The heart normally receives blood from the lungs via the pulmonary veins, which ordinarily connect the lungs to the left atrium. When the pulmonary veins form abnormally and do not drain into the left atrium, the condition is said to be anomalous pulmonary veins. There are two major types with very different consequences: partial and total. Partial is abbreviated PAPVR (short for partial anomalous pulmonary venous return) and is said to be present when some of the pulmonary veins connect normally to the left atrium but some do not. Total is abbreviated TAPVR (short for total anomalous pulmonary venous return) and is said to be present when none of the pulmonary veins connect to the left atrium, but instead to other very abnormal, small veins.
PAPVR
Patients with PAPVR are usually diagnosed as toddlers or older children and have minimal symptoms. The problem is that although the child may seem to be healthy overall, the abnormal blood flow that occurs as a result of the abnormal veins slowly results in heart enlargement and eventually, many patients develop heart failure. Before surgery could safely be offered, many patient would become sick from heart failure and die as older children or young adults. Thankfully, all forms of PAPVR can now be safely addressed with surgery in patients of all sizes and ages, and they can go on to live normal, active healthy lives. The specifics of how the veins form and are arranged is different for each patient and it is important to be evaluated at a center with experience imaging and managing PAPVR.
There are a variety of different techniques to re-route the venous flow back to the left atrium and since most patients also have an ASD, this is closed as well. We have more than 30 years of experience with this lesion and our surgeons have written about some of the techniques and are familiar with all of the options. From 2009-2013 alone, we repaired 27 patients with PAPVR with a 100 percent success and survival rate. An additional number of patients have undergone repair of PAPVR as part of other more complex operations with similar survival and success.
TAPVR
Patients with TAPVR are usually diagnosed as newborns. Some patients become extremely sick in the first few days of life and require emergency surgery and even for those who don’t, surgery is required early. This is a very serious heart condition which can become lethal in a very short period of time. Our surgeons have been specially trained in newborn heart surgery and thus utilize the now preferred approach to operate in the first week or so of life for the vast majority of patients. While this provides the best possible results, TAPVR remains a challenging problem. There are different subtypes of TAPVR and if your child is diagnosed, consultation with our surgical team can be directed at the specific risks for your child’s exact type. A survival of approximately 80-90 percent can now be expected for most forms, as is the case for our patients in the last five years.
Sometimes, patients with repaired PAPVR or TAPVR return to their doctors months or years later having developed some additional blockages in the repaired veins. Although further surgery is sometimes necessary, many of these patients can be managed with catheterization, an outpatient procedure that does not require an incision. If these blockages occur in you or your child, the options for additional treatment will be thoroughly explored and will be discussed with you based on the exact anatomy.