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{{Infobox_Disease |
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   Image          = Common pulmonary vein.jpg|
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'''For patient information, click [[{{PAGENAME}} (patient information)|here]]'''
{{WikiDoc Cardiology Network Infobox}}
{{CMG}}


'''Associate Editors-In-Chief:''' {{CZ}}; [[User:KeriShafer|Keri Shafer, M.D.]] [mailto:kshafer@bidmc.harvard.edu]
{{Template:Total anomalous pulmonary venous connection}}
{{CMG}}; '''Associate Editor-In-Chief:'''{{CZ}}; [[Priyamvada Singh|Priyamvada Singh, MBBS]] [mailto:psingh13579@gmail.com]; {{Sahar}} '''Assistant Editor-In-Chief:''' [[Kristin Feeney|Kristin Feeney, B.S.]] [mailto:kfeeney@elon.edu]


{{Editor Join}}
'''''Synonyms and Keywords:''''' TAPVR, Anomalous pulmonary venous connection, anomalous pulmonary venous drainage, anomalous pulmonary venous return


==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 classification|Classification]]==
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.
==[[Total anomalous pulmonary venous connection pathophysiology|Pathophysiology]]==


==Diagnosis==
==[[Total anomalous pulmonary venous connection causes|Causes]]==
===Symptoms===
* [[cyanosis]], [[tachypnea]], [[dyspnea]] since the overloaded pulmonary circuit can cause [[pulmonary edema]]


===Physical Examination===
==[[Total anomalous pulmonary venous connection differential diagnosis|Differentiating Total anomalous pulmonary venous connection from other Diseases]]==
* right ventricular [[heave]]
* fixed split S2
* [[S3 gallop]]
* systolic ejection [[murmur]] at left upper sternal border
* [[cardiomegaly]]


===Electrocardiographic Findings===
==[[Total anomalous pulmonary venous connection epidemiology and demographics|Epidemiology and Demographics]]==
* [[right axis deviation]] on ECG
* [[right ventricular hypertrophy]]


===Cardiac Catheterization===
==[[Total anomalous pulmonary venous connection risk factors|Risk Factors]]==


<googlevideo>4462444442806796487&hl=en</googlevideo> 
==[[Total anomalous pulmonary venous connection screening|Screening]]==


==[[Total anomalous pulmonary venous connection natural history, complications and prognosis|Natural History, Complications and Prognosis]]==


<googlevideo>-3314937685295122195&hl=en</googlevideo>
==Diagnosis==
 
[[Total anomalous pulmonary venous connection history and symptoms|History and Symptoms]] | [[Total anomalous pulmonary venous connection physical examination|Physical Examination]] | [[Total anomalous pulmonary venous connection laboratory findings|Laboratory Findings]] | [[Total anomalous pulmonary venous connection electrocardiogram | Electrocardiogram]] | [[Total anomalous pulmonary venous connection chest x ray|Chest X Ray]] | [[Total anomalous pulmonary venous connection MRI|MRI]] | [[Total anomalous pulmonary venous connection CT|CT]] | [[Total anomalous pulmonary venous connection echocardiography|Echocardiography]] | [[Total anomalous pulmonary venous connection other imaging findings|Other Imaging Findings]] | [[Total anomalous pulmonary venous connection other diagnostic studies|Other Diagnostic Studies]]
 
<googlevideo>2240824856713063576&pr=goog-sl</googlevideo> 
 
 
<googlevideo>4846170916216525758&hl=en</googlevideo>
 
 
<googlevideo>2692134944789571414&hl=en</googlevideo> 
 
 
<googlevideo>961264589763540810&hl=en</googlevideo>
 
 
<googlevideo>613495638748988173&hl=en</googlevideo>
 


==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 medical therapy|Medical Therapy]] | [[Total anomalous pulmonary venous connection surgery|Surgery]] | [[Total anomalous pulmonary venous connection primary prevention|Prevention]]
 
==External links==
* [http://heartcenter.seattlechildrens.org/conditions_treated/total_anomalous.asp Total Anomalous Pulmonary Venous Return information] from Seattle Children's Hospital Heart Center
 
==Additional Reading==
 
* 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
 
{{Congenital malformations and deformations of circulatory system}}
{{SIB}}
[[Category:cardiovascular system]]
[[Category:cardiology]]
[[Category:congenital heart disease]]
[[Category:pediatrics]]


[[pl:Całkowite nieprawidłowe przyłączenie żył płucnych]]
==Case Studies==
[[Total anomalous pulmonary venous connection case study one|Case #1]]
==References==
{{reflist|2}}
[[Category:Cardiovascular system]]
[[Category:Cardiology]]
[[Category:Congenital heart disease]]
[[Category:Pediatrics]]


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Latest revision as of 16:06, 16 April 2020

Template:DiseaseDisorder infobox For patient information, click here


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Physical Examination

Laboratory Findings

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief:Cafer Zorkun, M.D., Ph.D. [2]; Priyamvada Singh, MBBS [3]; Sahar Memar Montazerin, M.D.[4] Assistant Editor-In-Chief: Kristin Feeney, B.S. [5]

Synonyms and Keywords: TAPVR, Anomalous pulmonary venous connection, anomalous pulmonary venous drainage, anomalous pulmonary venous return

Overview

Classification

Pathophysiology

Causes

Differentiating Total anomalous pulmonary venous connection from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | Chest X Ray | MRI | CT | Echocardiography | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Prevention

Case Studies

Case #1

References

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