Total Anomalous Pulmonary Venous Return

Total anomalous pulmonary venous return (TAPVR) is a congenital heart defect in which the veins that bring oxygen-rich blood to the heart return to an abnormal location.

About Total Anomalous Pulmonary Venous Return

The heart is divided into a right side that has oxygen-poor blood and a left side that has oxygen-rich blood. The pulmonary veins are the four blood vessels that carry oxygenated blood from the lungs to the left side of the heart. In total anomalous pulmonary venous return (TAPVR), rather than returning to the left atrium (the normal collecting chamber of the heart), the four pulmonary veins drain abnormally. The potential abnormal locations include inside the heart (intracardiac), to veins above the heart (supracardiac and extracardiac), below the diaphragm (infradiaphragmatic), or a combination of locations. Total anomalous pulmonary venous return can occur by itself or in combination with other congenital heart defects.

Symptoms of Total Anomalous Pulmonary Venous Return

Symptoms of TAVPR vary based on where the veins connect and whether there is obstruction to the blood flow through the veins at the abnormal connection. If the veins are obstructed, the child may become very sick very quickly and can require emergency surgery. If the veins are not obstructed, the child may not require surgical intervention for weeks to months after being born.

Symptoms of TAPVR may include:

• Difficulty breathing
• Face or chest appear blue in color
• Fast breathing
• Poor feeding
• Poor growth

Diagnosing Total Anomalous Pulmonary Venous Return

During physical examinations, your child’s doctor listens to your child’s heart and lungs and may detect a heart murmur, which are extra sounds heard throughout the cardiac cycle due to increased blood flow. If your pediatrician suspects increased blood flow in the right atrium, a recommendation to see a pediatric cardiologist may be made. Your doctor may also refer you to a pediatric cardiologist if your child is breathing fast, has poor exercise tolerance (for babies, this includes poor feeding), or has an enlarged liver.

Tests performed when diagnosing TAPVR may include:

• Cardiac MRI or CT Scan: A cardiac MRI or CT scan is used to take more detailed images of the heart to help define the anatomy and detect anomalies.
• Chest X-Ray: A chest X-ray produces an image of the tissue and bones in the heart and lungs and helps your provider assess the shape, size, and structure of the heart and lungs as well as the aeration of or any congestion in the lungs.
• Echocardiogram: An echocardiogram uses ultrasound technology to create a moving image of the heart and its valves, allowing your provider to assess the structure and function of the heart. An echocardiogram also helps provide information about blood flow and how well the heart is pumping blood. It may be difficult to identify all four venous connections on echocardiogram and further imaging with an MRI may be necessary.
• Electrocardiogram (ECG or EKG): An electrocardiogram uses electrodes that are placed on the body to record the electrical activity taking place in the heart. An ECG/EKG test helps detect abnormal rhythms, such as cardiac arrhythmias, stress on the heart, and damage to the heart muscles. An ECG/CKG may also detect abnormally thickened or enlarged chambers of the heart as a result of a subaortic membrane.

Treating Total Anomalous Pulmonary Venous Return

Treatment of a TAPVR requires open-heart surgery. If the pulmonary venous pathway is obstructed, surgery may need to be performed as an emergency surgery shortly after birth. If the pathway is not obstructed, surgery may be able to be performed electively later during infancy.

Care Team Approach

The Texas Center for Pediatric and Congenital Heart Disease, a clinical partnership between Dell Children’s Medical Center and UT Health Austin, takes a multidisciplinary approach to your child’s care. This means your child and your family will benefit from the expertise of multiple specialists across a variety of disciplines. Your care team will include pediatric cardiologists, cardiothoracic surgeons, interventional cardiologists, critical care specialists, hospitalists, anesthesiologists, perfusionists, nurses, advanced practice providers, social workers, psychologists, child life specialists, dietitians, physical and occupational therapists, pharmacists, and more, who work together to provide unparalleled care for patients every step of the way. We collaborate with our colleagues at the Dell Medical School and The University of Texas at Austin to utilize the latest research, diagnostic, and treatment techniques, allowing us to identify new therapies to improve treatment outcomes. We are committed to communicating and coordinating your child’s care with referring physicians and other partners in the community to ensure that we are providing comprehensive, whole-person care.

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