Dextrocardia is a congenital anomaly of the cardiovascular system, a kind of dystopia in which the heart is partially or completely displaced to the right and occupies a position that is symmetrically symmetrical to the normal one.
Similarly, the heart and all large vessels are located. In some cases, the mirror location of some or all of the internal organs is revealed. According to statistics, dextrocardia occurs in about 0.01% of the population.
If the abnormal location is not accompanied by other pathologies, dextrocardia may not appear and is detected by accident, when seeking medical advice on other occasions.
The displacement of the heart to the right can be a consequence of pathological processes in the chest: lung atelectasis, hydrothorax, and tumor processes. With a secondary mechanical displacement, one speaks of a pathological de-stressation of the heart.
Causes of
The laying of organs and body systems occurs in the first trimester of pregnancy .The heart tube is formed during the first weeks of embryo development and, for unexplained reasons, curves to the right.
The most likely cause of the abnormal structure of the cardiovascular system is the mutation of the genes ZIC3Shh, HAND, Pitxz, ACVR2 .There is evidence of an autosomal recessive type of anomaly inheritance.
Types of
All cases of dextrocardia are divided into three types:
- Simple dextrocardia .Very rare option, when only the heart and the main vessels are mirrored.
- Dextrocardia with a mirror location of the part of the digestive and respiratory system.
- Dextrocardia with complete transposition of internal organs.
Danger and complications
Abnormal cardiac placement of in the absence of concomitant pathologies does not pose a particular health hazard to , does not affect quality and life expectancy. In such cases, patients often learn about their characteristics by accident, during a medical examination on another occasion.
One of the most significant risks for a patient with undiagnosed and uncomplicated dextrocardia and transposition of organs is a medical error in the diagnosis. Diagnosis of the most common diseases of internal organs is hampered by due to their unusual location and the rarity of similar clinical cases.
Difficulties arise when it is necessary to perform cavitary operations, especially when it comes to the need for an operation to transplant asymmetric organs.
In most cases, the dystopic location of the is accompanied by other developmental defects of the , among which are:
- Transposition of the main arteries;
- Pulmonary artery stenosis in children;
- Endocardial defects;
- Twin ventricular exit;
- Defect of interventricular septum;
- The Tetrad of Fallot.
An abnormal location of the heart of can be combined with severe pathologies of the development of the organ, for example, a two- or three-chambered heart is found in patients.
The danger of a patient's condition depends on the type of concomitant pathology and the degree of its severity. Part of the concomitant heart disease manifests immediately , during the first hours of life( the so-called "blue" defects), some( "white") can be detected later, due to asymptomatic flow at an early age.
Sometimes the right-sided heart is combined with other pathologies of the abdominal and pleural cavities. One of the most common comorbidities is the heterotaxic syndrome .Patients with heterotoxic syndrome often lack a spleen, in some cases several underdeveloped spleen with significant impairment of function are found.
A half of patients with PCD are diagnosed with Cartagraer-Sievert syndrome , which is inextricably linked to an abnormal position of the internal organs. In such patients, pathological disorders of the anatomical structure of the upper respiratory tract, numerous bronchiectasias are revealed.
Cilia on the surface of the mucous membranes, performing the functions of the active filter, may be absent completely. Men with this disease are sterile , since their spermatozoa are devoid of flagella.
This pathology is always combined with full or partial transposition of internal organs, which indirectly confirms the hypothesis of the hereditary nature of dextrocardia.
The dystopic location of the heart is often found in children with the Patau syndrome - trisomy over 13 pairs of chromosomes. This chromosomal disease is accompanied by multiple malformations affecting almost all organ systems, patients with Patau syndrome rarely live to school age.
Symptoms of
Uncomplicated dextrocardia combined with full transposition of internal organs usually does not manifest itself in any way and does not disturb the patient at all. Such cases are relatively rare, much more often dextrocardia is accompanied by other pathologies of , which can occur both from the first seconds and for several years of life.
There are no characteristic external symptoms caused by cardiac dystopia, but can indirectly be indicated:
- Jaundice staining of the skin and sclera from the moment of birth;
- Difficulty in breathing;
- Cyanosis or pallor of the skin;
- Heart rhythm disturbances.
In older children, in addition to the listed symptoms, there may be:
- Increased susceptibility to infectious diseases of the lungs and upper respiratory tract;
- Increased fatigue, muscle weakness;
- Lag behind physical development.
When to call a doctor
Complicated dextrokardia is detected in infancy and the child is immediately put on a dispensary record of from a cardiologist. In the detection of uncomplicated dextrocardia in older and adulthood arrive in a similar manner.
Diagnosis
Suspicion of dextrokardia may occur with a routine external examination of .The apical impulse of the heart upon palpation is revealed on the right, percussion shows the displacement of cardiac dullness, with an auscultation an unusual location of cardiac tones is revealed.
Additional diagnostic studies may be required to confirm the diagnosis:
- X-ray examination;
- ultrasound of the heart and blood vessels;
- Electrocardiography;
- Magnetic resonance imaging;
- Computed tomography.
- Angiography and cardiac catheterization.
The choice of survey methods depends on the type and severity of the underlying pathologies.
Since the electrical axis of the heart for the dextrocardia is mirrored to normal, the ECG curve looks like a mirror image of the normal .Electrocardiography is a mandatory type of examination for suspected cardiac arrhythmias for the differential diagnosis of congenital and acquired pathologies of the cardiovascular system.
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Treatment of
With the diagnosis of "cardiac dextrocardia", not complicated by concomitant diseases of and does not affect the general health state in any way, does not require treatment.
Tactics of treatment of in the presence of accompanying pathologies aimed at their elimination and is developed depending on the severity of the patient's condition. Often the only possible way to relieve the condition and save the patient's life is surgical intervention.
In preparation for surgery, conservative treatment is performed to stabilize the patient's condition. Within the framework of maintenance therapy appoint:
- Diuretics;
- Hypotensive preparations;
- Preparations that support the heart muscle.
Almost always, patients are prescribed for long-term antibiotic treatment and medicines from the probiotic group for the prevention of bowel microflora disorders.
Prognosis and prevention of
In the absence of concomitant diseases, there is no threat to the health and life of the patient .The presence of complications from other internal organs increases the risk of developing heart failure, acute infectious diseases, male infertility, intestinal malignancy.
In severe cases, accompanied by multiple congenital pathologies, the death rate of patients during the first year of life of may exceed 90% of ( Patau syndrome).
There are no effective prophylaxis measures for dextrocardia .Due to the likely hereditary nature of the anomaly, couples who have proven cases of dextrocardia among relatives should particularly carefully monitor their health during pregnancy planning. The general recommendations include adherence to the principles of healthy lifestyles.
