Atrial Septal Defect With Partial Anomalous Pulmonary Venous Return

An atrial septal defect (ASD) and partial anomalous pulmonary venous return (PAPVR) are two congenital heart defects that commonly occur together.

About Atrial Septal Defect With Partial Anomalous Pulmonary Venous Return

In a normal heart, the right and left atria (collecting chambers of the heart) are separated by a thin wall (atrial septum) that separates oxygen-rich blood traveling to the body from oxygen-depleted blood returning to the lungs. An atrial septal defect (ASD) occurs when a hole in the atrial septum allows the oxygen-rich and oxygen-poor blood to mix and affects how the heart and lungs develop.

The pulmonary veins are the blood vessels that bring the oxygen-rich blood from the lungs to the left atrium of the heart where the blood is drained from the veins and collected in the chamber to be pumped out to the rest of the body. Partial anomalous pulmonary venous return (PAPVR) occurs when one or more of the veins drain to an abnormal location on the right side of the heart.

Both ASD and PAPVR allow excess blood flow to the lungs and the right side of the heart, which, over time, can cause the right side of the heart to become enlarged. Excess blood flow to the lungs can also cause respiratory issues, such as rapid breathing and frequent respiratory illnesses.

Symptoms of Atrial Septal Defect With Partial Anomalous Pulmonary Venous Return

Most children with ASD with PAPVR do not develop symptoms for many years.

Symptoms of ASD with PAPVR may include:

• Frequent upper respiratory tract infections
• Irregular heartbeats or palpitations
• Tiring during exercise (for babies, this includes during feeding)
• Poor weight gain

Diagnosing Atrial Septal Defect With Partial 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, 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 or has poor exercise tolerance (for babies, this includes poor feeding).

Tests performed when diagnosing ASD with PAPVR 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.
• 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.

Treating Atrial Septal Defect With Partial Anomalous Pulmonary Venous Return

Treatment of ASD with PAPVR requires open-heart surgery to close the hole inside the heart and to reroute the anomalous pulmonary vein connection over to the left atrium. The timing of surgery is usually elective and based upon symptoms, the degree of right heart enlargement, and where the abnormal pulmonary vein connection is located.

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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