Hypoplastic Right Heart Syndrome treatment consists of three stages of surgery. There is no cure for the congenital heart defect. Normally, the right ventricle of the heart pumps blood to the lungs, giving it oxygen before the left side of the heart pumps the blood to the rest of the body. When the right ventricle is underdeveloped, as it is with Hypoplastic Right Heart Syndrome, it cannot push blood to the lungs to get oxygen. There is no way for the defunct ventricle to be surgically replaced or 'fixed', instead artificial passageways are created to allow the functioning ventricle (the left side) to pump the blood to the lungs. The surgeries will reroute the flow of blood, ensuring that the only functioning ventricle can do the work. In a normal functioning heart, the left ventricle circulates blood to the body. In children with Hypoplastic Right Heart Syndrome, the left ventricle must pump blood to the lungs and circulate it to the body. This effect is achieved using the three stages of surgical procedures: Norwood, Glenn and Fontan.
Norwood Procedure
The first stage of Hypoplastic Right Heart Syndrome treatment is the Norwood procedure. Doctors will perform this surgery in your child's first week of life. Surgeons insert an artificial tube, called a shunt, into the aortic arch. The shunt connects the aorta to the pulmonary artery. This passageway allows blood flow to the lungs. The Norwood procedure will be sufficient in sustaining a baby until they outgrow the shunt, usually between 4-6 months of age.
Glenn Procedure
The second stage of Hypoplastic Right Heart Syndrome treatment, the Glenn procedure, is usually performed when your child is 4-6 months old (but could be earlier or later based on your child's size and heart function). This surgery connects some of the veins that carry blood from the body to blood vessels that carry blood to the lungs. This mixing allows for increased blood flow to and from the lungs. This surgery can't be performed at birth because a child must be a certain size for this procedure to be safe and successful.
Fontan Procedure
The third stage of Hypoplastic Right Heart Syndrome treatment is the Fontan procedure. Your child will be ready for the third and final surgery when they are between 18-48 months old. Again, the timing of this surgery will be based upon your child's size and heart function. The Fontan completes the Glenn procedure, connecting the remaining veins that carry blood from the body to blood vessels that carry blood to the lungs. This surgery provides maximum blood flow to and from the lungs. Completion of the Fontan will likely be the last heart surgery your child needs since it's effects can last for the rest of your child's life. Lifetime follow-up with a cardiologist will be necessary. If heart function begins to decline during any point of your child's life, a heart transplant may be needed.
Norwood Procedure
The first stage of Hypoplastic Right Heart Syndrome treatment is the Norwood procedure. Doctors will perform this surgery in your child's first week of life. Surgeons insert an artificial tube, called a shunt, into the aortic arch. The shunt connects the aorta to the pulmonary artery. This passageway allows blood flow to the lungs. The Norwood procedure will be sufficient in sustaining a baby until they outgrow the shunt, usually between 4-6 months of age.
Glenn Procedure
The second stage of Hypoplastic Right Heart Syndrome treatment, the Glenn procedure, is usually performed when your child is 4-6 months old (but could be earlier or later based on your child's size and heart function). This surgery connects some of the veins that carry blood from the body to blood vessels that carry blood to the lungs. This mixing allows for increased blood flow to and from the lungs. This surgery can't be performed at birth because a child must be a certain size for this procedure to be safe and successful.
Fontan Procedure
The third stage of Hypoplastic Right Heart Syndrome treatment is the Fontan procedure. Your child will be ready for the third and final surgery when they are between 18-48 months old. Again, the timing of this surgery will be based upon your child's size and heart function. The Fontan completes the Glenn procedure, connecting the remaining veins that carry blood from the body to blood vessels that carry blood to the lungs. This surgery provides maximum blood flow to and from the lungs. Completion of the Fontan will likely be the last heart surgery your child needs since it's effects can last for the rest of your child's life. Lifetime follow-up with a cardiologist will be necessary. If heart function begins to decline during any point of your child's life, a heart transplant may be needed.
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