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Dextrocardia

Definition

Dextrocardia is a condition in which the heart is pointed toward the right side of the chest. Normally, the heart points to the left. The condition is present at birth (congenital).

Alternative Names

Dextroversion; Dextrorotation

Causes

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

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

In the simplest type of dextrocardia, the heart is a mirror image of the normal heart and there are no other problems. This condition is rare. When this occurs, the organs of the abdomen and the lungs will often also be arranged in a mirror image. 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, other heart defects are also present. The most common of these include:

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. In this condition, many of the organs are not in their usual places and may not work properly. For example, the spleen may be completely missing. The spleen is an important part of the immune system, so babies born without this organ are in danger of severe bacterial infections and death. In another form of heterotaxy, several small spleens exist, but they may not work correctly.

Heterotaxy may also include:

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

Possible risk factors for dextrocardia include a family history of the condition.

Symptoms

There are no symptoms of dextrocardia if the heart is normal.

Conditions that may include dextrocardia may cause the following symptoms:

  • Bluish skin
  • Difficulty breathing
  • Failure to grow and gain weight
  • Fatigue
  • Jaundice (yellow skin and eyes)
  • Pale skin (pallor)
  • Repeated sinus or lung infections

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
  • EKG -- a test to examine the electrical system of the heart
  • Magnetic resonance imaging (MRI) of the heart
  • Ultrasound of the heart (echocardiogram)
  • X-rays

Treatment

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.

The type of treatment needed depends on the heart or physical problems the infant may have in addition to dextrocardia.

If heart defects are present with dextrocardia, the baby will most likely need surgery. Babies that are very ill may need to take medicines before they can have surgery. These medicines help the baby grow larger so surgery is easier to perform.

Medicines include:

  • "Water pills" (diuretics)
  • Drugs that help the heart muscle pump more forcefully (inotropic agents)
  • Drugs 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 may need to get antibiotics before surgeries or dental treatments.

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.

Babies and children without a spleen may have frequent infections. This is at least partially preventable with daily antibiotics.

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)

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 color to the skin
  • Trouble breathing
  • Yellow skin (jaundice)

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.

There are no known ways to prevent dextrocardia. However, 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. This condition may contribute to your risk of having a child with certain forms of dextrocardia.

References

Zahka KG. Cardiovascular problems of the neonate. In: Martin RJ, Fanaroff AA, Walsh MC, eds. Fanaroff and Martin's Neonatal-Perinatal Medicine. 9th ed. Philadelphia, PA: Mosby Elsevier; 2010:part 8.

Review Date:5/14/2014
Reviewed By:Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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