What Is Dextrocardia?

Overview

Dextrocardia is a rare congenital condition characterised by the abnormal positioning of the heart within the chest. In individuals with dextrocardia, the heart is located on the right side of the chest instead of the normal left side. This reversal of heart orientation is present from birth and occurs during fetal development.

Types of dextrocardia

There are two main types of dextrocardia:

Dextrocardia with situs solitus

In this type of dextrocardia, often referred to as ‘isolated’ or ‘simple’ dextrocardia, the heart is the only organ with an abnormal anatomical position and other internal organs take their normal positions just like an individual without dextrocardia. Isolated dextrocardia can be further classified based on the orientation of the heart apex (either right- or left-sided).

Dextrocardia with situs inversus

In situs inversus, the internal organs are completely reversed or mirrored. For example, the heart is on the right side, the liver is on the left side, and all other internal organs have a mirrored position. Despite this reversal, the organs typically function normally and individuals with situs inversus usually have no significant health problems directly related to the condition.

Causes of dextrocardia

The exact cause of dextrocardia is not fully understood. It is considered a congenital condition, meaning it is present from birth and occurs during the early stages of foetal development. 

Several factors may contribute to the development of dextrocardia, including genetic and environmental factors. In some cases, dextrocardia can be inherited as an autosomal recessive trait, which means that both parents carry a mutated gene and pass it on to their children. However, dextrocardia can also occur sporadically, without a clear genetic link.

During embryonic development, the heart normally starts to form before rotating to its final position on the left side of the chest. In dextrocardia, this rotation process is disrupted, resulting in the heart being positioned on the right side. The exact cause of this disruption is not fully understood but is believed to involve abnormal development of structures in the early embryo.

It's worth mentioning that dextrocardia can be associated with other congenital conditions or syndromes that affect the development of multiple organs. These conditions may have a known genetic basis, such as Kartagener syndrome, which is characterised by dextrocardia, situs inversus (reversed organ positioning), and chronic respiratory infections.1 In these cases, the underlying genetic mutation is responsible for both dextrocardia and the associated condition.

Signs and symptoms of dextrocardia

In many cases, dextrocardia itself does not cause any noticeable signs or symptoms. The condition is often discovered incidentally during medical imaging for other reasons or when investigating potential heart or chest abnormalities. However, depending on the presence of associated conditions or heart defects, some individuals with dextrocardia may experience certain signs or symptoms.1 These can include:

  1. Murmurs
    Some individuals with dextrocardia may have heart murmurs, which are abnormal sounds heard during a heartbeat. Murmurs can be caused by underlying heart defects or abnormalities.
  1. Congenital heart defects
    Dextrocardia can be associated with other heart abnormalities, such as atrial septal defects (ASDs), ventricular septal defects (VSDs), or abnormalities of the heart valves. These conditions can lead to symptoms like fatigue, shortness of breath, poor growth, or cyanosis (bluish discolouration of the skin).
  1. Respiratory problems
    Individuals with dextrocardia may be more prone to respiratory issues, especially if they have associated conditions like Kartagener syndrome. (Kartagener syndrome is characterised by dextrocardia, situs inversus and chronic respiratory infections. Symptoms can include chronic cough, recurrent pneumonia and sinus infections.)
  1. Arrhythmias
    Some individuals with dextrocardia may be at a slightly higher risk of certain arrhythmias (irregular heart rhythms) due to associated heart defects or abnormal electrical pathways within the heart.

It's important to note that the absence of symptoms does not necessarily indicate a lack of associated conditions or heart defects. Regular medical follow-ups and evaluations are necessary to monitor the health of individuals with dextrocardia and detect any potential complications. 

Management and treatment for dextrocardia

The management and treatment of dextrocardia depend on the presence of associated conditions or heart defects, as well as any symptoms or complications that may arise. Here are some considerations:

  1. Regular medical follow-up
    Individuals with dextrocardia should have regular check-ups with a healthcare professional to monitor their heart health and overall well-being. This may involve periodic physical examinations, electrocardiograms (ECGs), echocardiograms, and other imaging tests to assess the heart's structure and function.
  1. Treatment of associated conditions
    If there are other heart defects or conditions present, appropriate treatment measures will be determined based on the specific diagnosis. This may involve medication, surgical interventions, or other procedures to manage or correct the issues.
  1. Arrhythmia management
    If arrhythmias are present, they may require treatment, such as medications to regulate the heart rhythm, catheter ablation to eliminate abnormal electrical pathways, or the implantation of a pacemaker if necessary. 
  1. Respiratory care
    If dextrocardia is associated with respiratory problems, such as Kartagener syndrome, proper management of respiratory symptoms and infections will be necessary. This may involve regular monitoring, antibiotics for infections, respiratory therapies, and lifestyle measures to promote respiratory health.
  1. Lifestyle considerations
    In general, individuals with dextrocardia are encouraged to maintain a healthy lifestyle, including regular exercise, a balanced diet, and avoiding factors that may put additional strain on the heart, such as smoking or excessive alcohol consumption.

It's important to note that the management and treatment of dextrocardia are highly individualised, and the approach will depend on the specific characteristics and associated conditions in each case. Therefore, it's crucial to consult with a healthcare professional who can provide personalised guidance and recommendations based on your situation.

Diagnosis

Dextrocardia is typically diagnosed through various medical imaging techniques that allow visualisation of the heart and its position within the chest cavity. The diagnostic process may involve the following:

  1. Physical examination
    During a routine physical examination, a healthcare professional may detect abnormal heart sounds or other signs that could suggest dextrocardia. However, a physical exam alone is not sufficient to confirm the diagnosis.
  1. Chest X-ray
    A chest X-ray can provide an initial indication of dextrocardia. It can show the position of the heart within the chest and whether it is located on the right side. However, further diagnostic tests are usually needed to confirm the diagnosis and determine the specific type of dextrocardia.
  1. Echocardiogram
    An echocardiogram is a commonly used imaging test that uses sound waves to create detailed images of the heart's structure and function. It can provide information about the heart's orientation and any associated heart defects or abnormalities.
  1. CT scan or MRI
    In some cases, a computed tomography (CT) scan or magnetic resonance imaging (MRI) may be performed. These imaging techniques can provide more detailed and three-dimensional images of the heart and chest cavity, helping to confirm the diagnosis of dextrocardia and assess any associated conditions or organ abnormalities.

Complications

Dextrocardia itself is not typically associated with complications. However, depending on the specific type of dextrocardia and the presence of associated conditions, individuals with dextrocardia may be at an increased risk of certain complications. These can include:

  1. Congenital heart defects
    Dextrocardia can occur alongside other congenital heart defects, such as atrial septal defects (ASDs), ventricular septal defects (VSDs), or abnormalities of the heart valves. These defects can disrupt the normal flow of blood through the heart and may require medical or surgical intervention to correct.
  1. Arrhythmias
    Some individuals with dextrocardia may have an increased risk of arrhythmias, which are irregular heart rhythms. This can be due to underlying heart defects or abnormal electrical pathways within the heart. Arrhythmias may cause symptoms like palpitations, dizziness, fainting or shortness of breath.
  1. Respiratory problems
    Dextrocardia can be associated with respiratory issues, particularly in cases of Kartagener syndrome. Kartagener syndrome is characterised by dextrocardia, situs inversus (reversed organ positioning), and chronic respiratory infections. Respiratory problems can include chronic cough, recurrent pneumonia, bronchiectasis, and sinus infections.
  1. Pregnancy complications
    If you have dextrocardia, pregnancy may pose some additional risks due to the altered position of the heart and potential associated heart defects. It is important for pregnant people with dextrocardia to receive specialised prenatal care and close monitoring throughout pregnancy to ensure the well-being of themselves and the baby.

It's important to note that not all individuals with dextrocardia will experience complications or health problems. The specific risks and complications vary depending on the individual case, associated conditions, and other factors. 

FAQs

How can I prevent dextrocardia?

Dextrocardia is typically a congenital condition, and there are no known prevention strategies. It is important to maintain a healthy lifestyle during pregnancy, including regular prenatal care and avoiding substances that can harm foetal development.

How common is dextrocardia?

Dextrocardia is a relatively rare condition. Isolated dextrocardia occurs in about 1 in 12,000 live births, while situs inversus occurs in approximately 1 in 10,000 to 1 in 20,000 individuals.2

Who is at risk of dextrocardia?

Dextrocardia can occur in individuals with no family history of the condition. However, there may be a genetic component, and it is more common in families with a history of congenital heart defects or situs inversus.

When should I see a doctor?

If you have concerns about the position of your heart or suspect dextrocardia, it is advisable to consult a healthcare professional. They can perform the necessary evaluations and diagnostic tests to confirm the condition and provide appropriate guidance.

Summary

Dextrocardia is a rare condition in which the heart is located on the right side of the chest instead of the usual left side. It can be classified into isolated dextrocardia, where only the heart is reversed, and situs inversus, where all the internal organs are mirrored. The exact cause of dextrocardia is often unknown but is believed to be a congenital condition that occurs during foetal development. 

Symptoms and complications can vary depending on the type of dextrocardia, but some individuals may have no symptoms or complications at all. Diagnosis is typically made through imaging tests, and treatment is usually not required unless there are associated heart defects or other medical conditions. 

Overall, dextrocardia is a rare condition that may require special considerations in medical evaluations and procedures due to the reversed anatomy.

References

  1. Nair R, Muthukuru SR. Dextrocardia. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 [cited 2023 Jul 28]. Available from: http://www.ncbi.nlm.nih.gov/books/NBK556074/ 
  2. Mishra M, Kumar N, Jaiswal A, Verma AK, Kant S. Kartagener’s syndrome: A case series. Lung India [Internet]. 2012 Dec [cited 2023 Jul 28];29(4):366. Available from: https://journals.lww.com/lungindia/Fulltext/2012/29040/Kartagener_s_syndrome__A_case_series.13.aspx 
This content is purely informational and isn’t medical guidance. It shouldn’t replace professional medical counsel. Always consult your physician regarding treatment risks and benefits. See our editorial standards for more details.

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Anna-Sophie Fellows

Incoming Medicine Undergraduate

Anna is a student who joined Klarity during her gap year. Having finished her A-Levels in Biology, Chemistry and German, she currently holds an unconditional offer to study Medicine at University. She has previous experience researching, writing and editing articles for newsletters within her school and when completing an Extended Project Qualification (EPQ) in medical ethics. She is passionate about medical education and increasing participation in research.

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