Total anomalous pulmonary venous connection overview

Total anomalous pulmonary venous connection Microchapters
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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]; Keri Shafer, M.D. [3] Priyamvada Singh, MBBS [[4]] Assistant Editor-In-Chief: Kristin Feeney, B.S. [[5]]

Overview

Total anomalous pulmonary venous connection (TAPVC) 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.

Pathophysiology

In patients of Total anomalous pulmonary venous connection (TAPVC) there is a mixing of oxygenated pulmonary venous blood with deoxygenated blood from systemic circulation.

Causes

Like many other congenital heart defects, the exact cause of total anomalous pulmonary venous connection is not known. Few factors like genetics, maternal alcohol syndrome and some drugs have been found to occur with increased frequency.

Differential Diagnosis

Total anomalous pulmonary venous connection should be differentiated from other cyanotic congenital heart diseases found in the pediatrics population

Epidemiology and Demographics

Total anomalous pulmonary venous connection is a rare cyanotic congenital heart disease and occurs in 6 to 12 per 100,000 live births.

Risk Factors

There are certain factors that increase the risk of congenital heart diseases.

Screening

Echocardiography can act as a tool to make early diagnosis of total anomalous pulmonary venous connection.