What Are The Symptoms Of PAPVR In Adults & How Do You Test For It?

If only one of the pulmonary veins has an anomaly, the condition does not show any symptoms, however, when both the veins have abnormalities, it can cause shortness of breath during strenuous work.1

For some people there are no signs and symptoms nevertheless, certain people have complications later in their lifetime.2

PAPVR is a rare congenital cardiovascular problem diagnosed during an ultrasound when the individual is tested for some injury.3

What Are The Symptoms Of PAPVR?

Pulmonary veins that come from individual alveoli are a group of blood vessels that carry oxygenated blood from the lungs to the heart and then pumps to the rest of the body. There are four pulmonary veins in total with two originate from each lung, right, and the left. Pulmonary heart disease is one of the most common diseases in the United States affecting hundreds of people every year.

If only one of the pulmonary veins has an anomaly, the condition does not show any symptoms, however, when both the veins have abnormalities, it can cause shortness of breath during strenuous work. Adults with this cardiovascular problem will require lifetime treatment and periodic follow-up appointments with physician professional in inherited heart problems.1

Adults who are parenthetically diagnosed have a better prognosis if the individual shows no symptoms. For some people there are no signs and symptoms nevertheless, certain people have complications later in their lifetime. Surgery is the best-suited treatment for PAPVR. Adult patients who have not undergone surgery in their early childhood may require repair surgery when the heart enlarges, or dilation occurs.

Typical symptoms include

  • Heart rhythm problems (heart arrhythmias) causing the heart to beat too fast, too slow, or very irregular.
  • A bluish tint to the skin and mucous membrane (cyanosis) due to deoxygenation
  • Swelling of body parts(edema) due to inflammation.

How Do You Test For PAPVR?

This imperfection often remains undiagnosed during infancy, and even during adulthood. The diagnosis and treatment for Partial anomalous pulmonary venous return may be challenging. However later in life, symptoms may necessitate surgical repair.

Due to medical developments and advancement in therapy, patients with CHD are reaching adulthood and living longer. PAPVR is a rare congenital cardiovascular problem diagnosed during an ultrasound when the individual is tested for some injury. Although PAPVR is partial and not critical unlike total PAPVR, early diagnosis of the condition produces better outcomes.

Tests to diagnose PAPVR include:

Electrocardiogram- a simple, painless test to measure the heart’s electric signals.

Chest X-ray– The most common diagnostic method for producing images of the heart, lungs and the chest

Pulse Oximetry– a painless procedure of measuring the oxygen in the blood

Echocardiogram-An ultrasound procedure to determine the functionality of the heart muscle and the valves.

Imaging Test And MRI– to get the detailed picture of the structures within the heart with the help of powerful magnetic field

Cardiac Catheterization– a procedure to diagnose and treat heart conditions.3

Partial anomalous pulmonary venous return (PAPVR) is an uncommon cardiovascular abnormality that has been identified in 0.4-0.7 percent of autopsies. Most of this condition is associated with atrial septal defect (ASD).

When an individual is affected with PAPVR, there is an abnormality in one or more of the pulmonary veins causing pulmonary hypertension. This eventually makes the heart work harder to pump oxygenated red blood through the lungs. When the pressure keeps increasing or stays high, it can result in heart failure.

References:

  1. Burns, Paul Burns, and Jasper. “Partial Anomalous Pulmonary Venous Return: Congenital Heart Disease – Cove Point Foundation: Johns Hopkins Children’s Hospital.” Partial Anomalous Pulmonary Venous Return | Congenital Heart Disease – Cove Point Foundation | Johns Hopkins Children’s Hospital, www.pted.org/?id=papvr4.
  2. “Congenital Heart Disease in Adults.” Mayo Clinic, Mayo Foundation for Medical Education and Research, 14 May 2020, www.mayoclinic.org/diseases-conditions/partial-anomalous-pulmonary-venous-return/cdc-20385691.
  3. “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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