Total anomalous pulmonary venous connection
- Total anomalous pulmonary venous connection
Infobox_Disease
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ICD10 = ICD10|Q|26|2|q|20
ICD9 = ICD9|747.41
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Total anomalous pulmonary venous connection (TAPVC), also known as total anomalous pulmonary venous drainage(TAPVD) and total anomalous pulmonary venous return(TAPVR), is a rare cyanotic congenital heart defect (CHD) in which all four pulmonary veins are ed and make connections to the systemic venous circulation.(Normally, pulmonary venous return carries oxygenated blood to the left atrium and to the rest of the body). A patent foramen ovale or an atrial septal defect "must" be present, or else the condition is fatal due to a lack of systemic blood flow.
Variations
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 or 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.
Clinical manifestation
* right ventricular heave
* fixed split S2
* S3 gallop
* systolic ejection murmur at left upper sternal border
* cardiomegaly
* right axis deviation on ECG
* right ventricular hypertrophy
* cyanosis, tachypnea, dyspnea since the overloaded pulmonary circuit can cause pulmonary edema
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.
External links
* [http://heartcenter.seattlechildrens.org/conditions_treated/total_anomalous.asp Total Anomalous Pulmonary Venous Return information] from Seattle Children's Hospital Heart Center
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2010.
Look at other dictionaries:
connection — A union of elements or things; a connecting structure. SYN: conexus, connexus. ambiguous atrioventricular connections connections in which half the atrioventricular junction is connected concordantly and the other half is discordantly connected.… … Medical dictionary
TAPVC — • total anomalous pulmonary venous connection … Dictionary of medical acronyms & abbreviations
Congenital heart defect — Classification and external resources The normal structure of the heart (left) in comparison to two common locations for a ventricular septal defect (right), the most common form of congenital heart defect.[1] … Wikipedia
TAPVC — Abbreviation for total anomalous pulmonary venous connection. See anomalous pulmonary venous connections, total or partial, under connection. * * * total anomalous pulmonary venous connection … Medical dictionary
Lungenvenenfehlmündung — Klassifikation nach ICD 10 Q26.2 Totale Fehleinmündung der Lungenvenen Q26.3 Partielle Fehleinmündung der Lungenvenen … Deutsch Wikipedia
Cyanotic heart defect — A cyanotic heart defect is a group type of congenital heart defects (CHDs). The patient appears blue (cyanotic), due to deoxygenated blood bypassing the lungs and entering the systemic circulation. This can be caused by right to left or… … Wikipedia
return — total anomalous pulmonary venous r. (TAPVR) See anomalous pulmonary venous connections, total or partial, under connection. venous r. the blood returning to the heart via the great veins and coronary sinus. * * * re·turn (re turnґ) a coming b … Medical dictionary
syndrome — The aggregate of symptoms and signs associated with any morbid process, and constituting together the picture of the disease. SEE ALSO: disease. [G. s., a running together, tumultuous concourse; (in med.) a concurrence of symptoms, fr. syn,… … Medical dictionary
TAPVR — Abbreviation for total anomalous pulmonary venous return. See anomalous pulmonary venous connections, total or partial, under connection. * * * total anomalous pulmonary venous return … Medical dictionary
Patent ductus arteriosus — Classification and external resources Heart cross section with PDA ICD 10 Q … Wikipedia
