Dextrocardia

What is dextrocardia?

Dextrocardia is a condition in which your heart is located in the right side of your chest and points to the right. Normally, your heart is in the left side of your chest and points to the left.

Dextrocardia is congenital, meaning it’s a condition you or your child have at birth. Some people with dextrocardia have no problems or complications. They learn by accident that they have it. Others have congenital heart defects or syndromes that cause symptoms and complications. How dextrocardia affects a person’s body depends on the type they have.

[h4] Types of dextrocardia

Some people have dextrocardia in isolation. That means your heart is flipped from left to right but all other organs are arranged normally. However, most people have dextrocardia along with other issues. Below are the different types of dextrocardia:

• Isolated dextrocardia: This is the simplest form. Your heart is a mirror image of a typical heart because it points to the right. There are no heart defects, and your heart functions normally. You won’t have any symptoms.
• Dextrocardia with situs inversus: In this type, your heart is a “mirror image” but other organs are flipped too. This affects organs in your chest and belly (abdomen) like your lungs and liver. This type may cause symptoms, usually related to other problems.
• Dextrocardia with heart defects: Many people with dextrocardia also have heart defects that impact their heart function. These defects cause symptoms and complications that often need treatment.

Heart defects found in people with dextrocardia include:

• Double outlet right ventricle: Your aorta extends from your right ventricle rather than your left ventricle.
• Endocardial cushion defect: There are problems with the walls that separate the four chambers of your heart.
• Pulmonary stenosis: Your pulmonary valve is narrowed and stiff.
• Pulmonary atresia: Your pulmonary valve isn’t formed properly and blood doesn't flow through it.
• Single ventricle: You should have two ventricles (lower chambers of your heart) but instead only have one.
• Transposition of the great arteries: The locations of your aorta and pulmonary artery are switched.
• Ventricular septal defect: There’s a hole in the wall (septum) between your left and right ventricles.

Dextrocardia was one of the earliest congenital heart malformations identified by scientists. It was first identified in the 1600s and written about in the 1700s. Over the years, we’ve learned more about this condition and how it connects with other syndromes.

Which syndromes are associated with dextrocardia?

Many people with dextrocardia have an additional congenital syndrome including:

• Heterotaxy: This condition causes mirrored organs in your chest and abdomen. But those organs aren’t formed properly or don’t work as they should. This condition can affect your heart, lungs, liver, gallbladder, intestines and spleen. It can lead to serious complications.
• Asplenia: This means you were born without a spleen. Some people with heterotaxy have this condition. It causes you to be more vulnerable to infections.
• Malrotation: People with malrotation have intestines that aren’t formed properly. They may coil in the wrong direction. This can lead to digestive issues.
• Kartagener syndrome: This is a type of primary ciliary dyskinesia. It’s caused by a genetic change (mutation) that affects the small hairs (cilia) in your respiratory tract. It leads to frequent infections in your lungs and sinuses.

Associated syndromes and heart defects cause problems for people with dextrocardia. These issues often need treatment or long-term care.

Which other heart conditions are similar to dextrocardia?

Dextrocardia is a right-sided cardiac malposition. This means your heart is in the right side of your chest when it should be in the left side. Other right-sided cardiac malpositions include dextroposition and dextroversion. The names sound very similar, so it’s easy to get them confused. Here’s what each term means:

• Dextrocardia: Your heart is in the right side of your chest and points to the right. It’s a “mirror image” of a typical heart. Your heart formed this way before you were born. So it’s sometimes called “primary dextrocardia.”
• Dextroposition: Your heart has shifted from the left side to the right side of your chest. It still points to the left. Lung problems or other issues unrelated to your heart cause this shift. This is sometimes called “secondary dextrocardia.”

• Dextroversion: Your heart is in the right side of your chest and also rotated.

How common is dextrocardia?

Dextrocardia is a rare congenital heart condition. It occurs in about 1 in every 12,000 pregnancies.

What are the symptoms of dextrocardia?

People with isolated dextrocardia have no symptoms. But other conditions can cause a range of symptoms. Symptoms in babies and children include:

• Constant lung or sinus infections.
• Fatigue.
• Skin that looks blue (cyanosis).
• Skin that looks pale.
• Skin and eyes that look yellow (jaundice).
• Trouble breathing (dyspnea).
• Trouble gaining weight and growing.

Adults with dextrocardia may have no symptoms. But heart defects and associated syndromes may cause symptoms. They vary based on your diagnosis. Talk with your healthcare provider to learn more about symptoms and how to manage them.

What causes dextrocardia?

Dextrocardia appears to be caused by genetic changes early in pregnancy. More than 60 genes play a role in how your organs are positioned in your body. Researchers are still looking for the specific gene that causes dextrocardia.

About 1 in 4 people with dextrocardia also have primary ciliary dyskinesia. Mutations in more than 30 different genes, including DNAI1 and DNAH5, can cause this genetic disorder. Some people can be a “carrier,” meaning they have a genetic mutation but no symptoms of the condition. If both your parents are carriers, you have a 25% chance of having primary ciliary dyskinesia (with symptoms) and a 50% chance of being a carrier.

How is dextrocardia diagnosed?

Dextrocardia may be diagnosed during pregnancy through prenatal ultrasound. But your child may need imaging tests later to confirm. Children without symptomatic heart defects or genetic syndromes may not be diagnosed with dextrocardia until years later.

A physical exam might diagnose dextrocardia. Your healthcare provider uses a stethoscope to listen to your heart or your child’s heart. A noticeable heartbeat on the right side of the chest can be a sign of dextrocardia.

Tests to diagnose dextrocardia

Tests that diagnose dextrocardia include:

• Chest X-ray.
• CT scan.
• Echocardiogram (echo).
• Electrocardiogram (ECG/EKG).
• Heart MRI.

Your provider may recommend genetic testing and other tests to check for syndromes.

Dextrocardia ECG reading

An electrocardiogram (ECG/EKG) reading can show if you have dextrocardia. ECG tests create a picture of your heart’s electrical activity, which has a normal graph when the heart is in its normal location on the left. The graph will look different than usual if your heart’s location is unusual — as is the case with dextrocardia (the right side of your chest).

ECG graphs follow predictable patterns. You might hear your provider talk about the three waves of electrical activity. These are the bumps and spiky points on each line of the graph. The first small bump is called the “P wave.” It shows your heart’s pacemaker, or the electrical signal that tells your heart to beat. Next, there’s a sharp spike called the “QRS complex." This shows your strongest heartbeat as your left ventricle pushes blood out to your body. The “T wave” shows your heart’s brief relaxation before the next heartbeat.

Dextrocardia causes ECG readings with waves that are flipped in the opposite direction. The P wave, QRS complex and T wave point downward rather than upward. This could happen if the ECG electrodes are in the wrong spots. So sometimes the provider giving the test may run it again to make sure the reading is accurate. But if this “different” ECG comes up again, it can signal dextrocardia.

Ask your provider to discuss your or your child’s ECG reading with you and explain what it means.

What is the treatment for dextrocardia?

Treatment for dextrocardia depends on which heart defects or syndromes you have.

If you have dextrocardia with no heart defects, you don’t need treatment. But it’s important to share this diagnosis with any healthcare providers giving you care. This information can be helpful for them to know during certain tests and procedures.

People with heart defects often need surgery. Such surgeries may happen early in infancy or childhood. Some babies need to take medicine leading up to the surgery including:

• Diuretics: These “water pills” help remove extra fluid from your baby's body.
• Inotropic agents: These drugs strengthen your baby's heart’s pumping action.
• ACE inhibitors: These drugs lower blood pressure so your baby's heart doesn’t have to work as hard.

The specific type of surgery your baby needs depends on their congenital heart defect. Your child’s provider will discuss treatment options with you.

Does dextrocardia need surgery?

Dextrocardia doesn’t need surgery or correction if it’s not causing any problems. But babies born with dextrocardia often have heart defects or genetic syndromes. And those defects or syndromes can prevent a baby’s heart from functioning as it should. In those cases, surgery may be needed.

How can I prevent dextrocardia?

There’s no specific way to prevent dextrocardia. It’s likely caused by genetic changes that happen early in pregnancy. But prenatal care may impact a baby’s chances of having congenital heart disease. If you’re pregnant or planning a pregnancy, here are some steps you can take:

• Quit smoking and avoid drinking alcohol.
• Avoid secondhand smoke.
• Don’t use recreational drugs, especially cocaine.
• Manage diabetes if you have it. Diabetes may increase a birthing parent’s risk of having a child with dextrocardia.

Talk with your healthcare provider about any concerns you have. Genetic testing may be helpful, especially if you have a biological family history of congenital heart disease.

How long can a person live with dextrocardia?

A person can live with dextrocardia for a long time, but the outlook varies based on other diagnoses. Babies born with isolated dextrocardia (no defects or syndromes) have a normal life expectancy. But babies born with heart defects or genetic syndromes may need treatments or surgeries to manage those conditions. Talk with your healthcare provider about what to expect and the long-term outlook.

What are the complications of dextrocardia?

Complications depend on the defects and syndromes you have along with dextrocardia. They vary widely based on the individual but can include:

• Blocked intestines (in people who have intestinal malrotation).
• Heart failure.
• Infections (in people who don’t have a spleen).
• Infertility (in people assigned male at birth who have Kartagener syndrome).
• Recurrent sinus infections and pneumonia.
• Respiratory failure.

When should I call my child's healthcare provider?

If your child has dextrocardia, talk with their healthcare provider about what to expect. You may need to look for symptoms related to heart defects or syndromes including:

• Getting sick often.
• Getting tired easily.
• Trouble gaining weight.

Call 911 if your child:

• Has skin that looks blue (cyanosis).
• Has skin that looks yellow (jaundice).
• Has trouble breathing.

If you have dextrocardia, talk with your healthcare provider about how other conditions may affect your body. Your provider will tell you which symptoms to look for and how to manage them. Call your provider any time you have questions or concerns.

What questions should I ask my child's healthcare provider?

If your child was diagnosed with dextrocardia, you probably have many questions. Your child's healthcare provider will share resources and give you plenty of information. You may also want to ask:

• Does my child have congenital heart defects?
• Does my child have any genetic syndromes?
• What tests will my child need? When do they need these tests?
• Will my child need surgery?
• Should anyone in our family have genetic testing?
• What is the outlook for my child?
• What can I do to manage any symptoms for my child at home?

A note from Cleveland Clinic

Dextrocardia is usually diagnosed along with other medical conditions. If your child has dextrocardia, their healthcare provider will discuss whether they need treatment. It’ll depend on your child’s heart function and whether other conditions are causing problems. Your child’s healthcare team will work with you every step of the way. It’s also helpful to connect with other parents who have children with heart problems. Building a supportive community can make a huge difference as you care for your child.