What is Dextrocardia?
Dextrocardia is a rare condition that a person is born with, where the heart is located on the right side of the chest instead of the left (as can be seen in a chest X-ray). Often, it’s found by accident while diagnosing other conditions. It can occur on its own, or along with a complete reversal of other organs’ position, a condition known as situs inversus totalis.
In people with dextrocardia, the heart’s orientation shifts too – instead of the bottom pointing towards the left as in a normal heart, it points towards the right. This is different from cardiac dextroposition, where the heart is pushed to the right side because of other factors such as a hernia or lung problems.
In some dextrocardia cases, the inner layout of the heart is also flipped, a condition referred to as situs inversus (as seen in a 56-year-old woman with left side pain). But when dextrocardia comes with the other organs in their normal positions, it’s called situs solitus.
What Causes Dextrocardia?
The exact cause of dextrocardia, a condition where the heart is on the right side of the chest instead of the left, is not known. But, it is believed to occur due to the abnormal positioning of organs when the baby is developing inside the mother’s womb.
Kartagener syndrome, a disease passed down through families, is associated with dextrocardia. People suffering from this syndrome have defective cilia, tiny hair-like structures that help move mucus out of the body. This condition leads to chronic sinusitis, bronchiectasis (a lung condition that causes cough and shortness of breath) alongside dextrocardia.
Another inherited disorder called heterotaxy can also result in dextrocardia. Heterotaxy happens when the organs in the chest and abdomen end up in abnormal positions during the baby’s development in the womb.
People with heterotaxy may experience various other birth defects involving their abdominal organs. For instance, they may have an unusual rotation of the intestine, abnormal blood vessels, unusual connections between the lungs and the blood vessels, and lack of a spleen.
Risk Factors and Frequency for Dextrocardia
Dextrocardia is a highly uncommon condition, seen in about 1 out of 12,000 pregnancies. Another related condition, Kartagener syndrome, occurs in approximately 1 out of 30,000 live births. Half of all patients with a condition known as primary ciliary dyskinesia also have a condition called situs inversus totalis. Dextrocardia without any other associated conditions is seen in just 0.6% of patients with primary ciliary dyskinesia. There are no known differences in the occurrence of dextrocardia related to ethnicity or gender.
- Dextrocardia is uncommon, occurring in about 1 in 12,000 pregnancies.
- Kartagener syndrome is seen in about 1 in 30,000 live births.
- 50% of patients with primary ciliary dyskinesia have a condition called situs inversus totalis.
- Only 0.6% of patients with primary ciliary dyskinesia have dextrocardia without other associated conditions.
- There’s no tendency for dextrocardia to affect certain genders or ethnicities more than others.
Signs and Symptoms of Dextrocardia
Dextrocardia is a condition where the heart is positioned on the right side of the chest instead of the left. There are different types of dextrocardia. “Isolated dextrocardia” means the heart is on the right side, but everything else is normal, so there are usually no symptoms. However, with “Dextrocardia with situs inversus” and “Kartagener syndrome,” more body parts are in reversed positions, leading to various symptoms. The symptoms may include:
- Bluish skin color
- Difficulty breathing
- Failure to grow and develop properly
- Feeling tired
- Yellow skin and eyes
- Pale skin
- Difficulty with physical activity
- Frequent sinus or lung infections
- Water on the brain
- Heart rhythm problems
- Blockages in the intestines
When a doctor examines a patient with dextrocardia, they might notice bluish skin color, changes in the fingers due to heart disease, a strong heartbeat felt on the right side of the chest, and heart sounds that are easier to hear on the right side of the chest.
Testing for Dextrocardia
Dextrocardia, a condition where the heart is situated on the right side of the body, is often identified by chance during usual imaging procedures that show the heart in an unusual location. An Electrocardiogram (EKG) could also point towards dextrocardia as it might present with characteristic changes like the rightward shift of heart’s electrical activity, inverted reading in lead I, peculiar P-wave in augmented vector left, and lack of normal R-wave progression in the anterior leads. One must note that similar EKG findings might appear due to limb reversal, but the precordial leads continue to show a normal pattern.
Techniques like an echocardiogram, a CT scan of the chest or an MRI of the chest can all be instrumental in evaluating dextrocardia and any associated abnormalities.
For diagnosing Kartagener syndrome, which is a specific type of dextrocardia, doctors usually begin with screening tests like nasal brush biopsy and measurements of nasal nitric oxide. A CT scan of the chest might reveal widened airway passages (bronchiectasis) along with dextrocardia in these patients. To confirm the diagnosis of primary ciliary dyskinesia, part of Kartagener syndrome, other available options include genetic testing and electron microscopy.
Treatment Options for Dextrocardia
Most people with a medical condition known as dextrocardia, where the heart is on the right side of the chest instead of the left, don’t show any symptoms and live a normal life. However, the way dextrocardia is handled can differ based on whether the person has any other birth defects.
If patients have additional heart abnormalities, like defects in the heart walls, incorrectly positioned blood vessels, or serious valve issues, they might need to undergo surgery to rebuild the affected parts of the heart.
Patients with issues in the heart’s electrical system, which can cause irregular heartbeats, might need a pacemaker, a device that helps the heart beat more regularly. Additionally, while there isn’t a current cure for a condition known as Primary Ciliary Dyskinesia (PCD) that some patients with dextrocardia might also have, doctors may recommend certain medications to help clear up airway secretions.
Antibiotics might be needed to fight off respiratory infections, particularly in the case of patients also having asplenia, a condition wherein the spleen is absent. If a particular lung condition known as bronchiectasis severely affects a patient’s quality of life, lung surgery could be an option. Newborns may sometimes experience intestinal blockages which would require surgical intervention to prevent serious complications.
Patients with dextrocardia and other complex heart abnormalities might find that a heart transplant is necessary. This procedure involves constructing new paths for the blood vessels in order to accommodate a regular donor heart.
What else can Dextrocardia be?
When a person’s heart is located on the right side of their chest instead of the left, it could be due to different reasons. Healthcare providers consider several possible conditions:
- Cardiac dextroposition: This is when the heart is pushed to the right side because of issues outside of the heart, such as a hernia in the diaphragm, removal of the right lung, or undeveloped right lung.
- Kartagener syndrome: This is a condition where the heart is situated on the right side, and there’s also an issue with cilia, the tiny hair-like structures in our body.
- Dextroversion: In this case, the heart is in an abnormal position towards the right and is also rotated.
- Transposition of the great vessels: This condition occurs when the major blood vessels in the heart connect in reverse, resulting in a switch of the heart’s chambers.
- Heterotaxy: A condition where internal organs are mirrored but may not grow or function as expected.
- Endocardial cushion defect: This is a heart defect present at birth, resulting in abnormal development of some of the heart’s structures.
Given these possibilities, healthcare providers will conduct tests to determine the right diagnosis for why the heart might be located on the right side.
What to expect with Dextrocardia
The future health outcomes for people with dextrocardia – a condition where the heart is positioned on the right side of the chest instead of the left – are largely impacted by whether or not they have other birth defects and what those defects entail.
It’s worth noting that those with dextrocardia but no other abnormalities can expect to live a normal, complication-free life. However, newborns with dextrocardia and without a spleen are more prone to bacterial infections, especially those caused by bacteria like Streptococcus pneumoniae, Haemophilus influenzae, and Meningococcus. These infections can become severe and life-threatening. As such, these particular patients may require preventative antibiotics as part of their ongoing care.
Possible Complications When Diagnosed with Dextrocardia
Dextrocardia, a condition where the heart points towards the right side of the chest instead of the left, can lead to several complications. These include:
- Failure to thrive, meaning the body doesn’t grow as it should
- Respiratory failure, where the lungs can’t provide the body with enough oxygen
- Congestive heart failure, which is a condition wherein the heart can’t pump blood effectively
- Severe infections, particularly from specific bacteria that have a protective coating
- Infertility in men with Kartagener syndrome, a specific complication of dextrocardia
- Regular sinusitis and pneumonia
- Intestinal obstruction caused by malrotation, which is a problem with the way the intestines are positioned
Preventing Dextrocardia
Dextrocardia is a health condition where the heart is located on the right side of the chest, instead of the left. On its own, this condition is usually harmless. However, it’s often linked with other birth defects that can cause a variety of issues. These issues include growth problems, frequent infections, and breathing difficulties in newborns and babies. Adults with dextrocardia might also struggle with infertility. If doctors suspect someone has this condition, they will typically perform a thorough check-up, including imaging scans and genetic testing, to check for any other related abnormalities.
