Overview
Dextrocardia with situs inversus is characterized by abnormal heart and internal organ positioning. When the body's internal organs are in a reversed position, this is referred to as situs inversus. Dextrocardia is used to describe the heart's congenital reversed position, in which the cardiac apex points to the right. Two types can be distinguished; they are situs inversus dextrocardia (right chambers are still anterior to the left chambers) and dextroversion in which the heart is rotated to the right and the left atrium and ventricle are anterior to their right counterparts).
Dextrocardia situs inversus is a rare congenital anomaly that affects approximately 1/6000 to 1/ 35000 live births. The defect in chromosome 14 causes this autosomal recessive genetic disorder.
Dextrocardia causes the tip of the heart to point to the right side of the chest rather than the left. The reversal of the organs, like a mirror image in the chest and abdominal cavity, is referred to as situs inversus. Some people who are affected have no obvious signs or symptoms. A small percentage of people, however, have congenital heart defects, most commonly transposition of the great vessels.
Dextrocardia situs inversus can also be associated with primary ciliary dyskinesia, also known as Kartagener syndrome. Kartagener syndrome should be suspected in the setting of the classic triad of situs inversus, related to chronic sinusitis and bronchiectasis caused by ciliary dyskinesia.
Symptoms
Some people who are affected have no obvious 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.
When to See a Doctor?
In severe cases, typically in infants with additional heart defects or another disease, certain symptoms need medical treatment.
Symptoms that require medical attention include:
- unexplained and continual exhaustion.
- inability to gain weight.
- chronic infections, especially of the sinus and lungs
- difficulty breathing
Causes and Risk factors
The exact cause of dextrocardia situs inversus is unknown but the condition occurs because the heart does not form correctly during fetal development.
- Dextrocardia affects approximately 1 in every 12000 people. One out of every 10,000 children suffers from dextrocardia situs inversus totalis. Gender, race, and ethnicity do not seem to have any impact on whether or not a person develops the condition.
- Dextrocardia with Situs Inversus, a rare birth condition, is caused by autosomal recessive genes. During fetal development, the primitive loop in the embryo moves in the reverse direction of its normal position, causing organ displacement.
- The exact reason for Dextrocardia with situs inversus is unknown, but the condition is caused by abnormal positioning of internal organs during fetal development.
- Some people have dextrocardia situs inversus as a result of a condition known as primary ciliary dyskinesia. Changes or mutations in different genes can cause primary ciliary dyskinesia.
- The parents of an affected individual usually carry one mutated copy of the gene and are referred to as carriers. Usually, the carriers do not show signs or symptoms of the condition.
Diagnosis
- Diagnosis of dextrocardia situs inversus is suspected in some cases based on the presence of concerning signs and symptoms; however, it is frequently discovered by chance when an X-ray and ultrasound are performed to investigate some other different condition.
- Chest radiography can help confirm the diagnosis of dextrocardia with situs inversus, in which the cardiac apex and aortic arch are on the right side of the chest.
- Computed tomography scan is also typically the preferred examination to confirm the diagnosis of dextrocardia situs inversus.
- Magnetic resonance imaging may be substituted in cases that are associated with congenital heart defects.
Treatment
Treatment typically depends on the heart or physical problems the person may have in addition to dextrocardia with situs inversus.
- For example, infants born with congenital heart defects or other organ malformations may require surgery.
- The management of people affected by Kartagener syndrome typically includes measures to enhance clearance of mucus, prevent respiratory infections, and treat bacterial infections.
Dextrocardia situs inversus does not usually necessitate treatment. However, many of the congenital conditions that accompany dextrocardia may require treatment such as:
- Medications which help the heart to pump blood more efficiently.
- Antibiotics to manage respiratory tract infections.
- Medications to remove the fluid out of the lungs.
- Pacemakers for certain heart conditions such as arrhythmias.
- Surgery for congenital heart defects and problems with other organs in the chest or abdomen.
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 |
Care at Medicover Hospitals
We have the best team of endocrinologists and neurosurgeons at Medicover hospitals, who provide the most comprehensive treatment and care. Our diagnostic department is equipped with cutting-edge technology and equipment to perform the tests needed for dextrocardia-situs-inversus diagnosis and treatment planning. Our medical professionals work closely with patients to evaluate their health and treatment progress in order to achieve a faster recovery.
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