Total anomalous pulmonary venous connection: Difference between revisions

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{{Infobox_Disease |
{{Infobox_Disease |
   Name          = {{PAGENAME}} |
   Name          = {{PAGENAME}} |
  Image          = Common pulmonary vein.jpg|
  Caption        = Common pulmonary vein|
   DiseasesDB    = |
   DiseasesDB    = |
   ICD10          = {{ICD10|Q|26|2|q|20}} |
   ICD10          = {{ICD10|Q|26|2|q|20}} |
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'''Associate Editors-In-Chief:''' {{CZ}}; [[User:KeriShafer|Keri Shafer, M.D.]] [mailto:kshafer@bidmc.harvard.edu]
'''Associate Editors-In-Chief:''' {{CZ}}; [[User:KeriShafer|Keri Shafer, M.D.]] [mailto:kshafer@bidmc.harvard.edu]


==Overview==
==[[Total anomalous pulmonary venous connection overview|Overview]]==
'''Total anomalous pulmonary venous connection''' ('''TAPVC'''), also known as '''total anomalous pulmonary venous drainage'''('''TAPVD''') and '''total anamalous pulmonary venous return'''('''TAPVR'''), is a rare [[cyanotic heart defect|cyanotic]] [[congenital]] [[congenital heart defect|heart defect]] ('''CHD''') in which all four [[pulmonary vein]]s are [[wiktionary:malposition|malposition]]ed and make [[wiktionary:anomoly|anomalous]] connections to the [[systemic vein|systemic venous]] [[circulatory system|circulation]]. (Normally, pulmonary venous return carries oxygenated blood to the left atrium and to the rest of the body). A patent [[foramen ovale (heart)|foramen ovale]] or an [[atrial septal defect]] ''must'' be present in order to allow systemic blood flow.


== Variations ==
==[[Total anomalous pulmonary venous connection classifications|Classifications]]==
There are four variants:
* ''Supracardiac'' (50%): blood drains to one of the [[innominate veins]] (brachiocephalic veins) or the [[superior vena cava]]
* ''Cardiac'' (20%): blood drains into [[coronary sinus]] or directly into right atrium
* ''Infradiaphragmatic'' (20%): blood drains into [[portal vein|portal]] or [[hepatic vein|hepatic veins]]
* ''Mixed'' (10%)
 
TAPVC can occur with ''obstruction'', which occurs when the anomalous vein enters a vessel at an acute angle and can cause pulmonary venous hypertension and cyanosis because blood cannot easily enter the new vein as easily.


==Diagnosis==
==Diagnosis==
===Symptoms===
[[Total anomalous pulmonary venous connection history and symptoms|History & Symptoms] | [[Total anomalous pulmonary venous connection physical examination|Physical Examination]] | [[Total anomalous pulmonary venous connection electrocardogram|Electrocardigram]] | [[Total anomalous pulmonary venous connection other diagnostic findings|Cardiac catheterization]]
* [[cyanosis]], [[tachypnea]], [[dyspnea]] since the overloaded pulmonary circuit can cause [[pulmonary edema]]
 
===Physical Examination===
* right ventricular [[heave]]
* fixed split S2
* [[S3 gallop]]
* systolic ejection [[murmur]] at left upper sternal border
* [[cardiomegaly]]
 
===Electrocardiographic Findings===
* [[right axis deviation]] on ECG
* [[right ventricular hypertrophy]]
 
===Cardiac Catheterization===
 
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==Treatment==
==Treatment==
In TAPVC without obstruction, surgical redirection can be performed within the first month of life. With obstruction, surgery should be undertaken emergently. [[PGE1]] should ''not'' be given because a [[patent ductus arteriosus]] adds even more volume into the already overloaded pulmonary flow.
[[Total anomalous pulmonary venous connection surgery|Surgical Therapy]]


==References==
==References==

Revision as of 19:08, 13 July 2011

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Total anomalous pulmonary venous connection
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ICD-9 747.41

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Associate Editors-In-Chief: Cafer Zorkun, M.D., Ph.D. [2]; Keri Shafer, M.D. [3]

Overview

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[[Total anomalous pulmonary venous connection history and symptoms|History & Symptoms] | Physical Examination | Electrocardigram | Cardiac catheterization

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  • Moss and Adams' Heart Disease in Infants, Children, and Adolescents Hugh D. Allen, Arthur J. Moss, David J. Driscoll, Forrest H. Adams, Timothy F. Feltes, Robert E. Shaddy, 2007 ISBN 0781786843

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