Total anomalous pulmonary venous connection

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Total anomalous pulmonary venous connection Classification and external resources
Common pulmonary vein
ICD-10	Q26.2
ICD-9	747.41

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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 congenital heart defect (CHD) in which all four pulmonary veins are malpositioned and make anomalous 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 in order to allow 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.

Diagnosis

Symptoms

• 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

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

• 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

v • d • e Congenital malformations and deformations of circulatory system (Q20-Q28, 745-747)
Cardiac chambers and connections	Persistent truncus arteriosus - Double outlet right ventricle (Taussig-Bing syndrome) - Transposition of the great vessels (dextro, levo)
Cardiac septa	Ventricular septal defect - Atrial septal defect (Lutembacher's syndrome) - Atrioventricular septal defect (Ostium primum) - Tetralogy of Fallot - Eisenmenger's syndrome
Right: pulmonary and tricuspid valves	pulmonary valves (stenosis, insufficiency) - tricuspid valves (stenosis, atresia) - Ebstein's anomaly
Left: aortic and mitral valves	aortic valves (stenosis, insufficiency, bicuspid) - mitral valves (stenosis, regurgitation) - Hypoplastic left heart syndrome
Other congenital malformations of heart	Dextrocardia - Levocardia - Cor triatriatum
Great arteries	aorta (Patent ductus arteriosus, Aortic coarctation, Interrupted aortic arch, Overriding aorta, Aneurysm of sinus of Valsalva, Vascular ring) - Pulmonary atresia
Great veins	Persistent left superior vena cava - Total anomalous pulmonary venous connection - Scimitar syndrome
Other	Arteriovenous malformation (Cerebral arteriovenous malformation)
See also non-congenital conditions (I, 390-459)

v • d • e WikiDoc Research Resources for Total anomalous pulmonary venous connection
Articles on Total anomalous pulmonary venous connection	Most recent articles on Total anomalous pulmonary venous connection • Most cited articles on Total anomalous pulmonary venous connection • Review articles on Total anomalous pulmonary venous connection • Articles on Total anomalous pulmonary venous connection in N Eng J Med, Lancet, BMJ
Media (Slides, Video, Images, MP3) on Total anomalous pulmonary venous connection	Powerpoint slides on Total anomalous pulmonary venous connection • Images of Total anomalous pulmonary venous connection • Photos of Total anomalous pulmonary venous connection • Podcasts & MP3s on Total anomalous pulmonary venous connection • Videos on Total anomalous pulmonary venous connection
Evidence Based Medicine Regarding Total anomalous pulmonary venous connection	Cochrane Collaboration on Total anomalous pulmonary venous connection • Bandolier on Total anomalous pulmonary venous connection • TRIP on Total anomalous pulmonary venous connection
Cost Effectiveness of Total anomalous pulmonary venous connection	Cost Effectiveness of Total anomalous pulmonary venous connection
Clinical Trials Involving Total anomalous pulmonary venous connection	Ongoing Trials on Total anomalous pulmonary venous connection at Clinical Trials.gov • Trial results on Total anomalous pulmonary venous connection • Clinical Trials on Total anomalous pulmonary venous connection at Google
Guidelines / Policies / Government Resources (FDA/CDC) Regarding Total anomalous pulmonary venous connection	US National Guidelines Clearinghouse on Total anomalous pulmonary venous connection • NICE Guidance on Total anomalous pulmonary venous connection • NHS PRODIGY Guidance • FDA on Total anomalous pulmonary venous connection • CDC on Total anomalous pulmonary venous connection
Textbook Information on Total anomalous pulmonary venous connection	Books and Textbook Information on Total anomalous pulmonary venous connection
Pharmacology Resources on Total anomalous pulmonary venous connection	Dosing of Total anomalous pulmonary venous connection • Drug interactions with Total anomalous pulmonary venous connection • Side effects of Total anomalous pulmonary venous connection • Allergic reactions to Total anomalous pulmonary venous connection • Overdose information on Total anomalous pulmonary venous connection • Carcinogenicity information on Total anomalous pulmonary venous connection • Total anomalous pulmonary venous connection in pregnancy • Pharmacokinetics of Total anomalous pulmonary venous connection •
Genetics, Pharmacogenomics, and Proteinomics of Total anomalous pulmonary venous connection	Genetics of Total anomalous pulmonary venous connection • Pharmacogenomics of Total anomalous pulmonary venous connection • Proteomics of Total anomalous pulmonary venous connection
Newstories on Total anomalous pulmonary venous connection	Total anomalous pulmonary venous connection in the news • Be alerted to news on Total anomalous pulmonary venous connection • News trends on Total anomalous pulmonary venous connection
Commentary on Total anomalous pulmonary venous connection	Blogs on Total anomalous pulmonary venous connection
Patient Resources on Total anomalous pulmonary venous connection	Patient resources on Total anomalous pulmonary venous connection • Discussion groups on Total anomalous pulmonary venous connection • Patient Handouts on Total anomalous pulmonary venous connection • Directions to Hospitals Treating Total anomalous pulmonary venous connection • Risk calculators and risk factors for Total anomalous pulmonary venous connection
Healthcare Provider Resources on Total anomalous pulmonary venous connection	Symptoms of Total anomalous pulmonary venous connection • Causes & Risk Factors for Total anomalous pulmonary venous connection • Diagnostic studies for Total anomalous pulmonary venous connection • Treatment of Total anomalous pulmonary venous connection
Continuing Medical Education (CME) Programs on Total anomalous pulmonary venous connection	CME Programs on Total anomalous pulmonary venous connection
International Resources on Total anomalous pulmonary venous connection	Total anomalous pulmonary venous connection en Espanol • Total anomalous pulmonary venous connection en Francais
Business Resources on Total anomalous pulmonary venous connection	Total anomalous pulmonary venous connection in the Marketplace • Patents on Total anomalous pulmonary venous connection
Informatics Resources on Total anomalous pulmonary venous connection	List of terms related to Total anomalous pulmonary venous connection

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