Before you begin reading about total anomalous pulmonary venous return, please read the explanation of how the normal heart works for a basic understanding of its structure and function.
Arteries carry blood away from the heart; veins carry it toward the heart. The pulmonary veins are very important: They carry the “red” blood that has picked up oxygen in the lungs back to the heart to be pumped out to the body.
There are typically four pulmonary veins, two from each lung. Normally they all connect
View TAPVR: Infracardiac Illustrationdirectly into the heart’s left atrium (left upper chamber). From the left atrium, blood travels into the left ventricle (left lower chamber), which pumps it out to the aorta and to the body.
“Anomalous” means abnormal. In a child born with total anomalous pulmonary venous return (TAPVR), the pulmonary veins connect to other veins, and ultimately drain their blood into the right atrium. You may also hear the term total anomalous pulmonary venous connection (TAPVC). Ordinarily, the right atrium only receives “blue” blood coming back from the body without oxygen, which then passes into the right ventricle and is pumped to the lungs. In TAPVR, the right atrium receives BOTH “blue” blood from the body and “red” blood from the lungs. Because of this, children with TAPVR have some degree of cyanosis (blue tint of the lips, skin and nails)
View TAPVR: Supracardiac IllustrationAs a result, the right side of the heart is overworked, pumping the extra blood it receives. Frequently, there is obstruction (blockage) to the veins that receive blood from the pulmonary veins, which causes a backup of blood in the lungs. This disrupts the normal flow of blood between the lungs and the body. Additionally, the blood leaving the heart for the body has a lower level of oxygen.
Children with TAPVR also have other heart defects. They have a hole in the wall separating the two upper chambers of the heart (atrial septal defect) and may have a patent ductus arteriosus, an extra blood vessel between the pulmonary arteries and the aorta. These heart defects can actually help the child with TAPVR survive by allowing more blood to get from the right side of the heart to the left side and out to the body. In fact, children without at least an atrial septal defect will not survive.
Our pediatric cardiologists will explain your child’s heart anatomy in detail. Generally, there are four types of total anomalous pulmonary venous return:
Total anomalous pulmonary venous symptoms include:
The severity of TAPVR symptoms varies.
In some cases, newborns with TAPVR have difficulty breathing and quickly become very ill. This occurs when the pulmonary veins are too narrow or are obstructed at some point, and blood can’t flow from the lungs as quickly as it should. This is called TAPVR with pulmonary obstruction. These children are typically transported to the Cardiac Center at CHOP from their birth hospitals, and are admitted to the Tabas Cardiac Intensive Care Unit to be stabilized.
In other cases, TAPVR is diagnosed in the first few months of life after a child demonstrates milder symptoms such as a heart murmur or cyanosis (blue tint to skin).
Diagnosis of TAPVR may require some or all of these tests:
TAPVR requires open heart surgery in all cases.
Critically ill newborns will have surgery immediately. If the child is not critically ill, doctors may wait up to two months to perform surgery, depending on the strength of the child and on the heart anatomy.
To understand the surgery, one important thing to know about TAPVR is that the pulmonary veins, despite their abnormal connections to other veins, all end in a collection (called a “confluence”) at the back of the left atrium. The surgeon opens the confluence so that the veins can drain into the left atrium. Then he or she ties off all the abnormal connections between the pulmonary veins and other veins, so that blood can follow only the path to the left atrium. The surgeon also closes septal defects (the abnormal holes) with tiny patches or stitches, and closes the patent ductus arteriosus. As the child ages, the lining of the heart grows over the stitches.
Children who were born with TAPVR and had it repaired must continue to see a pediatric cardiologist regularly. Children who were critically ill as newborns may have a longer road to recovery. In most cases, however, children won’t experience long-term effects — they won’t have to remain on any medicines or to limit sports.
Rarely, the pulmonary veins become obstructed later in life and additional surgery or a catheterization procedure is required. Also rarely, children experience arrhythmia (abnormal heart rhythm), which may be treated with medicines, radiofrequency ablation, or a pacemaker.
It is important that children who were born with total anomalous pulmonary venous return continue to see a cardiologist. We will help patients transition care to an adult congenital heart disease specialist.
The Philadelphia Adult Congenital Heart Center, a joint program of The Children’s Hospital of Philadelphia and the University of Pennsylvania, meets the unique needs of adults who were born with heart defects.
Because of enormous strides in medicine and technology, today most children born with total anomalous pulmonary venous return go on to lead productive lives as adults.
Contact the Cardiac Center at The Children's Hospital of Philadelphia for a second opinion or for more information.
Reviewed by: Julie Brothers, MD, FAAP
Date: June 2013
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