Dextrocardia

Author Ольга Кияница

2018-03-06

In normal condition, the heart is located more on the left side of the human body, with the right one. But in a comparatively small number of people, approximately 0.01% of the population, dextrocardia is determined, when the heart is located on the right side more than with the left. This congenital anomaly often does not violate the normal life of a person, although in some cases a special treatment is performed.

Dexcurrent (DK) is a condition in which the heart is on the right side of the chest. Usually the heart is on the left. The condition is determined immediately after the birth of the child, so it is considered a congenital developmental anomaly. Typically, the pathology is not life-threatening, although it is often encountered along with more serious complications such as heart defects and disturbance of the organs in the abdominal cavity.

The term "dextrocaria" is similar to the Latin dexter, which means "right", that is, the right, and the Greek kardia, which means "heart".It is considered a rare congenital disease.

Non-perpendicular to other defects in the development of dextrocaria can in no way manifest itself in the quality of life of the patient. Frequently determined by accidental medical examination. If, however, the heart is shifted to the right due to the development of a pathological process in the thoracic cavity (tumor growth, formation of the hydrothorax), then this is not true dextrocaria, but pathological dextroposing of the heart.

Video Mirror bodies

History and statistics

The dextrocard was first described by the Italian, Jerome Fabrice, who became famous as an anatomist and surgeon. The description of the disease was carried out in 1606. After 37 years it was repeatedly described and this time an anomaly became interested in the whole world. Marco Aurelio Severino managed to achieve this.

  • To date, dextrocaria is encountered quite rarely, with an incidence of approximately 8-25,000 in frequency newborns
  • Complete dextrocardia occurs in 1 case out of every 10,000.
  • When comparing all anomalies of the cardiovascular system, the heart is located to the right of about 3% of people.

Bez-soputstvuyushhih

Reasons

It is believed that non-dominant (so-called autosomal-recessive) genes cause dextrocaria.While the fetus develops in the uterus, these abnormal genes make the primary or heart tube move in the opposite direction.Depending on the degree and time of reversal, the heart and organs of the abdominal cavity may also develop in reverse.

These autosomal recessive genes, which cause dextrocaria, must inherit from both parents. If the anomalous genes have only one parent, then the disease does not manifest.

It is worth noting that gender, race and ethnicity, apparently, do not affect the development of a person's pathological condition.

Species

There are several forms of dextrocaria:

  • Uninsulated (situs viscerum inversus totalis) - with this pathology, all internal organs are placed transpositively, that is, back to normal state.

Mirrored_heart_and_lungs

  • Isolated - with this anomaly, odd organs (the stomach, liver, spleen) are located normally.Depending on the state of the heart chambers, this form is divided into several subspecies:
    • With inversion of the ventricles and atrium.
    • Without inversion of the atrium and ventricles.

In the latter case, that is, in the presence of an isolated dextrocardia without inversion of the atria and ventricles, synonyms are used in the form of dehydrotensification, dextroporation, axial dextrocaria.

An isolated form is rare.According to Korth and Schmidt (S. Korth, J. Schmidt, 1955) - in 12 cases out of 1000. According to Bohun et al.(2007) the frequency of dextrocaria is 1 case per 12 thousand.

Bohun CM, Potts JE, Casey BM, Sandor GG (July 2007)

Dexcurrent can be combined with other anomalies of development.

Cardagene's syndrome

Pathology is often accompanied by situs inversus.Characterized by primary ciliary dyskinesia - an inherited condition where cilia, which are located in the respiratory tract and in the normal state help the mucus to move, become immobile.

Approximately 20% of patients with dextrocardia have Cardgener syndrome.

Dextroversion

It is an abnormally located heart that is further to the right and then deployed to the right. The right ventricle is usually located behind the left, while the left ventricle remains on the left. This pathology is diagnosed by electrocardiography (ECG).

De-stroke

This condition is usually associated with acquired diseases of the lungs, diaphragm or pleura (membranes surrounding the lungs).Operative interventions, muscle damage or deformation may also contribute to the development of dextropositis.

Transposition of large vessels (TBS)

TBS occurs when the main vessels of the heart are connected in the opposite direction due to changes in the position of the heart chambers. This condition is defined quite rarely.

Congenital transposition of large arteries (VTBA)

Pathology occurs when the lower half of the heart changes to the opposite. In particular, the anomaly affects the ventricles and associated valves.

The disease is even less common than TBC, and the cause is still unknown to the end. According to the Association of Adult Congenital Hearts, about 0.5-1% of all children born with heart problems have VTBA.

Atresia of the three-valve valve

This condition is a congenital defect, when the three-leaf valve does not develop to a sufficient degree. Normally, the three-valve valve prevents the blood from returning to the right atrium from the right ventricle. With this pathology hemodynamics in the heart is broken, which leads to dangerous consequences.

Single-leaf or biconvex ventricles

These cases occur when the aorta, which usually carries oxygen-containing blood from the left ventricle to the rest of the body, and the pulmonary artery transferring deoxygenated blood from the right ventricle to the lungs, are connected in the same (left or right) ventricle.

Stenosis or atresia of the pulmonary valve

This occurs when the pulmonary (pulmonary) valve of the heart, which allows the blood to leave the right ventricle, narrowes (stenosis) or does not develop (atresia).

Heterotaxia

It is a congenital developmental anomaly, in which the mirror internal organs do not develop or function properly. Depending on the variety of organs involved, their amount and degree of severity, heterotaxia can be life-threatening. For example, a spleen may be absent, whereas this organ is an important part of the immune system, so children born without spleen are at risk of serious bacterial infections, which often leads to death. With another form of heterosa, the spleen is determined in small sizes, due to which the organ often works incorrectly.

Heterotaxia may be presented:

  • Anomalies of the bile-excreting system
  • Problems with lungs
  • Problems with the structure or position of the intestine
  • Serious heart defects
  • Anomaly of blood vessels.

What Is Dextrocardia Video?

Clinic

Many people with congenital dextrocardia do not always know that they have such an unusual pathology.This is due to the lack of pronounced symptoms. Often, doctors detect only minor changes in the evaluation of the imaging of the chest or heart.

The most important symptom of dextrocaria is the determination of the maximum expressed heartbeat sounds to the right of the center of the chest, and not to the left, as is normal.

In severe cases, usually in infants with additional heart defects or other illnesses, some symptoms require specific treatment.

Symptoms requiring urgent medical attention:

  • inexplicable and constant exhaustion;
  • inability to gain weight;
  • chronic infections, especially sinus sinuses and lungs;
  • shortness of breath;
  • yellowish or yellowish skin;
  • cynosity of the skin, especially around the fingers and toes.

Complications

Mirrored organs can function normally, but their unusual positioning often complicates the diagnosis of other diseases.For example, someone with a dextran cord situs inversus appendicitis causes a sharp pain in the lower left abdomen instead of the right one.

In the presence of these anatomical differences, difficulties in conducting surgical interventions may occur.

Other complications associated with dextrocaria:

  • bowel disorder, usually obstructed due to malrotation (reversal);
  • bronchial diseases, such as chronic pneumonia, associated primarily with loss of cilia (hairy mucous membranes of the respiratory tract);
  • disorders of the esophagus;
  • cardiovascular disorders;
  • heart failure;
  • infections and sepsis.

Diagnostics

Most cases of dextrocaria are diagnosed using electrocardiography and chest x-ray.

An electrocardiogram that detects inverted or reverse electrical waves usually indicates dextrocardia.

simptomy-dekstrokardii

On the radiography of the chest organs, an abnormal arrangement of the heart is visible visually.The conduction of the anterior right oblique position shows the results of the anterior left position, which is a confirmation of the mirror location of the vessels and heart.

If a suspicion of dextrocardia is suspected, a computer tomography (CT) scan or scanning of magnetic resonance imaging (MRI) may also be used.

Since dextrocaria is often combined with complete transposition of the internal organs, an ultrasound examination is mandatory.

Video Full transposition of internal organs

Treatment and prognosis

Many people with dextrocardia do not complain, their general condition is good, therefore in such cases treatment is not carried out.

Infants with dextrocardia, which are accompanied by heart defects, may require surgical intervention. If necessary, children are given a medicine that increases the strength of the heart beat and reduces blood pressure before surgery.

For patients with Cartagena's syndrome, symptomatic treatment is often prescribed, which may include drugs from the following groups:.

  • expectorant or cleansing agents from mucus;
  • diuretics that enhance diuresis;
  • hypotensive, lowering arterial pressure;
  • antibiotics are especially relevant in bacterial infections.

Genetic counseling can be useful to those with dextrocardia who want to create a family.

For most people with dextrocardiac situs inversus, life expectancy is within normal limits.In cases of isolated dexterity, congenital heart defects are more common, which increases the risk of developing various health-hazard complications.

Prevention

The illness is manifested with adverse heredity, therefore, the competent approach to family planning for all patients with dextrocardia is important. Therefore, before creating a family, you should consult a geneticist.

Preventing the development of anomalies in the fetus allows for the fulfillment of all those rules that are given to pregnant women. In particular, it is worth abandoning bad habits (alcohol and smoking), eat well and stay longer in the fresh air. It is also important in the first trimester to avoid infectious patients in order not to contract or harm the normal.


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