Total anomalous pulmonary venous connection overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Maheep Singh Sangha, M.B.B.S.
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.
Natural History, Complications and Prognosis
The natural history of untreated patients of TAPVC is not very favorable. It depends on the type of obstruction and amount of shunting across the atrium.
Diagnosis
History and Symptoms
The clinical features in total anomalous pulmonary venous connection depends on the type of anatomic variant present in the patient. This in turn determines the amount of mixing between the pulmonary and systemic circulation.
Physical Examination
The physical finding depends on the degree of obstruction and the degree of left-to-right shunting.
Electrocardiogram
Electrocardiogrphy findings are not very specific. However, they can show changes due to dilatation of right side of heart.
Chest X-ray
The findings on chest radiography varies depending on the type of anatomic variant of total anomalous pulmonary venous connection (TAPVC). However, the findings are not useful in making the diagnosis.
Magnetic Resonance Imaging
Magnetic resonance imaging can be helpful as a diagnostic tool in conditions where the echocardiographic findings are inconclusive.
CT
Computed tomography can be helpful as a diagnostic tool in conditions where the echocardiographic findings are inconclusive.
Echocardiography
2D echocardiography along with doppler ultrasonography is an useful tool to diagnose total anomalous pulmonary venous connection.
Other Imaging Findings
In patients with total anomalous pulmonary venous connection, cardiac catheterization can be used as a diagnostic and therapeutic modality. However, with availability of better non-invasive tools the use of these are decreasing.