Dextrocardia is a condition where your heart, wayward thing that it is, faces the right, instead of the left side of your body…Go figure.
It is a rare, congenital condition (present at birth) and usually not life-threatening.
However, it shows up along with more serious complications such as heart defects and disorders of the organs in the abdomen.
Finding Dextrocardia without any other heart defects is unusual.
There are two major types of Dextrocardia:
1. Dextrocardia Situs Inversus
In this type of Dextrocardia, the tip of the heart and its four chambers are pointing towards the right side of the body instead of the left, as they should be.
Also known as Isolated Dextrocardia (which means without any other associated heart defects), it is a rare condition and happens with equal frequency in men and women.
2. Dextrocardia With Situs Inversus Totalis, which is a term used to describe the inverted position of the chest and abdominal organs means that there is a total reversal or mirror image of the normal position of the internal organs.
This condition affects about 1 out of every 10,000 children.
No specific gender, race or ethnicity seem to have any impact on whether or not a person develops them.
Causes and Risk Factors
As mentioned above, these conditions are present at birth and are caused by abnormal genes, specifically non-dominant genes, also called “autosomal recessive” genes. That means that two copies of the abnormal gene must be present in order for the disease or trait to develop.
Depending on the extent and timing of the reversal, the heart and abdominal organs may also develop in a reversed form.
A person must inherit a copy of the abnormal gene from both parents to develop the condition.
Many people with Congenital Dextrocardia don’t know they have it, and the condition may not produce many symptoms.
However, one telltale symptom of dextrocardia is that people with this condition have most heart sounds on the right side of the chest instead of the left.
In severe cases, typically in infants with other heart defects or another disease, certain symptoms need medical treatment.
The symptoms may include:
- Unexplained and continual exhaustion
- Inability to gain weight
- Chronic infections, especially of the sinus and lungs
- Difficulty breathing
- Jaundiced or yellowed skin
- Blue-tinted skin, especially around the fingers and toes
Although the reversed organs themselves may function normally, their irregular positioning often makes the diagnosis of other conditions tricky.
For example, in someone with dextrocardia situs inversus, appendicitis will cause sharp pain in the lower left part of the abdomen instead of the right.
When these anatomical differences occur, they can also make surgery difficult.
Other complications associated with dextrocardia may include:
- Bowel disorders, usually from obstruction due to the reversal
- Bronchial diseases, like chronic pneumonia
- Esophageal disorders
- Cardiovascular disorders
- Heart failure or Heart Disease
- Infection and sepsis*
*Sepsis (blood poisoning) is commonly caused by a bacterial infection in the blood (called septicemia).
In other words, septicemia leads to the state of sepsis.
- Poisons are released by the bacteria involved in septicemia
- The immune system mounts a massive inflammatory response to these poisons – this is called sepsis
Most cases of Dextrocardia are diagnosed using an electrocardiogram (EKG) and a chest X-ray.
- An EKG that shows inverted or reversed electrical waves usually points to dextrocardia.
Once a doctor suspects this condition, they may use a CT scan or an MRI scan to confirm it.
Dextrocardia may be caused by, associated with, or related to other conditions such as:
- Dextroposition: This happens when only the heart is moved to the right. The other organs are not mirrored.
It’s usually associated with acquired diseases of the lungs, diaphragm, or pleura (the membrane surrounding the lungs).
Surgeries, muscle damage or deformities may also contribute to its development.
This condition happens when the mirrored internal organs do not develop or function properly.
- Depending on the organs involved and the severity of the case, it can be life-threatening.
Many people with dextrocardia don’t even display symptoms, so the condition can go untreated.
- Infants with dextrocardia, which is accompanied by heart defects, may need surgery.
- Many children will be given medication that increases the force of the heartbeat and lowers their blood pressure before surgery.
However, Dextrocardia must be treated if it prevents vital organs from functioning properly.
- Pacemakers and surgery to repair septal defects can help the heart work normally.
The person with this condition may have more infections than the average. Medications can cut the risk of infection.
- If the person doesn’t have a spleen or it doesn’t work properly, the doctor will prescribe antibiotics to prevent infection. They may need to be taken over the long-term to fight off respiratory illness.
The heart pointing toward your right side makes blockages in the digestive system more likely. This is because dextrocardia can sometimes result in a condition called intestinal malrotation, in which the gut doesn’t develop correctly.
For that reason, the doctor will watch out for an abdominal obstruction, also called bowel or intestinal obstruction because an obstruction prevents waste from leaving the body.
- Intestinal obstruction is dangerous, and if it’s not treated, it can be life-threatening. Surgery may be required to correct it.
Genetic counseling is available and may be helpful for adults with dextrocardia who are looking to start a family.
- For most people, life expectancy is normal.
In cases of Isolated Dextrocardia (when only the chambers of the heart are reversed), however, congenital heart defects are more frequent and are associated with greater health risks.