Dextrocardia - Overview

Definition of Dextrocardia :

Dextrocardia is a condition in which the heart is pointed toward the right side of the chest instead of normally pointing to the left. It is present at birth (congenital).

Causes, incidence, and risk factors:

During the early weeks of pregnancy, the baby's heart develops. Sometimes, for reasons that are unclear, the heart develops and turns so that it points to the right side of the chest instead of the left side.

There are several types of dextrocardia. Most involve other defects of the heart and abdomen area.

The simplest type of dextrocardia is one in which the heart is a mirror image of the normal heart, and no other problems exist. This condition is rare. Usually in this case, the organs of the abdomen and the lungs will also be arranged in a mirror image of their normal position. For example, the liver will be on the left side instead of the right.

Some people with mirror-image dextrocardia have a problem with the fine hairs (cilia) that filter the air going into their nose and air passages. This condition is called Kartagener syndrome.

In the more common types of dextrocardia, heart defects are present in addition to the abnormal location of the heart. The most common heart defects seen with dextrocardia include:

• Double outlet right ventricle
• Endocardial cushion defect
• Pulmonary stenosis or atresia
• Single ventricle
• Transposition of the great vessels
• Ventricular septal defect

The abdominal and chest organs in babies with dextrocardia may be abnormal and may not work correctly. A very serious syndrome that appears with dextrocardia is called heterotaxy. Heterotaxy means the organs (atria of the heart and abdominal organs) are not in their usual places.

In heterotaxy, the spleen may be completely missing. Because the spleen is an extremely important part of the immune system, babies born without a spleen are in danger of severe bacterial infections and death. In another form of heterotaxy several small spleens exist, but may not work correctly.

Heterotaxy may also include:

• Abnormal gallbladder system
• Problems with the lungs
• Problems with the structure of the intestines
• Severe heart defects

Possible risk factors for dextrocardia include:

• Family history of the condition
• Mother with diabetes (may play a role in some forms of dextrocardia)