Total anomalous pulmonary venous connection (patient information)

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 * Associate Editor(s)-In-Chief: Jinhui Wu, M.D.; Priyamvada Singh, M.B.B.S. [mailto:psingh@perfuse.org]; Assistant Editor(s)-In-Chief: Kristin Feeney, B.S. [mailto:kfeeney@perfuse.org]

Overview
Total anomalous pulmonary venous connection is a rare congenital heart disease, in which the pulmonary veins that bring oxygen-rich blood from the lungs back to the heart connect to the right atrium instead of the left atrium. This may lead low-oxygen blood flow into the body. Usual symptoms include cyanosis, lethargy, frequent respiratory infections, and poor feeding and grow. Echocardiography and cardiac MRI can tell the diagnosis of total anomalous pulmonary venous connection. The main treatment of total anomalous pulmonary venous connection is early complete surgical repair. Prognosis of total anomalous pulmonary venous connection depends on whether the surgery has been performed or not.

What are the symptoms of total anomalous pulmonary venous connection?
Symptoms vary widely. Sometimes no symptoms may be present in infancy or early childhood. Sometimes signs may display critically like the following:
 * Cyanosis
 * Lethargy
 * Frequent respiratory infections
 * Poor feeding and grow
 * Rapid breathing

Other health problems may also cause these symptoms. Only a doctor can tell for sure. A person with any of these symptoms should tell the doctor so that the problems can be diagnosed and treated as early as possible.

Who is at highest risk?
The cause of total anomalous pulmonary venous connection is not clear. Some patients have other congenital heart disease.

Diagnosis

 * Echocardiography: This kind of painless test can help the doctor closely examine total anomalous pulmonary venous connection. It uses sound waves to produce an image of the ventricles, atrium and great vessels. An echocardiogram can tell the relationship of the great vessels and ventricles or atria. Furthermore, the doctor can measure the speed of blood flow by echocardiogram. The doctor can also find other congenital defect by echocardiogram.
 * Chest x-ray: An x-ray image of chest allows the doctor to check the size and shape of your great vessles and heart.
 * Chest CT or MRI: A chest CT or MRI can demonstrate the details of the heart extremely well, such as the great vessles, vascular, atrial and ventricular structures and their relationships to one another.
 * Cardiac catheterization: This is not a common diagnostic measure for total anomalous pulmonary venous connection. In a catheter room, the doctor threads a thin tube through a blood vessel in the patient's arm or groin to an artery in the heart and injects dye to see great vessels, atria and ventricles on an x-ray. Cardiac catheterization can reveal associated cardiac abnormalities that are known or suspected.

When to seek urgent medical care?
Call your health care provider as soon as possible if your baby has symptoms of total anomalous pulmonary venous connection.

Treatment options
The main treatment for patients with total anomalous pulmonary venous connection is early complete surgery to repair the defects. During surgery, doctors connect the pulmonary veins to the left atrium, close the defect between the right and left atrium, and repair other defects.

Diseases with similar symptoms

 * Atrial septal defect

Where to find medical care for total anomalous pulmonary venous connection?
Directions to Hospitals Treating Total anomalous pulmonary venous connection

What to expect (Outlook/Prognosis)?
Prognosis of total anomalous pulmonary venous depends on whether the surgery has been done with the patient. Without surgery, death may occur by age one in babies with more severe defects.

Prevention
The preventive measure of total anomalous pulmonary venous connection is unknown.