Total Anomalous Pulmonary Venous Return (TAPVR) | Diagnosis & Treatment

How is total anomalous pulmonary venous return diagnosed?

In some cases, total anomalous pulmonary venous return is found before birth on a fetal echocardiogram.

Most babies with total anomalous pulmonary venous return (TAPVR) have symptoms on the day they’re born. Severe TAPVR can usually be diagnosed promptly based on symptoms and tests, including chest x-ray and cardiac ultrasound. These babies are generally admitted immediately to the hospital.

Babies with less severe TAPVR may have symptoms in the first few days of life. If your newborn baby is born with a bluish tint to the skin or is having difficulty breathing, you may be referred to a pediatric cardiologist to determine a diagnosis.

Your baby’s doctor may order one or more additional tests to diagnose or confirm a diagnosis of TAPVR:

What are the treatment options for total anomalous pulmonary venous return?

Newborns with severe TAPVR will need emergency surgery shortly after birth. They often need to be admitted to the cardiac intensive care unit (CICU) and require intensive support with medications and a ventilator (breathing machine).

Some babies with severe TAPVR may need a specialized life support system called ECMO (extracorporeal membrane oxygenation), an advanced technology that functions as a replacement for a critically ill child's heart and lungs.

Babies with less severe TAPVR usually have surgery in the days or weeks after they're diagnosed.

The goal of surgery for TAPVR is to restore normal connections of the pulmonary veins to the heart, alleviate any obstructions or narrowing of the pulmonary veins, to tie up any vessels that have developed and to close the atrial septal defect (ASD).

What is the long-term outlook for children with TAPVR?

Thanks to updates in surgical techniques for repairing TAPVR, the long-term outlook is continually improving.

Children who have had a repair for TAPVR will require lifelong checkups with a cardiologist to make sure their veins remain open. If the veins become narrowed, they may need catheterizations or surgeries to repair these veins.