What Causes Dextrocardia & How To Treat It?

What is Dextrocardia?

Dextrocardia is an extremely rare congenital cardiovascular condition in which the heart which normally points towards the left is observed to be pointing towards the right. This condition can be immediately observed after birth of a baby. Dextrocardia is basically of two types namely Dextrocardia situs inversus and Dextrocardia situs inversus totalis. In the former, the tip of the heart along with its all four chambers point towards the right side of the body while in the latter variant along with the heart other vital organs like the liver, stomach, and spleen are also out of position. As a matter of fact, these organs are completely located completely opposite to where their anatomical space is [2].

Dextrocardia in majority of the cases is found incidentally or is found as a result of screening done for some other genetic abnormality. Studies suggest that the prevalence of Dextrocardia is less than 1% which speaks for the rarity of the disease. A recent study done showed that the prevalence had fallen down to 0.20% of late. An early diagnosis of Dextrocardia enables physicians to screen for other abnormalities, especially those which can be corrected [1].

It is common for children with Dextrocardia to display symptoms of a ventral septal defect or a defective pulmonary artery. There may also be extracardiac abnormalities seen with Dextrocardia, especially in cases of situs inversus totalis. This article explains the possible causes and treatment options for Dextrocardia [1].

What Causes Dextrocardia?

Dextrocardia is inherited as an autosomal recessive trait. This means that two copies of the defective gene, one from each parent, needs to be inherited for a child to develop this condition. The defect in the gene results in the vital organs such as the heart to develop in a reverse direction while the fetus is developing. Depending on the extent of the reversal, other organs like the liver or spleen may also develop is a reversed direction [2].

Studies estimate that Dextrocardia develops in 1 out of 12,000 people in which the Dextrocardia situs inversus totalis variant of the condition occurs in 1 of every 10,000 children. There is no evidence in the research to suggest that there is any effect of ethnicity, gender, or race in the development of this condition. Even though there is a reversal in the direction of vital organs of the body in an individual with Dextrocardia, there may be not problems with their functioning [2].

However, due to the positioning of these organs in case if they develop any abnormality it will be very tough to diagnose them. As an example, if an individual with Dextrocardia develops appendicitis then he or she will have pain on the left side of the abdomen instead of the right which is the norm. This can often lead to a misdiagnosis especially in cases where the physician does not have knowledge that the individual has Dextrocardia. Not only there may be an error in diagnosis but treatment will also be erroneous in such cases [2].

There may also be certain other complications that are associated with Dextrocardia. These include problems with bowel function due to reversal of the organs. An individual with Dextrocardia also will be prone to respiratory conditions like pneumonia primarily due to loss of the hair like projections called cilia which aids in respiration. An individual with Dextrocardia will also be prone to have cardiovascular abnormalities due to the reversed positioning of the heart to include serious conditions like heart failure [2].

People with Dextrocardia are also prone to develop variety of infections. It should be noted here that since Dextrocardia in most cases remains completely asymptomatic it may remain undiagnosed and untreated for a fairly long period of time.

How is Dextrocardia Treated?

Coming to the treatment options, children with this condition who have cardiac defects will require surgery to correct the deformity. Medications will be given to improve the pulse rate of the child and treat blood pressure abnormalities if any prior to surgery [2].

People with Kartagener Syndrome, which is a genetic disorder that is a combination of three medical conditions, including situs inversus, chronic sinusitis, and bronchiectasis and is common finding in Kartagener syndrome also need treatment. They will be given medications to treat sinus abnormalities and correct the lung problem to bring down the impact of Dextrocardia [3].

The treatment options for these individuals include medications for clearing the accumulated mucous. Medications will also be given to treat symptoms of bronchiectasis and sinusitis. It is essential for people with Dextrocardia to get genetic counseling so that they can be prepared to cope with this problem if they wish to start a family in the future [2, 3].

Coming to the prognosis of Dextrocardia, there is no impact in the life expectancy of an individual with Dextrocardia. However, in people with cardiac abnormalities, there is a greater risk for cardiovascular illnesses some of which may be potentially serious and thus regular monitoring will be required [2].