Did you know, Parents , that there is a rare condition where the baby’s heart points to the right side of the chest and not the left. This is known as dextrocardia.
In general, this condition is not life-threatening for your baby. However, babies who have it are usually at risk for more serious complications, such as heart defects and abdominal organ disorders.
Definition of Dextrocardia
Dextrocardia is a condition where the heart is located on the right side of the chest and points to the right. While in general, the heart is on the left side of the chest and points to the left.
This condition is congenital or has been present since a person was born. Some people with dextrocardia do not have any problems or complications. They find out by accident that they have it.
In other cases, a person with this condition has a congenital heart defect or syndrome that causes symptoms and complications. How this rare disorder affects a person’s body depends on the type they have. Below are the different types of dextrocardia:
- Isolated dextrochromia i: This is the simplest form. The heart is a mirror image of a typical heart because it points to the right. There are no heart defects and everything is functioning normally. Your baby also does not show any symptoms.
- Dextrocardia with situs inversus : In this type, the heart is a “mirror image” but other organs are also reversed. It affects organs in the chest and abdomen such as the lungs and liver. This type can cause symptoms, usually related to other problems.
- Dextrocardia with heart defects : Many people with dextrocardia also have heart defects that affect their heart function. These defects cause symptoms and complications that often require treatment.
Symptoms of Dextrocardia
There are no symptoms of this rare disorder if the child’s heart is functioning normally. Conditions that may include dextrocardia can cause the following symptoms:
- Bluish skin
- Difficulty breathing
- Failure to thrive and gaining weight
- Fatigue
- Jaundice (yellow skin and eyes)
- Pale skin (pale)
- Recurrent sinus or lung infections
Babies with this condition may also have a hole in their heart septum. The septum is the divider between the left and right chambers of the heart. A septal defect can cause problems with the way blood flows in and out of the baby’s heart. This will usually result in a heart murmur.
Additionally, babies with dextrocardia can also be born without a spleen. The spleen is a major part of the immune system. Without a spleen, your baby is at higher risk for infections throughout the body.
Causes of Dextrocardia
Dextrocardia is caused by genetic changes that occur early in pregnancy. More than 60 genes play a role in the positioning of your organs. Researchers are still searching 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 “carriers,” meaning they have the genetic mutation but do not have symptoms of the condition. If both parents are carriers, the child has a 25% chance of having primary ciliary dyskinesia (with symptoms) and a 50% chance of being a carrier.
Frequency of Occurrence
Dextrocardia affects about 1 in every 12,000 people. Dextrocardia situs inversus totalis affects about 1 in every 10,000 children. Neither gender, race, nor ethnicity have any impact on whether or not a person develops the condition.
Risk Factors
A non-dominant gene (also called autosomal recessive) is the cause of dextrocardia. This abnormal gene causes the primitive tube, or heart, to reverse direction as the fetus develops in the womb.
Depending on the degree and timing of the reversal, the heart and abdominal organs may also develop in an inverted form. Because of the recessive gene that causes dextrocardia, a person must inherit a copy of the abnormal gene from both parents to develop the condition. Therefore, possible risk factors for dextrocardia include a family history of the condition.
Diagnosis of Dextrocardia
Dextrocardia can be diagnosed during pregnancy through a prenatal ultrasound. Afterward, your child will still need imaging tests to confirm the diagnosis.
Some children do not show any symptoms of certain heart defects or genetic syndromes. If this is the case, your child may not be diagnosed with dextrocardia until years later.
A physical exam can also be done to diagnose dextrocardia. A healthcare provider uses a stethoscope to listen to your or your child’s heart. A heartbeat that is visible on the right side of the chest can be a sign of dextrocardia.
Additionally, some other tests that can be used to diagnose dextrocardia include:
- Chest X-ray
- CT scan
- Echocardiogram (echo)
- Electrocardiogram (ECG)
- cardiac MRI
- Your healthcare provider may recommend genetic testing and other tests to check for the syndrome.
Dextrocardia Management
Treatment for dextrocardia depends on what heart defect or syndrome your child has. If your child is diagnosed with dextrocardia without any heart defect, then no special treatment is needed.
It is important for you to share this diagnosis with any health care providers with whom you regularly check your child’s health. This information can help them know during certain tests and procedures.
A person with a heart defect often needs surgery. Such surgery can occur in early infancy or childhood. Before surgery, some babies will need to take medications including:
- Diuretics: “Water pills”, these help remove extra fluid from your baby’s body.
- Inotropic agents, these drugs strengthen the pumping action of your baby’s heart.
- 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 will need depends on their congenital heart defect. Your pediatrician will first discuss treatment options with you.
Possible Complications
Dextrocardia may appear together with or develop into the following complications, including:
Kartagener’s Syndrome
Kartagener’s syndrome is when dextrocardia situs inversus is accompanied by primary ciliary dyskinesia, an inherited condition in which the cilia that help move mucus become immobile. About 20 percent of children with dextrocardia also have Kartagener’s syndrome.
Dextroversion
This condition occurs when the heart is abnormally positioned further to the right and rotated to the right. The right ventricle usually moves back to the left, but the left ventricle remains on the left. This complication can be diagnosed using an electrocardiogram (ECG).
Dextroposition
This occurs when the heart is displaced to the right. Unlike dextrocardia, other organs are not mirrored. Dextroposition is usually associated with acquired disease of the lungs, diaphragm, or pleura (the membrane that surrounds the lungs).
Surgery and muscle damage or deformity can also contribute to the development of dextroposition. Doctors will often use an EKG to differentiate between dextroposition and dextrocardia.
Transposition of the Great Vessels (TGA)
TGA occurs when the main vessels of the heart are connected in reverse due to a reversal of the heart chambers. This condition is quite rare.
Congenital Corrected Transposition of the Great Arteries (CCTGA)
This condition occurs when the bottom of the heart is reversed, specifically the lower ventricle and the valves that connect it. This condition is even rarer than TGA, and the cause is still unknown. According to the Adult Congenital Heart Association, about 0.5 to 1 percent of all babies born with heart problems have CCTGA.
Tricuspid Atresia
This condition is a congenital defect in which the tricuspid valve fails to develop. The tricuspid valve prevents blood from flowing back into the right atrium from the right ventricle.
Single, Double-Outlet, or Double-Inlet Ventricles
These cases occur when the aorta, which normally carries oxygenated blood from the left ventricle to the rest of the body, and the pulmonary artery, which carries deoxygenated blood from the right ventricle to the lungs, are both connected to one (either the left or right) ventricle.
Pulmonary Stenosis or Atresia
This occurs when the heart’s pulmonary (pulmonary) valve that allows blood to leave the heart narrows (stenosis) or fails to develop (atresia).
Heterotaxis
This condition occurs when the mirrored internal organs do not develop or function properly. Depending on the organs involved and the severity of the case, heterotaxis can be life-threatening.
Endocardial Cushion Defect
This is a congenital heart defect in which the tissue that separates the chambers of the heart fails to develop, disrupting blood flow. This condition is also known as an atrioventricular canal defect.
Prevention of Dextrocardia
There is no specific way to prevent dextrocardia in children. This condition is likely caused by genetic changes that occur early in pregnancy.
However, prenatal care can affect the chances of a baby having congenital heart disease. If you are pregnant or planning a pregnancy, here are some steps you can take:
- Quit smoking and avoid drinking alcohol
- Avoid cigarette smoke
- Don’t use drugs
- Manage diabetes if you have it. Diabetes can increase your risk of having a child with dextrocardia.
Talk to 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.
Although there is no specific way you can prevent dextrocardia from occurring in children, maintaining a healthy lifestyle can increase the potential for your baby to be born healthy.
Hopefully this article is useful.
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Republished with permission from theAsianParent Indonesia
