Double Outlet Right Ventricle Repair
This is a very rare congenital heart defect in which both the pulmonary artery and the aorta arise from the right ventricle. Normally, only the pulmonary artery that carries blood to the lungs for oxygen arises from the right ventricle. The aorta, which carries oxygenated blood from the heart to the body, normally arises from the left ventricle.
This defect almost always coincides with a Ventricular Septal Defect (VSD) an abnormal opening in the septum (wall that separates the two ventricles) that allows blood to pass between the right and left ventricles. In the case of Double Outlet Right Ventricle (DORV), a Ventricular Septal Defect is helpful because it allows the oxygen rich blood of the left ventricle passage to the right ventricle, which pumps it to the aorta and out of the heart to the body. If this passage did not exist, there would be no way for oxygen-rich blood to get to the aorta. Still, this oxygen rich blood mixes with oxygen poor blood, so oxygen levels in the blood are not optimal, and the heart must work extra hard to maintain circulation.There are four types of classification
Causes
The heart forms during the first 8 weeks of fetal development. It is at this time that defects such as DORV emerge. Some congenital heart defects may have a genetic link, either occurring due to a defect in a gene, a chromosome abnormality, or environmental exposure, causing heart problems to occur more frequently in certain families. Most of the time, these kinds of heart defects occur sporadically (by chance), which no clear reason for their development.
Symptoms
The position the ventricular septal opening will affect the type of symptoms noted, the severity of symptoms, and the age at which they first occur. The following are the most common symptoms of DORV defects. However, each child may experience symptoms differently. Symptoms include:
Diagnosis
If the child is experiencing any symptoms mentioned above or your newborn baby was born with a bluish tint to the skin, he or she may be referred to a pediatric cardiologist. A pediatric cardiologist specializes in the diagnosis and medical management of congenital heart defects, as well as heart problems that may develop later in childhood. The cardiologist will perform a physical examination, listening to the heart and lungs, and making other observations that help with the diagnosis.
A heart murmur, which is simply a noise heard through the stethoscope, caused by the turbulence of blood flow will also be investigated. The location in the chest that the murmur is heard best, as well as the loudness and quality of the murmur will give the cardiologist an initial idea of the kind of heart problem.
A combination of the following medical tests is also used to diagnose DORV defects:
Treatment.
Treatment for DORV will be based on:
The treatment of choice is typically surgery that aims to connect the aorta to the left ventricle. This can be done through several different approaches that vary depending on your child’s circumstance.
One method is known as intra-ventricular repair, meaning that the repair occurs entirely within the right ventricle. Pediatric cardiac surgeons create a kind of tunnel known as a baffle that connects the left ventricle to the aorta through the VSD. This way, blood flows from the left ventricle to the aorta.
Another procedure is called an arterial switch operation and it involves just that switching the positions of the great arteries. There are two steps involved. During the first step, a tunnel is created from the VSD to the pulmonary artery, connecting the left ventricle to the pulmonary artery. So now, with the right ventricle connected to the aorta, the connections between the great ventricles and great vessels are completely in reverse of what they should be. The second step involves disconnecting and reconnecting the vessels to their proper ventricles. The coronary arteries must be transferred to the newly positioned aorta as well, or blue blood will supply the muscle of the heart. Associated holes between the chambers of the heart are also closed.
Hospital stay of would typically be for about 8 days.
The other major cardiac procedures are:
Few popular hospitals for Double Outlet Right Ventricle Repair are:
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