Total anomalous pulmonary venous connection

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**Total anomalous pulmonary venous connection**
*Classification & external resources*
ICD-10	Q 26.2
ICD-9	747.41

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 shunt must be present in order to obtain systemic blood flow.

1 Variations
2 Clinical manifestation
3 Treatment
4 External links

[edit] Variations

There are four variants:

Supracardiac (50%): blood drains to innominate vein 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.

[edit] 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

[edit] 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.