What is total anomalous pulmonary venous return?
Total anomalous pulmonary venous return (TAPVR) is a birth defect in which the lung veins do not attach to the left atrium. Instead, they attach to the right atrium or to the blood vessels leading into the right atrium.
Normally, four veins come from the lungs (two from the left lung and two from the right lung). They carry oxygen-rich (red) blood into the right atrium. Then the blood flows into the left ventricle, which pumps it out to the body.
After delivering oxygen around the body, the blood returns to the heart and goes into the right atrium. Then it flows into the right ventricle, which pumps it to the lungs to pick up oxygen.
In TAPVR, the four lung veins usually join to form one common vein. Instead of connecting to the left atrium, the common vein connects to the right atrium or to the vessels that bring oxygen-poor (blue) blood from the body into the right atrium. Sometimes, the four lung veins may take different routes to connect to the right atrium or to the vessels that bring oxygen-poor blood from the body into the right atrium. This is called mixed TAPVR. In either case, most of the blood flows only between the right side of the heart and the lungs, not out to the rest of the body.
Babies with TAPVR get some blood to their body because they have a hole in the septum between the upper chambers of the heart, called an atrial septal defect. When oxygen-poor blood from the body and oxygen-rich blood from the lungs mix in the right atrium, some of this blood flows through the hole into the left atrium. Then the blood flows into the left ventricle, which pumps it out to the body.
Total Anomalous Pulmonary Venous Return in Children
With this condition, the baby’s body does not get as much oxygen as it should.
Their right ventricle and pulmonary artery (the artery that carries blood from the right ventricle to the lungs) will both become larger than normal because they are handling too much blood.
In some babies, the lung veins are blocked, which causes more severe problems with blood flow and breathing.
Total Anomalous Pulmonary Venous Return at Seattle Children’s
Our heart team has treated many children with total anomalous pulmonary venous return. We have extensive experience with the surgery these patients require. We also have a pediatric cardiac anesthesia team and a cardiac intensive care unit to help care for children who undergo heart surgery.
When you come to Seattle Children's, a team of people will take care of your child. Along with your child's cardiologist, you are connected with surgeons, cardiac intensive care unit doctors, cardiac anesthesiologists, neonatologists, nurses, cardiac sonographers, dieticians, child life specialists, social workers and others, if their expertise is needed. We work together to meet all of your child's health needs and help your family through this experience.
Seattle Children's has been treating children since 1907. Our team members are trained in their fields and also in meeting the unique needs of children. For example, the doctors who give your child anesthesia are board certified in pediatric anesthesiology. This means they have extra years of training in how to take care of kids. Our child life specialists know how to help children understand their illnesses and treatments in ways that make sense for their age.
The Adult Congenital Heart Disease Program shared by Children’s and the University of Washington can help with care throughout your child’s life.
Contact the Heart Center at 206-987-2015 for a cardiac referral, a second opinion or more information.