Right-sided heart – is it possible?
A normal heart points towards the left side of the chest. This part which is called the apex of the heart consists of tips of right and left ventricles. Dextrocardia is a condition in which the apex of the heart points towards the right side of the chest. This is a congenital malposition of the heart. This congenital abnormality, where only the heart is affected is called ‘isolated dextrocardia’. When other organs in the body are also located on the opposite side, this condition is called ‘situs inversus’.
The genesis of dextrocardia
The heart of a fetus develops during the initial weeks of pregnancy. It is during this time that the heart turns to the right side to develop dextrocardia. The cause of this occurrence is not known. These are many combinations of this abnormality as this may either present as the only condition or is associated with abnormal position of other organs.
In mirror image change of position, all organs on right go to left and vice versa. This includes the lungs and abdominal organs. The liver is located on the left and spleen on the right. The organs are normal in structure and function including the heart. It is a rare condition and usually remains asymptomatic.
Associated complications of right-sided heart
Kartagener syndrome: Some people with situs inversus have abnormal ciliary function in the respiratory passage. The cilia are required for the clearance of excessive secretions or mucus. When this function is not efficient, this results in accumulation of mucus and infection. The patients develop chronic sinusitis and bronchiectasis or lung infections.
Heart defects associated with dextrocardia
The most common type of right sided heart is associated with many types of birth defects in the heart. Some of these are listed below.
- Transposition of the great vessels – the large vessels of the heart are abnormally positioned leading to abnormal flow patterns.
- Ventricular septal defect – an abnormal opening in the septum or wall between right and left ventricles.
- Double outlet right ventricle – both great vessels, pulmonary artery and aorta are connected to the right or left ventricle.
- Endocardial cushion defect – this is associated with a defect in atrial septum, ventricular septum or the atrio-ventricular valves.
Other organ defects associated with dextrocardia
The organs in the chest and abdomen are also positioned abnormally which results in aberrations of their function. The spleen may be absent. Since spleen has a major role to play in the development of natural immunity, there is a risk of infection due to poor immunity. These patients may also have abnormalities of intestines, gall bladder and lungs.
Depending on the abnormality of the heart and other organs, these patients may present with the following clinical features. Bluish skin and difficulty in breathing occurs due to poor oxygenation of blood. This results in poor growth and failure to gain weight. The patients may develop jaundice and recurrent sinusitis and lung infections.
The diagnosis of dextrocardia is done easily. Based on clinical suspicion, an echocardiography helps in confirmation of the diagnosis.
Treatment of right-sided heart
In patients with mirror image placed organs on the opposite sides of body, usually no treatment is requires as the organ functions are normal. Patients with recurrent sinusitis and lung infections will require antibiotics and drainage of any collection of pus. Treatment in relation to the heart depends on the abnormality of heart structure and function. In many cases surgery to correct the birth defect is required. Children who are critically ill will require stabilization with drugs before and after surgery. In some cases abdominal surgery is done for treatment of complications due to abnormal position of organs like intestinal obstruction. Children with missing spleen require antibiotic cover till their adulthood and especially during any surgical intervention like a dental procedure.
Dextrocardia is a rare condition. It is associated with birth defects in the heart and abnormal location of other organs in the chest and abdomen. It requires an assessment by a team of specialists. Many of these children with only dextrocardia have a good life expectancy while others survive depending on the severity of heart and other organ related problems.