Overview
Partial anomalous pulmonary venous return (PAPVR) is a rare form of congenital heart disease (congenital means you’re born with it). It affects the flow of oxygen-rich blood from your lungs to your heart. Your pulmonary veins normally return all freshly oxygenated blood to the left side of your heart. But in PAPVR, they deliver some of this blood to the right side, which should only hold oxygen-poor blood.
This leads to a higher volume of blood than normal in your right heart chambers, which have to work harder to compensate. This extra work may take a toll on your heart and your overall health.
PAPVR affects people differently depending on how much blood goes to the wrong place. You may have a mild form of the condition and never develop symptoms. But if you do develop symptoms or complications, healthcare providers can manage your condition. Surgery is typically successful at treating PAPVR and helping you live a long and healthy life.
Normal blood flow vs. PAPVR blood flow
To understand how PAPVR affects blood flow, it helps to learn how blood normally flows through your heart and lungs. Here’s a breakdown:
- Oxygen-poor blood from your body flows into two large veins called your superior and inferior vena cava. These veins empty directly into your heart’s right atrium (top right chamber).
- The oxygen-poor blood flows from your right atrium into your right ventricle.
- Your right ventricle pumps the blood to your lungs through your pulmonary arteries. In your lungs, the blood gains oxygen and is ready to go back to your heart.
- The oxygen-rich blood travels through several pulmonary veins to return to your heart, entering at your left atrium.
- The oxygen-rich blood flows into your left ventricle.
- Your left ventricle pumps this blood out to your body through your aorta.
Notice that normally, oxygen-poor blood stays in the right chambers, and oxygen-rich blood stays in the left chambers.
With PAPVR, this system doesn’t work as it should. PAPVR creates a left-to-right cardiac shunt, or an atypical pattern of blood flow through your heart. “Partial anomalous” means some (but not all) of the oxygen-rich blood from your lungs doesn’t return to your heart in the normal way.
This is because one or more of your pulmonary veins carry some oxygen-rich blood into your right atrium, rather than your left atrium. So, your right atrium contains a mix of oxygen-poor and oxygen-rich blood. Your right ventricle has no choice but to pump this mixed blood out to your lungs.
This means two things are happening that shouldn’t. First, some oxygen-rich blood unnecessarily returns to your lungs, where it just came from. Second, your right ventricle has a higher load of blood it must pump to your lungs. This extra work can take a toll on your heart over time.
PAPVR with other heart disorders
Some people who have PAPVR have no other heart disorders. This is called isolated PAPVR. However, many people with PAPVR also have other anomalies such as an atrial septal defect (ASD).
Rarely, PAPVR occurs along with an underdeveloped right lung and pulmonary artery (scimitar syndrome).
How rare is partial anomalous pulmonary venous return?
Researchers estimate that about 4 to 7 in 1,000 people have PAPVR. This number may be higher, though, since some people never show symptoms and therefore may never receive a diagnosis.
Symptoms
When to see a doctor
Complications
- High blood pressure.
- Diabetes.
- Heart failure.
- Some types of heart valve disease.
Prevention
- Control high blood pressure, high cholesterol and diabetes.
- Don't smoke or use tobacco.
- Eat a diet that's low in salt and saturated fat.
- Exercise at least 30 minutes a day on most days of the week unless your health care team says not to.
- Get good sleep. Adults should aim for 7 to 9 hours daily.
- Maintain a healthy weight.
- Reduce and manage stress.
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