Open-hearted tetralogy of Fallot surgery, timing of tetralogy of Fallot surgery, necessity of treatment, second treatment options and the latest treatment methods for tetralogy of Fallot in Turkey.
Tetralogy of Fallot surgery is usually done in the first year of a child's life to correct a congenital heart defect. Tetralogy of Fallot is a heart problem made up of four related heart defects. These defects include ventricular septal defect, pulmonary valve stenosis, aortic malposition, and right ventricular hypertrophy, all of which together cause tetralogy of Fallot.
What is tetralogy fallot?
Tetralogy of Fallot is a rare and complex (present at birth) heart defect. It is named after the doctor who first described the condition in 1888.
A tetralogy fallot is the name for a group of four heart defects:
1. Large ventricular septal defect
The ventricles are the two lower chambers of the heart, and the septum is the wall that separates the left and right chambers.
A ventricular septal defect is a hole in the septum that allows oxygen-rich blood to flow from the left ventricle into the right ventricle and mix with oxygen-poor venous blood there, instead of flowing into the aorta on its way to the rest of the body.
Occasionally, oxygen-poor blood can flow through the hole (from right to left), bypassing the lungs and causing cyanosis and shortness of breath in the child (low levels of oxygen in the circulation).
2. Pulmonary stenosis
Blood flows normally from the right ventricle through a valve or valve to the pulmonary arteries where it is loaded with oxygen.
Pulmonary stenosis is a narrowing of the valve between the right ventricle and the pulmonary artery, or muscle growth just below the valve (subvalvular stenosis).
Often, the stenosis is not only limited to the valve but also the muscles just below it. The constriction of the passage causes the heart to pump more forcefully to push blood through it and reduces the amount of blood flowing through the valve.
3. Right ventricular hypertrophy
The additional pumping required by pulmonary stenosis causes the right ventricle to thicken (hypertrophy).
4. Overlay of the aorta
The aorta is the main artery that carries oxygen-rich blood from the heart to the rest of the body. Normally, the aorta is attached to the left ventricle and oxygenated blood flows from the ventricle to the aorta. In the case of a tetralogy fallot, the aorta is located between the left and right ventricles, above the ventricular septal defect.
This location allows the aorta to receive blood from both sides of the heart, so that the oxygen-poor blood from the right ventricle mixes with the oxygenated blood from the left ventricle.
How common is tetralogy fallot and what causes it?
Tetralogy fallot is a relatively rare disease, with about 5 in 10,000 births affected by tetralogy of Fallot. Although this heart defect was recognized 120 years ago, the exact cause is still unknown.
The defect occurs during the development of the baby's heart before birth. About 15 percent of children with tetralogy of Fallot have a specific genetic defect that is also linked to other birth defects such as a cleft palate.
How is tetralogy of Fallot diagnosed?
This complex set of defects causes the body to suffer from a lack of oxygen. The reason is that not enough blood flows into the pulmonary artery to take oxygen from the lung, and the blood flowing through the aorta to the body is poor in oxygen.
In most cases, the defect is so severe when the baby is born that the lack of oxygen causes a bluish color (cyanosis) to appear in the baby's skin, lips and fingernails. Cyanosis usually worsens gradually during the first few weeks of a child's life. Tetralogy fallot is usually diagnosed very early in a child's life, but it may not be diagnosed for a few months or even several years.
How is tetralogy of Fallot corrected?
The tetralogy fallot can be corrected with surgery. This is generally done during the first year of a child's life, although it can sometimes be done at an older age.
The goal of these surgeries is to prevent diversion of blood through the ventricles and relieve pulmonary valve stenosis to improve blood flow to the lungs.
Complete corrective surgery for a tetralogy fallot involves closing the opening between the ventricles (perforations) with a patch, opening the obstruction to blood flow to the lungs and dilating the right ventricular outlet. The way the blockage is opened depends on the specifics of each person's condition, but can include removing some of the thicker muscle below the pulmonary valve, widening the valve itself - sometimes by placing a patch through it - and, if necessary, widening the branches of the arteries that go to each lung . If the valve is completely blocked, a tube (or conduit) with a valve may be needed to allow blood to reach from the heart to the lung arteries.
What are the treatment options for tetralogy fallot?
There is no medical treatment for tetralogy fallot. Structural abnormalities of the heart require operation and surgical correction. In most cases, children undergo tetralogy of Fallot correction by one year of age.
The first step in treatment in children may be palliative surgery (partial repair) to repair blood flow to the lungs without directly correcting structural defects. Palliative surgery usually involves some type of shunt to increase blood flow to the lungs. These techniques can relieve a child's symptoms for several years, until adulthood when more precise repair surgery can be performed.
Are there other types of surgery for tetralogy fallot?
In some children, complete repair of tetralogy of Fallot early in life (early surgery) may not be optimal. This may be the case for a very young or premature baby, a very critically blue baby, or other medical problems early in life.
In these children, primary surgery may be performed to supply the lungs with more blood and to overcome the cyanosis. One way to do this is to surgically place a small tube, called a shunt, from the body's artery (aorta) into the pulmonary artery to provide more blood flow to the lungs.
This does not completely correct the tetralogy of Fallot and a complete second surgery will be needed later when the child is older.
Another option that can be done soon after the birth of a baby with tetralogy fallot is to place a stent or mesh tube in the patent ductus arteriosus - a natural bridging artery between the aorta (the body) and the pulmonary artery that every baby has when in the mother's womb, but It closes a few days after birth. This procedure is done in the cardiac catheterization department, and a complete repair must be done at a later time.
Repeat operations may be necessary in some people with tetralogy of Fallot. Surgery to open a narrowed pulmonary valve often leads to valve leakage (regurgitation). This valve that leaks blood in the opposite direction is usually tolerated by the body without symptoms, but it can lead to symptoms or significant enlargement of the right side of the heart in some people over time.
In these cases, another surgery may be needed to replace the pulmonary valve, often in adolescence or adulthood. You can read about Switching valves by robots in Turkey.
What care is needed for an adult who has undergone surgical repair of tetralogy of Fallot?
Specialists in adult congenital heart disease recommend that an adult who had palliative surgery for a tetralogy fallot as a child should have a comprehensive cardiac evaluation periodically.
The shunts used can relieve some of the problems associated with tetralogy of Fallot such as stenosis, high blood pressure in the pulmonary artery or causing an increased load on the left side of the heart. Definitive surgery often results in a large leak in the pulmonary valve. The size and function of the right side of the heart should also be closely monitored.
Some patients may also develop large collateral vessels of the aorta as a way to increase blood flow to the lungs. These must be closed prior to final surgical repair.
Having any of these problems or worsening symptoms, such as cyanosis or fatigue, are reasons why an adult should undergo a full surgical repair.
In some cases of tetralogy of Fallot, a patient may reach adulthood without any surgical repair, but this is not uncommon. Specialists recommend that these individuals consider a complete surgical repair to prevent future complications or sudden death.
How is tetralogy fallot surgically treated in adults in Turkey?
Surgical repair of tetralogy of Fallot is complex, delicate and highly technical, and must be performed by a surgeon who specializes in adult congenital heart disease. The process includes a combination of techniques to fix all four defects.
For a complete repair, the surgeon closes the ventricular septal defect with a patch. The exit passage from the right ventricle is opened, and the pulmonary valve is repaired or replaced. The pulmonary arteries of both lungs are dilated, and sometimes a tube is placed between the right ventricle and the pulmonary artery to improve blood flow.
How successful is tetralogy of Fallot surgery in adults?
Surgical repair of tetralogy of Fallot in adults has a very high success rate when performed by an experienced congenital heart surgeon. In one long-term study, the 36-year survival rate for adults who had surgical repair of tetralogy of Fallot was 86 percent.
Are there any potential problems or complications of tetralogy of Fallot surgery?
Although surgical treatment of tetralogy of Fallot is very effective in repairing structural defects and blood flow through the heart, it can cause some persistent abnormalities in heart function. If these problems arise, they can be treated with additional surgery.
Many adults who have tetralogy of Fallot repair do not need further surgical treatment. In a study published by the Canadian Network of Adult Congenital Heart Disease, only 10 to 15 percent of people needed repeat surgery over a 20-year period.
Possible complications associated with surgical repair of a tetralogy fallot include:
- Electrical disturbances: Placing a patch on a ventricular septal defect can impair the atria's ability to transmit electrical signals to the ventricles. This can be corrected by a pacemaker.
- Irregular heartbeat: Atrial fibrillation (the two upper chambers of the heart contract irregularly and often too quickly) is a common complication after heart surgery. This can be treated with medication or a non-surgical procedure. Ventricular tachycardia (very fast heartbeats in the lower chambers of the heart) is much rarer but more serious, and life-threatening. A congenital cardiologist can estimate whether an individual is at risk after tetralogy of Fallot surgery.
- Leaking valves: Heart valves are designed to allow blood to flow in one direction. When there is a leak in the valve, blood can return to the chamber where it came from. Patients with tetralogy of Fallot are also at risk of developing an ascending aortic aneurysm. The most common valve problems after tetralogy of Fallot repair is pulmonary valve leakage, but the aortic valve and tricuspid heart valve can also leak. Leaked valves are generally repaired surgically, but new methods of non-surgical implantation of valves are currently being explored.
- Ventricular septal defect: Sometimes a ventricular septal defect does not close completely and some leakage remains around the patch. If the leak is large or causes significant symptoms, it can be repaired surgically.
What follow-up is required after tetralogy of Fallot surgery in Turkey?
Adults who have had a tetralogy fallot repair need regular heart exams with an adult congenital cardiologist. Various tests of heart function such as an echocardiogram, Holter monitor, EKG or exercise stress test may be needed periodically to make sure the heart is working well.
People with repaired tetralogy of Fallot usually have some restrictions on certain strenuous activities, such as competitive sports.
Is pregnancy safe for women who have had tetralogy of Fallot surgery?
Women who have had a tetralogy fallot surgically repaired and are considering becoming pregnant should see an adult congenital cardiologist and an obstetrician who specializes in caring for women with special medical conditions.
With proper prenatal care and close monitoring, most women with repaired tetralogy of Fallot are able to become pregnant and give birth safely. An obstetrician, in consultation with an adult congenital cardiologist, can help determine the risks in connection with pregnancy.
What after surgical correction of tetralogy of Fallot?
The long-term outlook for children and adults with tetralogy of Fallot is good — but you will need regular follow-up with a cardiologist throughout your life to monitor heart function and heart rhythm problems and whether subsequent surgery to replace the pulmonary valve may be necessary. Various tests may be used to help the cardiologist monitor you, including:
- Electrocardiogram (EKG).
- Holter screens.
- Do stress tests.
- Cardiac magnetic resonance imaging.
Your doctor will determine if you can participate fully in sports and exercise, or if you need any restrictions. Most people can participate in at least some recreational exercise. In many cases, a pregnancy for women with tetralogy of Fallot is successful, although it's important to discuss risks and recommendations with your cardiologist before becoming pregnant.
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