Dextrocardia

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What is dextrocardia?

Dextrocardia is a birth defect in which the tip (apex) of the heart points to the right side. The apex of the normal heart points to the left side.

Is this condition a medical emergency?

Dextrocardia is not a medical emergency.

Types of this condition

Dextrocardia types are as follows:

A. Isolated dextrocardia/dextrocardia of embryonic arrest/dextrocardia with situs solitus: Only the heart is affected with the tip of the heart pointing to the right side while the rest of the organs are in their normal anatomical positions.

B. Dextrocardia situs inversus: The heart is a mirror image of a normal heart and is situated on the right side.

C. Situs inversus may be further classified as:

  • Situs inversus with levocardia: The anatomical position of the organs is reversed but the apex of the heart points to the left side.
  • Situs inversus with dextrocardia: It is the same as dextrocardia situs inversus totalis.

D. Dextrocardia situs inversus totalis: The heart is a mirror image of a normal heart and is situated on the right side and other organs such as the liver, gall bladder, spleen, etc., are also on the opposite side of their normal anatomical position.

Some other conditions in which Dextrocardia may be present include:

A. Kartagener syndrome: It is a syndrome associated with dextrocardia in which there is an absence of cilia (fine hair-like cells) lining the various organs especially the bronchi

B. Heterotaxy: It is a condition that presents with abnormalities of the heart and other organs in the abdomen and the chest. Abnormalities of the other organs may include an absence of the spleen, abnormal gall bladder system, abnormalities of structure and position of the intestines, abnormal development of the lungs, in addition to severe heart defects.

Dextrocardia with situs inversus may be associated with other heart defects such as:

  • Double outlet right ventricle: The connection of the aorta is with the right ventricle instead of the left.
  • Endocardial cushion defect: The separation of the chambers of the heart is poorly defined.
  • Pulmonary stenosis or atresia: The pulmonary valve is either extremely narrow or not formed.
  • Single ventricle: The wall separating the ventricles is missing, and there is only a single lower heart chamber instead of two.
  • Transposition of the great vessels: The position of the aorta and the pulmonary vessels is switched from their normal positions.
  • Ventricular septal defect: There is a defect in the wall that separates the left and the right lower chamber of the heart.

What causes dextrocardia?

The exact cause of dextrocardia with situs inversus or situs solitus is not fully understood, but it a developmental defect that occurs when the primitive loop in the embryo moves in the reverse direction causing the displacement of the organs. This development defect can be due to genetic defects with or without primary ciliary diskinesia.

Risk factors

The risk of Dextrocardia is more if there is a family history of the condition.

Dextrocardia symptoms and signs

Most individuals with situs inversus do not show any symptoms or signs and lead a normal life. Symptoms and signs if at all present may include:

  • Increased incidence of lung infections
  • The following may be seen in pediatric dextrocardia
    • Growth and developmental delays
    • Breathing difficulties
    • Blue skin or lips
    • Jaundice
    • Fatigue and tiredness

Investigations

Some investigations that may be done to establish a diagnosis of dextrocardia may include:

  • Imaging tests:
    • Radiography: Dextrocardia chest X-ray shows the heart tip pointing to the right side instead of the left, the position of the bronchi is also reversed in Dextrocardia situs inversus, additionally, if abdominal radiography is done then the C loop of the duodenum, the liver, and the spleen may also be seen on the opposite side.
    • CT scanning: The heart and the major vessels are seen as a mirror image of their normal anatomy.
    • MRI: It is useful for visualizing the upper chamber inversion in dextrocardia situs inversus;
    • Echocardiography: It is useful in visualizing the upper chambers of the heart; dextrocardia echocardiography shows the connections of the major vessels to the upper chambers. Fetal ultrasound is helpful in the identification of Dextrocardia situs inversus prenatally.
    • Angiography: It is usually done when other congenital heart disease in addition to dextrocardia situs inversus need to be identified.
  • Electrocardiography(ECG): Dextrocardia ECG findings usually show low voltages in some leads as they are placed opposite on the wrong side when compared to a regular heart; in addition ECG for dextrocardia also shows negative P waves, QRS complex, and T waves in some leads.

How is dextrocardia diagnosis done

A diagnosis of Dextrocardia is established based on medical history, clinical evaluation, and results of the investigations done. Imaging tests are the essential tools for establishing adult and pediatric dextrocardia situs inversus and fetal ultrasound can help to identify Dextrocardia prenatally.

Dextrocardia treatment options

Most people with Dextrocardia do not exhibit any symptoms and lead a normal life. Symptomatic individuals in whom Dextrocardia is associated with other defects related to the heart or other organs may require symptomatic and supportive management or surgical intervention and dextrocardia treatment is only done in such individuals.

Medical management

Medical management of situs inversus may include the following:

  • Antibiotics: For the management of infections
  • Diuretics: For the management of fluids and reduce the load on the heart
  • Inotropic Agents: To help heart muscle contraction
  • ACE Inhibitors: For decreasing blood pressure and also the workload of the heart

Interventional including surgery and indications for surgery

Interventional management of dextrocardia is limited to individuals with other symptomatic defects that required intervention. Interventions may include:

  • Open surgical procedures for the management of heart defects
  • Minimally invasive surgical procedures for the management of heart defects

Role of diet/exercise/lifestyle changes/preventive

Some measures that may be taken by people with dextrocardia may include

  • Exercising and maintaining a healthy lifestyle
  • Eating a healthy diet and maintaining an ideal body weight
  • Practicing good hand and food hygiene to prevent infections

Complications

Some Dextrocardia complications that may be seen include:

  • Intestinal obstructions
  • Increased risk of severe infections
  • Heart arrhythmias
  • Myocardial infarction
  • Congestive heart failure

Prognosis

The prognosis for Dextrocardia is generally good although dependent on its type as well as the presence of abnormalities of the heart and other organs.

When to contact the doctor or hospital/how to identify the emergency or complications

It is advisable to seek medical attention if the symptoms and signs of Dextrocardia or associated heart defects are noticed.

Indications for hospitalization if required

Hospitalization is not required for dextrocardia, but it may be needed if surgical intervention is required for its management.

Screening methods

Screening and genetic counseling are advisable in individuals with a family history of dextrocardia.

Suggested clinical specialist/departments to consult for this condition

Dextrocardia will be attended by specialists from the department of cardiology.

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