Dextrocardia (patient information)

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Overview

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).

What causes Dextrocardia?

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)

Possible Complications?

Complications depend on whether dextrocardia is part of a larger syndrome, and whether other problems exist in the body. Complications include:

• Bacteria in the blood (septic shock)
• Blocked intestines (due to a condition called intestinal malrotation)
• Congestive heart failure
• Death
• Infection (heterotaxy with no spleen)
• Infertility in males (Kartagener syndrome)
• Repeated pneumonias
• Repeated sinus infections (Kartagener syndrome)

Exams and Tests

There are no signs of dextrocardia if the heart is normal. Conditions that can include dextrocardia may cause the following signs:

• Abnormal arrangement and structure of the organs in the abdomen
• Enlarged heart
• Problems with the structure of the chest and lungs, seen on x-rays
• Rapid breathing or problems breathing
• Rapid pulse

Tests to diagnose dextrocardia include:

• Computed tomography (CT) scans
• Magnetic resonance imaging (MRI) of the heart
• Ultrasound of the heart (echocardiogram)
• X-rays

Treatment options

A complete mirror image dextrocardia with no heart defects requires no treatment. It is important, however, to let the child's health care provider know the heart is on the right side of the chest. This information can be important in some exams and tests. Treatment for conditions that include dextrocardia depends on whether the infant has other heart or physical problems in addition to dextrocardia. If heart defects are present with dextrocardia, the baby will most likely need surgery. Critically ill babies may need treatment with medication before surgery. These medications help the baby grow larger so surgery is less difficult to perform. Medications include:

• "Water pills" (diuretics)
• Medications that help the heart muscle pump more forcefully (inotropic agents)
• Medications that lower blood pressure and ease the workload on the heart (ACE inhibitors)

The baby might also need surgery to correct problems in the organs of the abdomen. Children with Kartagener syndrome will need repeated treatment with antibiotics for sinus infections. Children with a missing or abnormal spleen need long-term antibiotics. All children with heart defects should get antibiotics before surgeries or dental treatments.

When to Contact a Medical Professional?

Call your health care provider if your baby:

• Is often ill
• Does not seem to gain weight
• Tires easily

Seek emergency care if your baby has:

• A bluish tinge to the skin
• Trouble breathing
• Yellow skin (jaundice)

Outlook (Prognosis)

Babies with simple dextrocardia have a normal life expectancy and should have no problems related to the location of the heart. When dextrocardia appears with other defects in the heart and elsewhere in the body, how well the baby does depends on the severity of the problems. The death rate in babies and children without a spleen may be high due to infections. This is at least partially preventable with daily antibiotics.

Prevention

Some syndromes that include dextrocardia may run in families. If you have a family history of heterotaxy, talk to your health care provider before becoming pregnant. While there are no known ways to prevent dextrocardia, avoiding the use of illegal drugs (especially cocaine) before and during pregnancy may lower the risk of this problem. Talk to your health care provider if you have diabetes, because it may contribute to your risk of having a child with certain forms of dextrocardia.

Sources

http://www.nlm.nih.gov/medlineplus/ency/article/007326.htm