With untreated or “uncorrected” patients only 3% survive past the age of 40. But as with all things, there are exceptions to this. If the condition is corrected early the patient has a normal life expectancy. Even with corrective operations later, life expectancy can be normal.
I was wondering what the life expectancy is of a Tetralogy of Fallot baby because I am one. My TOF was corrected with surgery in 1976 in Bloemfontein South Africa. Except for some problems with Atrial Fibrillation I have lived a normal and full life. My name is Janco Vorster and I am a Tetralogy of Fallot survivor. I blog about Tetralogy of Fallot, Atrial Fibrillation, Ectopic heartbeats, PAC, Anxiety, Panic attacks, and #thoughtarrest. What is Tetralogy of Fallot, how serious is it and what are some of the success stories?
HOW SERIOUS IS TETRALOGY OF FALLOT?
If infants who have Tetralogy of Fallot (TOF) are not treated their condition will in most cases become more severe. They can have “TET spells” which is caused by a rapid drop in oxygen in the blood. It can be seen in the skin, lips and nails becoming blue, usually after feeding, crying or when they are agitated. It is most common in infants from 2-4 months old.
This congenital heart defect TOF can be fatal if left untreated. Untreated there could be less blood flow to the lungs and severe cyanosis ( a bluish discoloration of the skin due to poor circulation or inadequate oxygenation of the blood) may cause life-threatening conditions.
TETRALOGY OF FALLOT 4 DEFECTS
Tetralogy means a set of four related symptoms or abnormalities frequently occurring together. These abnormalities or defects affect the structure of the heart. With Tetralogy of Fallot (TOF), this is the four defects:
Right ventricular hypertrophy. The muscular wall of the right ventricle is too thick. Over time it may cause the heart to stiffen, weaken and in some cases then fail.
Pulmonary valve stenosis. It is a narrowing of the pulmonary valve. It is the valve that separates the lower right chamber of the heart (right ventricle) from the main blood vessel leading to the lungs (pulmonary artery). This narrowing of the valve reduces blood flow to the lungs.
Ventricular septal defect. It is a hole (defect) in the wall (septum) that separates the two lower chambers of the heart — the left and right ventricles. This hole allows deoxygenated blood and oxygenated blood to mix, which is not a good thing. It produces an “oxygen-poor” type of blood that, creates cyanosis and in time can weaken the heart.
Overriding aorta. Because of the aortas “misplacement”, it is shifted slightly to the right and lies directly above the ventricular septal defect. This causes mixing of the oxygen-poor blood from the right ventricle with the oxygen-rich blood from the left ventricle.
Some children or adults with Tetralogy may also have other heart defects.
TETRALOGY OF FALLOT TREATMENT
If TOF is diagnosed after birth the first concern is the oxygen levels of the infant. If the oxygen levels are to low a procedure called prostaglandin infusion can be done to correct it, before the main repair surgery is done, usually at 6 months old.
Surgical repair of the defect is always necessary. This is done with open-heart surgery on the patient usually very early in their life before their first birthday. When the surgery must be done will depend on a few factors that the surgeon will take into consideration.
The aim of the surgery will be to correct the four defects mentioned above. In the absence of any risk factors, more than 95 percent of infants with TOF successfully undergo surgery in the first year of life.
TETRALOGY OF FALLOT COMPLICATIONS
Infective endocarditis, after repair surgery, can occur which is an infection of the inner lining of the heart or heart valve caused by a bacterial infection.
Other post-operative complications will not be discussed in this post, but some of the longterm complications of TOF repair could be:
Heart arrhythmias
Pulmonary regurgitation or PR which is a leaky valve between the heart and the lungs.
Enlarged aorta
Leaking tricuspid valve which is also called tricuspid regurgitation or TR.
Leaking at the ventricular septal defect (VSD) area.
Problems with heart muscle and lungs.
As I mentioned above the repair of Tetralogy of Fallot is usually very successful and the possibility of above mentioned complications must be managed by regular visits to your cardiologist at least once a year.
Regular checkups at a cardiologist are very important. In my case, my cardiologist concentrates more on the rhythm “issues” like my arrhythmia called Atrial Fibrillation and then I also regularly go to a pediatric cardiologist who has vast experience of all types of Congenital Heart Diseases(CHD), and specially Tetralogy of Fallot. The reason I go to the pediatric cardiologist is because he looks at the physical and structural aspects of my heart.
In the United States of America and other countries, there are dedicated adult congenital heart specialists at certain centers that have in-depth knowledge of complications and special care that TOF adults need.
TETRALOGY OF FALLOT AFTER CARE AND RECOVERY TIME
After corrective surgery Tetralogy of Fallot babies will spend some time in ICU.
There are a few factors that the doctor will take into consideration before a TOF patient will be discharged. This will differ from patient to patient.
Special equipment may be used after surgery to help your TOF baby recover. It may look scary to see so many pipes connected to your baby, but it all helps him/her recover. Some of this equipment may include:
Chest tube: This will help to drain blood out of the chest area. Bleeding may still take place a few hours or even days after the surgery.
Urinary catheter: This is a small tube that makes it possible for urine to drain out of the bladder. This urine measurement is also an indication of how well the heart is functioning.
Heart monitor: This machine constantly shows what the heart is doing. It shows heart rhythm, heart rate blood pressure, and other values. This machine is usually the one that makes the beep..beep sound and is connected to most if not all patients in ICU.
Ventilator: This machine helps your child breathe. It is a small pipe that is connected to a machine and inserted into the windpipe. Some children will benefit from this machine and can stay on it for up to several days after the surgery.
Intravenous (IV) catheters: It is a small tube, sometimes called a “line” that is inserted through the skin. It may supply medicine and other fluids into your child’s body to help him/her recover.
Intra-arterial catheters: It is a specialized line that measures blood pressure.
Nasogastric (NG) tube: A flexible small tube that keeps the stomach drained of acid and gas bubbles that may build up during surgery.
Most of the TOF babies turn pink after the corrective surgery. The results in many cases are excellent and no further surgery is needed. The most important issue, where results where not optimal, is the leaking of the pulmonary valve also known as ” pulmonary regurgitation “. Pulmonary regurgitation can be treated with a tissue valve replacement.
Write down any questions you have about your child’s aftercare. This may seem so “stupid” thing to do but you won’t believe how important this is while your child is in hospital but more so when you are alone at home.
What other medicine may he/she drink in case of a cold, flu, headache, infection or what vitamins must he take. Give your child’s medication as indicated, and when you skip it for any reason call your doctor immediately.
OLDEST UNTREATED (UNCORRECTED) TOF PERSON
In 2009 there was a research report written on an 87-year-old lady that survived without any corrective surgery done to correct her TOF. According to the report, she could perform all activities related to daily life. This is very rare because patients seldom live past the age of 40 if their TOF is not corrected. There was also another report of a 73-year-old male person, with uncorrected TOF, who died of renal failure. He never attended school or played any sports and had a weak heart.
TETRALOGY OF FALLOT SUCCESS STORIES
An”old” research report of 2009 already reported two females of 83 and 78 years old who underwent repair operations late in life in their 30’s. Corrective surgery should be done earlier but in their cases, both survived and lived long lives although surgery was only done at 34 and 38.
Robert Grabel writes on Adult Congenital Heart Association and had his first surgery at 10 and his second surgery a valve replacement surgery recently. He has done 14 marathons and more than 20 half-marathons, as well as some long-distance cycle events. He is still going strong after he recently received a pacemaker defibrillator combination.
Shaun White the American professional snowboarder and skateboarder. He is a three-time Olympic gold medalist and holds the record for the most X-Games gold medals and most Olympic gold medals by a snowboarder. He holds many other records and his achievements are plentiful.
Madeline Collins graduated from West Virginia University summa cum laude with a degree in Child Development and Family Studies. She was named Miss West Virginia in 2018.
Molly Dent Davies in her own words ” Born in Kenya in 1951. They immediately knew I had a serious heart defect and I was not expected to live for long. It was a struggle to keep me alive. I survived somehow till I was 2 when I was taken by air to London and had a ‘shunt’ done by the British pioneering surgeon Lord Russel Brock which saved my life. Fast forward to 1970 when I had my repair TOF in Groote Schuur hospital done by Marius Barnard and the heart team, I was 18 years old then. I have had 3 children now 46, 43 & 33 years old and there are also 5 grandchildren. I have done well till a few years ago when I had heart arrhythmia and have had to be cardioverted.” She will have an ICD implanted soon. She has also written a book about her life “Blue Heart” and is 68 years old now.
I believe I’m also a success story. I played league squash for more than 15 years and competed in a few tournaments which demanded back to back matches on back to back days. I changed to cycling on my doctor’s suggestion and have competed in 8 Cape Town Cycle Tours of 109 km (67 miles) other long distance events and one half-marathon.
Please leave a comment or tell your TOF story.