \u00e0 tout \u00e2ge.<\/strong> Il est donc recommand\u00e9 que la plupart des CPSS soient ferm\u00e9s chirurgicalement ou par de petits dispositifs plac\u00e9s dans le vaisseau anormal. <\/p>\n

[\/vc_column_text][\/vc_column][vc_column width=\u00a0\u00bb1\/12″][\/vc_column][\/vc_row][vc_row][vc_column][vc_column_text]<\/p>\n

Types anatomiques de shunts<\/h2>\n

[\/vc_column_text][\/vc_column][\/vc_row][vc_row disable_element=\u00a0\u00bbyes\u00a0\u00bb][vc_column][vc_tta_accordion title_tag=\u00a0\u00bbh4″ style=\u00a0\u00bbmodern\u00a0\u00bb active_section=\u00a0\u00bb1″ collapsible_all=\u00a0\u00bbtrue\u00a0\u00bb][vc_tta_section title=\u00a0\u00bbNormal\u00a0\u00bb tab_id=\u00a0\u00bb1657715222977-0fd07aac-7cc3″][vc_row_inner content_placement=\u00a0\u00bbmiddle\u00a0\u00bb][vc_column_inner width=\u00a0\u00bb1\/2″][vc_column_text]En bleu clair,<\/strong> la veine m\u00e9sent\u00e9rique drainant l’intestin et la veine spl\u00e9nique drainant la rate se rejoignent pour former la veine porte principale qui entre dans le foie et donne les veines portes intrah\u00e9patiques. Cela permet au sang provenant de l’intestin avec les produits de la digestion d’\u00eatre filtr\u00e9 par le foie avant d’atteindre le c\u0153ur via les veines h\u00e9patiques <\/p>\n

En violet,<\/strong> la veine cave inf\u00e9rieure draine le sang de la partie inf\u00e9rieure du corps et re\u00e7oit les deux veines r\u00e9nales et la veine h\u00e9patique juste avant de rejoindre le c\u0153ur dans l’oreillette droite.<\/p>\n

Apr\u00e8s la naissance, il n’y a pas de communication directe entre les veines portes et syst\u00e9miques.[\/vc_column_text][\/vc_column_inner][vc_column_inner width=\u00a0\u00bb1\/2″][vc_single_image image=\u00a0\u00bb221″ img_size=\u00a0\u00bbfull\u00a0\u00bb add_caption=\u00a0\u00bbyes\u00a0\u00bb alignment=\u00a0\u00bbcenter\u00a0\u00bb onclick=\u00a0\u00bblink_image\u00a0\u00bb el_class=\u00a0\u00bbmin-svg\u00a0\u00bb][\/vc_column_inner][\/vc_row_inner][\/vc_tta_section][vc_tta_section title=\u00a0\u00bbIntrah\u00e9patique\u00a0\u00bb tab_id=\u00a0\u00bb1657715222991-eac4db95-2363″][vc_row_inner gap=\u00a0\u00bb10″][vc_column_inner width=\u00a0\u00bb1\/4″][vc_column_text]Deux types anatomiques de CPSS sont actuellement class\u00e9s comme intrah\u00e9patiques, les shunts porto-h\u00e9patiques<\/strong> et le canal veineux persistant.<\/strong><\/p>\n

Les shunts porto-h\u00e9patiques<\/strong> consistent en des communications directes entre une ou plusieurs branches portes et une ou plusieurs veines h\u00e9patiques.<\/p>\n

Le canal veineux persistant<\/strong> est la perm\u00e9abilit\u00e9 anormale du canal veineux qui est une connexion physiologique entre la veine porte intrah\u00e9patique gauche et la veine h\u00e9patique pendant la grossesse et qui se ferme normalement spontan\u00e9ment apr\u00e8s la naissance. En cas de malformation, le canal veineux reste perm\u00e9able.[\/vc_column_text][\/vc_column_inner][vc_column_inner width=\u00a0\u00bb1\/4″][vc_single_image image=\u00a0\u00bb221″ img_size=\u00a0\u00bbfull\u00a0\u00bb add_caption=\u00a0\u00bbyes\u00a0\u00bb alignment=\u00a0\u00bbcenter\u00a0\u00bb onclick=\u00a0\u00bblink_image\u00a0\u00bb el_class=\u00a0\u00bbmin-svg\u00a0\u00bb][\/vc_column_inner][vc_column_inner width=\u00a0\u00bb1\/4″][vc_single_image image=\u00a0\u00bb222″ img_size=\u00a0\u00bbfull\u00a0\u00bb add_caption=\u00a0\u00bbyes\u00a0\u00bb alignment=\u00a0\u00bbcenter\u00a0\u00bb onclick=\u00a0\u00bblink_image\u00a0\u00bb el_class=\u00a0\u00bbmin-svg\u00a0\u00bb][\/vc_column_inner][vc_column_inner width=\u00a0\u00bb1\/4″][vc_single_image image=\u00a0\u00bb223″ img_size=\u00a0\u00bbfull\u00a0\u00bb add_caption=\u00a0\u00bbyes\u00a0\u00bb alignment=\u00a0\u00bbcenter\u00a0\u00bb onclick=\u00a0\u00bblink_image\u00a0\u00bb el_class=\u00a0\u00bbmin-svg\u00a0\u00bb][\/vc_column_inner][\/vc_row_inner][\/vc_tta_section][vc_tta_section title=\u00a0\u00bbExtrah\u00e9patique\u00a0\u00bb tab_id=\u00a0\u00bb1657716760594-f43a93c9-1a89″][vc_row_inner][vc_column_inner width=\u00a0\u00bb1\/3″][vc_column_text]Les CPSS extrah\u00e9patiques<\/strong> sont des connexions entre les veines portes et les veines syst\u00e9miques qui peuvent se produire entre la veine porte principale et la VCI ou en amont de la veine porte depuis n’importe quelle veine aff\u00e9rente \u00e0 la veine porte principale et se drainant dans n’importe quelle veine aff\u00e9rente \u00e0 la VCI, la VCI elle-m\u00eame ou le c\u0153ur lui-m\u00eame.<\/p>\n

Comme le flux sanguin se draine principalement par le CPSS, cette malformation peut provoquer la disparition compl\u00e8te de la veine porte principale et des veines portes intrah\u00e9patiques et est historiquement appel\u00e9e malformation d’Abernethy de type 1 ou absence cong\u00e9nitale de la veine porte.<\/p>\n

Cette connexion peut provoquer une hypoplasie de la veine porte principale et de ses branches, historiquement appel\u00e9e malformation d’Abernethy de type 2.[\/vc_column_text][\/vc_column_inner][vc_column_inner width=\u00a0\u00bb1\/3″][vc_single_image image=\u00a0\u00bb221″ img_size=\u00a0\u00bbfull\u00a0\u00bb add_caption=\u00a0\u00bbyes\u00a0\u00bb alignment=\u00a0\u00bbcenter\u00a0\u00bb onclick=\u00a0\u00bblink_image\u00a0\u00bb el_class=\u00a0\u00bbmin-svg\u00a0\u00bb][vc_single_image image=\u00a0\u00bb224″ img_size=\u00a0\u00bbfull\u00a0\u00bb add_caption=\u00a0\u00bbyes\u00a0\u00bb alignment=\u00a0\u00bbcenter\u00a0\u00bb onclick=\u00a0\u00bblink_image\u00a0\u00bb el_class=\u00a0\u00bbmin-svg\u00a0\u00bb][\/vc_column_inner][vc_column_inner width=\u00a0\u00bb1\/3″][vc_single_image image=\u00a0\u00bb225″ img_size=\u00a0\u00bbfull\u00a0\u00bb add_caption=\u00a0\u00bbyes\u00a0\u00bb alignment=\u00a0\u00bbcenter\u00a0\u00bb onclick=\u00a0\u00bblink_image\u00a0\u00bb el_class=\u00a0\u00bbmin-svg\u00a0\u00bb][vc_single_image image=\u00a0\u00bb226″ img_size=\u00a0\u00bbfull\u00a0\u00bb add_caption=\u00a0\u00bbyes\u00a0\u00bb alignment=\u00a0\u00bbcenter\u00a0\u00bb onclick=\u00a0\u00bblink_image\u00a0\u00bb el_class=\u00a0\u00bbmin-svg\u00a0\u00bb][\/vc_column_inner][\/vc_row_inner][\/vc_tta_section][\/vc_tta_accordion][\/vc_column][\/vc_row][vc_row][vc_column][vc_column_text]

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1<\/div>
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Normal<\/div><\/div> <\/div>\r\n <\/div>\r\n <\/div>
[vc_row][vc_column][vc_row_inner content_placement=\"middle\"][vc_column_inner width=\"1\/2\"][vc_column_text]In light blue, the mesenteric vein draining the bowel and the splenic vein draining the spleen join to form the main portal vein that enters the liver and gives the intrahepatic portal veins. This allows the blood coming from the bowel with products of digestion to be filtered through the liver before getting to the heart via the hepatic veins\r\n\r\nIn purple, the inferior vena cava drains the blood from the inferior part of the body and receives the two renal veins and the hepatic vein just before joining the heart in the right atrium.\r\n\r\nAfter birth, there is no direct communication between the portal and systemic veins.[\/vc_column_text][\/vc_column_inner][vc_column_inner width=\"1\/2\"][vc_single_image image=\"221\" img_size=\"full\" add_caption=\"yes\" alignment=\"center\" onclick=\"link_image\" el_class=\"min-svg\"][\/vc_column_inner][\/vc_row_inner][\/vc_column][\/vc_row] <\/div>\r\n <\/div>\r\n\r\n <\/div>\r\n <\/div>
2<\/div>
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Intrahepatic<\/div><\/div> <\/div>\r\n <\/div>\r\n <\/div>
[vc_row][vc_column][vc_row_inner gap=\"10\"][vc_column_inner width=\"1\/4\"][vc_column_text]Two anatomical types of CPSS are currently classified as intrahepatic, the portohepatic shunts<\/strong> and the patent ductus venosus.<\/strong>\r\n\r\nPorto-hepatic shunts<\/strong> consist of direct communications between one or several portal branches and one or several hepatic veins.\r\n\r\nPatent Ductus Venosus<\/strong> is the abnormal patency of the ductus venosus that is a physiological connection between the intrahepatic left portal vein and hepatic vein during pregnancy and that normally closes spontaneously after birth. In case of malformation, the Ductus venosus remains patent.[\/vc_column_text][\/vc_column_inner][vc_column_inner width=\"1\/4\"][vc_single_image image=\"221\" img_size=\"full\" add_caption=\"yes\" alignment=\"center\" onclick=\"link_image\" el_class=\"min-svg\"][\/vc_column_inner][vc_column_inner width=\"1\/4\"][vc_single_image image=\"222\" img_size=\"full\" add_caption=\"yes\" alignment=\"center\" onclick=\"link_image\" el_class=\"min-svg\"][\/vc_column_inner][vc_column_inner width=\"1\/4\"][vc_single_image image=\"223\" img_size=\"full\" add_caption=\"yes\" alignment=\"center\" onclick=\"link_image\" el_class=\"min-svg\"][\/vc_column_inner][\/vc_row_inner][\/vc_column][\/vc_row] <\/div>\r\n <\/div>\r\n\r\n <\/div>\r\n <\/div>
3<\/div>
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Extrahepatic<\/div><\/div> <\/div>\r\n <\/div>\r\n <\/div>
[vc_row][vc_column][vc_row_inner][vc_column_inner width=\"1\/3\"][vc_column_text]Extrahepatic CPSS<\/strong> are connections between portal veins and systemic veins that can happen between the main portal vein and the IVC or upstream the portal vein from any vein afferent to the main portal vein and draining in any vein afferent to the IVC, the IVC itself of the heart itself.\r\n\r\nBecause the blood flow drains mainly through the CPSS, this malformation can cause the complete disappearance of the main portal vein and intrahepatic portal veins and is historically called Abernethy 1 malformation or congenital absence of portal vein.\r\n\r\nThis connection can cause hypoplasia of the main portal vein and its branches, historically called Abernethy 2 malformation.[\/vc_column_text][\/vc_column_inner][vc_column_inner width=\"1\/3\"][vc_single_image image=\"221\" img_size=\"full\" add_caption=\"yes\" alignment=\"center\" onclick=\"link_image\" el_class=\"min-svg\"][vc_single_image image=\"224\" img_size=\"full\" add_caption=\"yes\" alignment=\"center\" onclick=\"link_image\" el_class=\"min-svg\"][\/vc_column_inner][vc_column_inner width=\"1\/3\"][vc_single_image image=\"225\" img_size=\"full\" add_caption=\"yes\" alignment=\"center\" onclick=\"link_image\" el_class=\"min-svg\"][vc_single_image image=\"226\" img_size=\"full\" add_caption=\"yes\" alignment=\"center\" onclick=\"link_image\" el_class=\"min-svg\"][\/vc_column_inner][\/vc_row_inner][\/vc_column][\/vc_row] <\/div>\r\n <\/div>\r\n\r\n <\/div>\r\n <\/div><\/div> <\/div>\r\n <\/div>\r\n[\/vc_column_text][\/vc_column][\/vc_row][vc_row][vc_column offset=\u00a0\u00bbvc_hidden-xs\u00a0\u00bb][vc_empty_space height=\u00a0\u00bb20px\u00a0\u00bb][\/vc_column][\/vc_row][vc_row][vc_column][vc_btn title=\u00a0\u00bbHow are CPSS diagnosed?\u00a0\u00bb style=\u00a0\u00bbcustom\u00a0\u00bb custom_background=\u00a0\u00bb#4767e7″ custom_text=\u00a0\u00bb#ffffff\u00a0\u00bb size=\u00a0\u00bblg\u00a0\u00bb align=\u00a0\u00bbcenter\u00a0\u00bb link=\u00a0\u00bburl:%2Fnatural-history-and-diagnosis%2F|title:How%20are%20CPSS%20diagnosed%3F|\u00a0\u00bb][\/vc_column][\/vc_row]<\/p>\n<\/div>","protected":false},"excerpt":{"rendered":"

[vc_row full_width=\u00a0\u00bbstretch_row\u00a0\u00bb css=\u00a0\u00bb.vc_custom_1658395068807{background-position: center !important;background-repeat: no-repeat !important;background-size: cover !important;}\u00a0\u00bb el_class=\u00a0\u00bbheader-bg\u00a0\u00bb][vc_column offset=\u00a0\u00bbvc_hidden-xs\u00a0\u00bb][vc_empty_space height=\u00a0\u00bb100px\u00a0\u00bb][\/vc_column][\/vc_row][vc_row full_width=\u00a0\u00bbstretch_row\u00a0\u00bb css=\u00a0\u00bb.vc_custom_1658395180859{background-position: center !important;background-repeat: no-repeat !important;background-size: cover !important;}\u00a0\u00bb el_class=\u00a0\u00bbheader-bg\u00a0\u00bb][vc_column width=\u00a0\u00bb1\/12″][\/vc_column][vc_column width=\u00a0\u00bb10\/12″][vc_column_text] Qu’est-ce qu’un shunt [\u2026]<\/span><\/p>\n","protected":false},"author":1,"featured_media":0,"parent":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"_acf_changed":false,"footnotes":""},"class_list":["post-2891","page","type-page","status-publish","hentry"],"acf":[],"yoast_head":"\nQu'est-ce qu'un shunt porto-syst\u00e9mique cong\u00e9nital ? - IRCPSS<\/title>\n<meta name=\"description\" content=\"Un CPSS est une malformation vasculaire rare o\u00f9 le sang passe directement des intestins au c\u0153ur sans traverser le foie pour \u00eatre filtr\u00e9.\" \/>\n<meta name=\"robots\" content=\"noindex, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<meta property=\"og:locale\" content=\"fr_FR\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Qu'est-ce qu'un shunt porto-syst\u00e9mique cong\u00e9nital ? - IRCPSS\" \/>\n<meta property=\"og:description\" content=\"Un CPSS est une malformation vasculaire rare o\u00f9 le sang passe directement des intestins au c\u0153ur sans traverser le foie pour \u00eatre filtr\u00e9.\" \/>\n<meta property=\"og:url\" content=\"https:\/\/dev.ircpss.com\/fr\/quest-ce-quun-shunt-porto-systemique-congenital\/\" \/>\n<meta property=\"og:site_name\" content=\"IRCPSS\" \/>\n<meta property=\"article:modified_time\" content=\"2025-11-18T08:53:44+00:00\" \/>\n<meta name=\"twitter:card\" content=\"summary_large_image\" \/>\n<meta name=\"twitter:label1\" content=\"Dur\u00e9e de lecture estim\u00e9e\" \/>\n\t<meta name=\"twitter:data1\" content=\"5 minutes\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\\\/\\\/schema.org\",\"@graph\":[{\"@type\":\"WebPage\",\"@id\":\"https:\\\/\\\/dev.ircpss.com\\\/fr\\\/quest-ce-quun-shunt-porto-systemique-congenital\\\/\",\"url\":\"https:\\\/\\\/dev.ircpss.com\\\/fr\\\/quest-ce-quun-shunt-porto-systemique-congenital\\\/\",\"name\":\"Qu'est-ce qu'un shunt porto-syst\u00e9mique cong\u00e9nital ? 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