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What is Dextrocardia Situs Inversus?
Written by Medicover Team and Medically Reviewed by Dr G Ravi Kiran , Cardiologist
Dextrocardia with situs inversus is a rare congenital condition where the heart and internal organs are positioned in a mirror image of their standard locations. In this condition:
- Dextrocardia means the heart's apex (tip) points to the right side of the chest instead of the left.
- Situs inversus refers to the reversal of all major organs, such as the liver, stomach, and spleen.
This condition is uncommon, occurring in about 1 in 6,000 to 1 in 35,000 live births. It is typically caused by a genetic mutation, often linked to chromosome 14. Most individuals with dextrocardia situs inversus live healthy lives without symptoms, although some may be born with congenital heart defects or other anatomical anomalies.
In some cases, this condition is associated with primary ciliary dyskinesia (PCD), a disorder affecting the movement of tiny hair-like structures (cilia) in the respiratory tract. When dextrocardia occurs alongside chronic sinus infections and bronchiectasis, it may indicate Kartagener syndrome, a subtype of PCD.
What Are The Symptoms of Dextrocardia Situs Inversus?
Some individuals who are affected may exhibit no apparent signs or symptoms. Others may experience the following signs and symptoms of dextrocardia situs inversus, such as:
- Breathing difficulty
- Cyanosis (bluish skin discoloration due to insufficient oxygen in the blood)
- Frequent sinus or lung infections
- Pallor (very pale skin)
- Jaundice (yellow skin and eyes)
- Fatigue, especially when feeding
- Failure to grow and gain weight.
- Doctors may discover that the tip of the heart is pointing towards the right side of the chest rather than the left.
- Congenital heart defects, most commonly transposition of the great vessels, affect a small percentage of the population.
- The organs in the chest cavity and abdomen are reversed in mirror image.
What Are the Common Causes of Dextrocardia Situs Inversus?
Dextrocardia with situs inversus occurs when the internal organs, including the heart, form in reversed positions during early fetal development. Although rare, several genetic and developmental factors can lead to this condition.
Dextrocardia Situs Inversus Causes
- Genetic Mutations: Most cases result from mutations in genes responsible for the body's left-right orientation during embryonic development.
- Abnormal Organ Rotation: During fetal development, organs may rotate incorrectly, resulting in a mirrored placement of organs.
- Primary Ciliary Dyskinesia (PCD): In some individuals, dextrocardia is linked to PCD, a rare inherited disorder that affects ciliary function, often resulting in Kartagener syndrome.
- Sporadic Developmental Error: In some cases, the cause is unclear, likely due to developmental disruptions during pregnancy.
Risk Factors of Dextrocardia Situs Inversus
- Family History: A parent carrying the mutated gene (even without symptoms) may pass it on to their child.
- Genetic Syndromes: The Presence of associated genetic syndromes, such as Kartagener syndrome or other ciliopathies, increases the risk.
- Consanguinity: Marriages among close relatives can slightly raise the risk of inherited congenital conditions.
- No Link to Gender or Ethnicity: The condition affects both males and females equally and is not influenced by race or ethnicity.
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Get Second OpinionHow Is Dextrocardia Situs Inversus Diagnosed?
Diagnosing dextrocardia with situs inversus often happens incidentally during tests for unrelated health issues, as many individuals show no symptoms.
However, if signs like unusual heart sounds or recurrent respiratory infections appear, doctors may recommend specific tests.
Diagnostic Methods:
- Physical Examination: A doctor may notice that heart sounds are heard more clearly on the right side of the chest, raising suspicion of dextrocardia.
- Chest X-ray: Often, the first imaging test to visualise the heart is a chest X-ray, which typically shows the heart located on the right side of the chest instead of the left.
- Echocardiogram (Echo): This ultrasound test assesses the heart's structure and function. It helps confirm reversed positioning and check for related heart defects.
- Electrocardiogram (ECG or EKG): This test may show an unusual pattern because the heart's electrical signals behave differently when it's positioned on the right.
- Abdominal Ultrasound or CT/MRI Scan: Imaging tests of the abdomen help determine whether abdominal organs, such as the liver, stomach, and spleen, are also reversed (situs inversus totalis).
- Genetic Testing: If a hereditary condition, such as Kartagener syndrome or primary ciliary dyskinesia (PCD), is suspected, genetic screening may be performed to identify mutations.
- Pulmonary Function Tests (if respiratory symptoms are present): These tests are used to evaluate lung health in cases where chronic sinusitis or bronchiectasis are present.
What are the Treatment Options for Dextrocardia Situs Inversus?
Treatment varies based on any heart or physical issues, as well as dextrocardia and situs inversus.
- Surgery for Congenital Heart Defects: Babies with heart defects or other organ problems might need surgery.
- Management of Kartagener Syndrome: People with Kartagener syndrome receive care to clear mucus, prevent infections, and treat bacterial infections.
Treatment for Associated Conditions
- Heart Medications: Medicines to help the heart pump blood better.
- Antibiotics for Infections: Drugs to manage respiratory infections.
- Fluid Removal Medications: Medications to remove fluid from the lungs.
- Pacemakers for Heart Issues: Devices used to treat specific heart rhythm problems.
- Surgery for Organ Problems: Surgical intervention for congenital heart issues or other chest and abdominal organ problems.
When to See a Doctor for Dextrocardia Situs Inversus?
You should consult a doctor if:
- Your child is diagnosed with dextrocardia or situs inversus during a routine scan or check-up.
- There are symptoms like:
- Frequent lung or sinus infections
- Breathing difficulties
- Poor growth in infants
- Unusual heart sounds (heard on the right side)
- Fatigue or bluish skin (cyanosis) in babies or children
- You or your child has been diagnosed with primary ciliary dyskinesia or Kartagener syndrome
- You're planning surgery or a medical procedure and have a known or suspected organ reversal.
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Dos and Don'ts
Follow dos and don'ts for dextrocardia situs inversus to prevent the symptoms and its complications.
This condition requires proper treatment and a set of do's and don'ts to be followed to manage dextrocardia situs inversus. During the treatment and even after the treatment, one need to follow these:
|
Do's |
Don'ts |
|
Take enough sleep for at least 7 to 9 hours. |
Eat junk, oily or deep fried foods. |
|
Eat healthy food and follow a proper diet. |
Forget to take medications. |
|
Control your blood pressure levels |
Forget to go for your routine checkups. |
|
Manage your stress. |
Ignore signs and symptoms. |
|
Take precautions to avoid any kind of infection. |
Do smoke or consume alcohol. |
|
Regular exercise or yoga |
Let your cholesterol levels increase |
Our Experience with Dextrocardia Situs Inversus at Medicover Hospitals
At Medicover Hospitals, we specialise in diagnosing and treating rare conditions, such as dextrocardia with situs inversus. Our team of experts, including cardiologists and genetic specialists, offers accurate diagnoses, individualised care, and ongoing support. We focus on early detection, managing related conditions, and helping patients lead healthy lives with confidence.
