What Leads To PAPVR & Can It Be Cured?

The exact cause of partial anomalous pulmonary venous (PAPVR) is not known but medical studies shows that it can be due to the abnormal development of pulmonary veins.1

In PAPVR, the pulmonary vein drains the blood to the right atrium instead of the left, resulting in oxygenated red blood flow to the lungs instead of on to the rest of the body.2

These heart defects are usually treated through repair surgery and it produces excellent reproducible outcomes.3,4

What Leads To PAPVR?

The exact cause of the condition is not known but medical studies show that it can be due to the abnormal development of pulmonary veins. In PAPVR, the pulmonary vein drains the blood to the right atrium instead of the left, resulting in oxygenated red blood flow to the lungs instead of on to the rest of the body. The condition is generally noticed at the time of birth.

As a result, the infant doesn’t receive sufficient oxygen and experience complications. To live with this problem, the infants with partial anomalous pulmonary venous return have a hole between the right and left atrium for the blood to get mixed and to transmit throughout the body. This condition is referred to as ASD.

However, in certain cases, the child in addition to ASD suffers other cardiovascular diseases as well. Since PAPVR is a birth defect, repair surgery is best suited to treat the abnormal connection and to patch the hole. Prognosis of PAPVR is excellent however TAPVR produces acute complications and can be life-threatening.1

A study was conducted on two patients who exhibited PAH secondary to PAPVR with increased dyspnea at rest and worsening lower extremity swelling. The patients had no prior symptoms and had a good quality of life. Both of these patients had no history of smoking and alcohol.

Physical examinations on these patients showed irregular heartbeat with a pulse rate of 130 and the blood pressure was also fairly high. Imaging tests were done on the chest and it showed no pulmonary emboli however smaller bilateral pleural effusions were observed. The early prevalence of left-to-right pushing causes the condition to be clinically undetected.2

Can PAPVR Be Cured?

These heart defects are usually treated through repair surgery and it produces excellent reproducible outcomes. PAPVR surgery is an open-heart surgery typically performed to fix the abnormal functionality of the pulmonary vein. The surgery is very safe and effective. After surgery, your child may be required to take antibiotics to prevent infections of the heart valves.

Most cases after surgery recover well and the long-term outcome of Partial anomalous pulmonary venous return (PAPVR) is excellent. However, you need careful follow-ups with your specialist after the surgery.

Some children may have persistent problems. During such instances, follow-up surgery or procedures will be required.3,4

malous pulmonary venous return (PAPVR) is a rare cardiovascular disease developed due to the abnormality of the pulmonary veins that suffers a failure of the left atrium. This results in increased pressure on the right side and often accompanied by Atrial Septal Defect (ASD, a congenital heart problem resulting in the hole in the wall between two chambers of the heart)

The defect allows oxygenated red blood to drain through the blood chambers of the heart.

References:

  1. Sears, Edmund H, et al. “Partial Anomalous Pulmonary Venous Return Presenting with Adult-Onset Pulmonary Hypertension.” Pulmonary Circulation, Medknow Publications & Media Pvt Ltd, 2012, www.ncbi.nlm.nih.gov/pmc/articles/PMC3401879/.
  2. “Congenital Heart Defects – Facts about TAVPR.” Centers for Disease Control and Prevention, Centers for Disease Control and Prevention, 12 Nov. 2019, www.cdc.gov/ncbddd/heartdefects/tapvr.html.
  3. TC. Demos, HV. Posniak, et al. “Surgical Repair of Partial Anomalous Pulmonary Venous Return with Intact Atrial Septum in a 65-Year-Old Woman: a Case Report.” Journal of Medical Case Reports, BioMed Central, 1 Jan. 1970, jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-018-1874-x.
  4. “Congenital Heart Defects – Facts about TAVPR.” Centers for Disease Control and Prevention, Centers for Disease Control and Prevention, 12 Nov. 2019, www.cdc.gov/ncbddd/heartdefects/tapvr.html.

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