PAPVR is not critical but TAPVR is a serious heart defect and when left untreated survival becomes challenging1.
There are potential complications with any major surgery and the risk includes bleeding abnormal heart murmurs and associated infections.2
The condition is improbable to be reversible unlike treating partial anomalous pulmonary venous return with appropriate diagnosis and surgery.3, 4
Is PAPVR A Serious Condition?
PAPVR is an abnormal cardiovascular problem due to abnormal development of the prenatal heart that occurs during the first eight weeks of pregnancy. Most heart defects are often associated with the atrial septal defect and surgical intervention is the only possible procedure to repair the condition.
PAPVR is not critical but TAPVR is a serious heart defect and when left untreated survival becomes challenging. It encompasses less than 1% of all congenital cardiovascular complications. During a normal condition, pulmonary veins drain the oxygen-rich blood to the left atrium. There are four pulmonary veins in total which perform the functionality of bringing the oxygen-rich red blood from the lungs to the upper chamber of the heart.1
The cause of the condition remains unknown however the degree of signs and symptoms of the condition depends on the affected veins. When there is too much blood flow in the lungs the veins are affected and there will be a no longer a direct path for the oxygen-rich blood to drain through the heart. Because of this, there will be low stamina and shortness of breath in these children.
Generally, these symptoms are very sporadic and sometimes never even occur. However, over the years, when the individual turns into an adult excess blood flow result in pulmonary tension and may necessitate emergency surgery. There are potential complications with any major surgery and the risk includes bleeding abnormal heart murmurs and associated infections.2
Can PAPVR Be Reversed?
Most patients with partial anomalous pulmonary venous return often wonder if there are any ways to reverse the condition. However, the condition is improbable to be reversible unlike treating partial anomalous pulmonary venous return with appropriate diagnosis and surgery.
If an important left to right shunt is left untreated, pulmonary vascular remodeling can happen to ensure in the progression of pulmonary arterial hypertension (PAH). So, reparative surgery is required to alter the disease and perhaps the only curative option. But unfortunately, the survival rate of heart-lung transplant is not significant and remain as low as 30-40 %.
Some patients may require catheter embolization (a minimally invasive treatment to remove the block of one or more blood vessels) to prevent the excessive flow of blood towards the heart. The results are significant and there is no residual flow through the anomalous connection.3, 4
Partial anomalous pulmonary venous return is a rare congenital irregularity in which some of the pulmonary veins deplete incorrectly into the superior vena cava (SVC) or immediately into the right atrium (RA). This causes the oxygen-rich plasma to flow back to the lungs instead of on to the rest of the body.
PAPVR is present from the time of birth. Since the oxygen-rich red blood is flowing continuously to the right atrium instead of the left atrium the chambers of the heart become enlarged and dilated resulting in abnormal heart rhythms and increased lung pressure.
- “Anomalous Pulmonary Venous Return.” Anomalous Pulmonary Venous Return | CS Mott Children’s Hospital | Michigan Medicine, www.mottchildren.org/conditions-treatments/ped-heart/conditions/anomalous-pulmonary-venous-return.
- “Pediatric Total Anomalous Pulmonary Venous Return (TAPVR) – Conditions and Treatments: Children’s National Hospital.” Conditions and Treatments | Children’s National Hospital, childrensnational.org/visit/conditions-and-treatments/heart/total-anomalous-pulmonary-venous-return-tapvr.
- El-Kersh, Karim, et al. “Partial Anomalous Pulmonary Venous Return: A Case Series with Management Approach.” Respiratory Medicine Case Reports, Elsevier, 3 Apr. 2019, www.ncbi.nlm.nih.gov/pmc/articles/PMC6456451/.
- Pendela, Venkata Satish, et al. “Partial Anomalous Pulmonary Venous Return Presenting in Adults: A Case Series With Review of Literature.” Cureus Journal of Medical Science, 1 June 2020, www.cureus.com/articles/32734-partial-anomalous-pulmonary-venous-return-presenting-in-adults-a-case-series-with-review-of-literature.
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