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Types of Dextrocardia

Medically Reviewed by Dan Brennan, MD on April 14, 2021

Dextrocardia is a heart condition that makes the heart move out of its usual position. It points towards the right side of your chest instead of the left side. The condition is congenital, meaning that people are born with it, but it's rare.

Dextrocardia can also happen when all your visceral organs are on the "wrong" side of the body. For example, the liver and spleen may also be out of their usual position. This type of dextrocardia is called situs inversus. Sometimes doctors can correct the condition through surgery. Dextrocardia can be congenital, which happens at birth, or develop later in life.

What Causes Dextrocardia?

Researchers don't yet know what causes dextrocardia, but they know that it happens as a fetus develops. The heart's anatomy assumes various forms where it may face the opposite side or have defects in its chambers. Flaws in the abdomen, lungs, or chest may also cause the heart to develop facing the other side of the body.

If that's the case, you’re likely to have more problems with other body organs. When you have multiple organ defects, the condition is called heterotaxy syndrome. Dextrocardia occurs in many types.

Double Outlet Right Ventricle (DORV)

In this dextrocardia type, the aorta connects to the right ventricle instead of the left ventricle. The right ventricle is the part of the heart that pumps blood without oxygen to the lungs. The left ventricle pumps blood with oxygen to the body. The aorta carries oxygen-rich blood to the body from the heart. No arteries run from the left ventricle responsible for pumping blood to the body.

What causes a double outlet right ventricle? Under normal conditions, the aorta connects to the left ventricle. The pulmonary artery connects to the right ventricle. In DORV, both of these arteries flow out of the right ventricle. The problem is that the right ventricle carries blood without oxygen, which then circulates in the body.

In most cases, a ventricular septal defect (VSD) happens alongside DORV. Blood with oxygen from the lungs flows from the heart's left side, passing through the VSD opening and into the right ventricle. When you have DORV and VSD, blood with oxygen mixes with blood without oxygen. You may not get enough supply of oxygen, which causes your heart to work harder.

Endocardial Cushion Defect (ECD)

ECD is an abnormal heart condition that makes the walls of the heart's chambers form poorly. Sometimes the walls are absent. The valves that separate the lower and upper chambers of the heart develop defects as the heart forms. As a congenital disease, this condition is present from birth.

What causes endocardial cushion defect? ECD happens as the heart gets formed before birth. The endocardial cushions are thick and develop into the walls that form the borders of the heart chambers. They also create the tricuspid and mitral valves that separate the atria (upper collecting chambers) and ventricles (lower pumping chambers) of the heart.

When there is no separation of the heart’s two sides, several problems occur:

  • The blood flow to the heart increases, raising the pressure in the lungs. In ECD, the blood finds its way through the abnormal openings to the lungs.
  • The condition makes the heart work harder to pump blood throughout the body. This causes the heart muscle to become large and weak, leading to heart failure. Eventually, your body swells and you develop breathing problems and difficulty in feeding and normal development.
  • The increased blood pressure in the lungs leads to a condition called cyanosis. This means that blood starts to flow from the right to the left side of the heart. The blood without oxygen mixes with blood with oxygen. The heart pumps blood with less oxygen than usual to the body. In the end, you develop bluish skin, or cyanosis.

Pulmonary Valve Stenosis

This heart valve disorder involves the pulmonary valve that separates the right ventricle and the pulmonary artery. This is the artery that carries blood without oxygen to the lungs. The valve narrows and doesn't open wide enough to transport blood, limiting the amount that reaches the lungs. This is called stenosis.

The causes of pulmonary valve stenosis are congenital, meaning that they are present at birth. A problem occurs when the heart is developing while the fetus is still in the womb. Experts suspect that genetic factors are responsible for the problem. The problem may occur alone or with other heart defects that are present at birth. Pulmonary valve stenosis runs in families, but is rare. Its symptoms can be mild or severe.

Final Thoughts

Dextrocardia is a heart condition that changes the structure and location of the heart. The defects occur during pregnancy as a fetus develops in the womb, but can also be present in adults. The condition occurs in many forms, some of which doctors can correct through surgery if detected early. Other people have no symptoms.

WebMD Medical Reference

Sources

SOURCES:‌‌

American Heart Association: “Pulmonary Valve Stenosis.”

American Heart Association Journals: “Essential Modifiers of Double Outlet Right Ventricle.”American Journal of Roentgenology: “Approach to Dextrocardia in Adults: Review.”

Boston Children’s Hospital: “Double Outlet Right Ventricle DORV.”

The British Medical Journal: “Types of Dextrocardia.”

Genetic and Rare Disease Information Center: “Dextrocardia with situs inversus,” “Dextrocardia.”

Journal of Medical Case Reports: “Complete endocardial cushion defects in pregnancy: a case report.”

Minnesota Department of Health: “Endocardial Cushion Defect (also called atrioventricular (AV) canal of septal defects.”

National Center for Biotechnology Information: “Dextrocardia.”

National Organization for Rare Disorders: “Dextrocardia with Situs Inversus.”

Radiology: “On the Various Types of Dextrocardia and Their Diagnostics.”

StatPearls: “Cyanosis.”

World Journal of Cardiology: “Pulmonary valve stenosis: Etiology, diagnosis, and management.”

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