FAQ - Tetralogy of Fallot
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What is Tetralogy of Fallot, and how can you associate this with medication?


It's four congential heart defects--http://www.americanheart.org/presenter.jhtml?identifier=11071
Treatment is surgical, if that's what you're asking.  (+ info)

Does anyone know why babies born with tetralogy of fallot (a heart defect) have failure to thrive?


Many babies with heart defects have feeding problems, and problems gaining and maintaining weight. Often the heart has to work so hard that it takes a lot of energy to keep it pumping - these babies have to eat more to compensate for their heart working harder. On the flip side, eating is an activity that takes a lot of energy. Lots of heart babies have a hard time getting all the calories they need because they get tired before they are done feeding, so they don't take in enough calories to gain weight. This can lead to failure to thrive - it's a catch-22. Some babies with TOF or other defects have feeding tubes for a while - others are on high-calorie formula to maximize the amount of calories they take in.  (+ info)

Tetralogy Of Fallot question, please only answer if you are familiar with this condition...?


My son is two months old. He was diagnosed with Tetralogy of Fallot at 1 day old. He is considered a "pink tet" (the degree of obstruction in the pulmonary tract (right ventricular outflow, pulmonary valve and pulmonary arteries is low.) however I am concerned because he at his last doctors appointment he only weighed 9 lbs 10 ounces which puts him below the 5th percentile for his weight. I know TOF babies have weight gain issues and that it can cause growth problems...does anyone know if this is true 100% of the time? Also, does anyone know of anyone or any websites where I can meet or chat with other parents going through this type of birth defect? My husband has been deployed to Iraq and I dont have anyone to talk to about my son. Any help or advice would be great. Thank you!
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I am sorry about your husband's deployment to Iraq. My prayer are with him and your family.

The tetralogy of Fallot is a congenital heart defect which classically has four anatomical components. It is the most common cyanotic heart defect and the most common cause of blue baby syndrome.
As classically described, tetralogy of Fallot involves four heart malformations which present together:

A ventricular septal defect (VSD): a hole between the two bottom chambers (ventricles) of the heart. The defect is centered around the 'outlet septum', the most superior aspect of the septum, and in the majority of cases is single and large. In some cases septal hypertrophy can narrow the margins of the defect. [1]
Pulmonic stenosis: Right ventricular outflow tract obstruction, a narrowing at (valvular stenosis) or just below (infundibular stenosis) the pulmonary valve. The stenosis is mostly the result of hypertrophy of the septoparietal trabeculae,[1] however the deviated outlet septum is believed to play a role. [2]. The degree of stenosis varies between individuals with TOF is the primary determinant of symptoms and severity. This malformation is infrequently described as sub-pulmonary stenosis or subpulmonary obstruction. [3]
Overriding aorta: defined as when the aortic valve is not restricted to the left ventricle, thus having biventricular connections. The aortic root can be moved anteriorly or override the septal defect, but it is still to the right of the root of the pulmonary artery. The degree of override is quite variable, being between 5-95% of the valve being connected to the right ventricle.[1]
Right ventricular hypertrophy: The right ventricle is more muscular than normal, causing a characteristic coeur-en-sabot (boot-shaped) appearance as seen by chest X-ray. Due to the misarrangement of the external ventricular septum, the right ventricular wall increase in size to deal with the increased obstruction to the right outflow tract. This feature is now generally agreed to be a secondary anomaly, as the level of hypertrophy generally increases with age. [4]

There is anatomic variation between the hearts of individuals with tetralogy of Fallot. The degree of right ventricular outflow tract obstruction varies between patients and is generally determines clinical symptoms and disease progression. Tetralogy of Fallot may present with other anatomical anomalies, including:

stenosis of the left pulmonary artery, in 40% of patients
a bicuspid pulmonary valve, in 40% of patients
right-sided aortic arch, in 25% of patients
coronary artery anomalies, in 10% of patients
an atrial septal defect, in which case the syndrome is sometimes called a pentalogy of Fallot
an atrioventricular septal defect
partially or totally anomalous pulmonary venous return
forked ribs and scoliosis
Tetralogy of fallot with pulmonary atresia or pseudotruncus arteriosus is a severe variant in which there is complete obstruction of the right ventricular outflow tract and absence of the pulmonary trunk. In these individuals, there is complete right to left shunting of blood. The lungs are perfused via extensive collaterals from the systemic arteries.


[edit] Epidemiology and etiology
Tetralogy of Fallot occurs in approximately 3 to 6 per 10,000 births and represents 5-7% of congenital heart defects. Its cause is thought to be due to environmental or genetic factors or a combination. It is associated with chromosome 22 deletions and diGeorge syndrome. It occurs slightly more often in males than in females.

Embryology studies show that it is a result of anterior malalignment of the conal septum, resulting in the clinical combination of a VSD, pulmonary stenosis, and an overriding aorta. Right ventricular hypertrophy results from this combination, which causes resistance to blood flow from the right ventricle.


[edit] Pathophysiology
Tetralogy of Fallot results in low oxygenation of blood due to mixing of oxygenated and deoxygenated blood in the left ventricle through the VSD and preferential flow of both oxygenated and deoxygenated blood from the ventricles through the aorta because of obstruction to flow through the pulmonary valve. This is known as a right-to-left shunt.

Children with tetralogy of Fallot may develop acute severe cyanosis or hypoxic "tet spells". The precise mechanism of these episodes is in doubt, but presumably results from an increase in resistance to blood flow to the lungs with increased preferential flow of desaturated blood to the body.


[edit] Symptoms
The primary symptom is low blood oxygen saturation with or without cyanosis from birth or developing in the first year of life. Without cyanosis, the baby is referred to as a "pink tet". Other symptoms include a heart murmur which may range from almost imperceptible to very loud, difficulty in feeding, failure to gain weight, retarded growth and physical development, dyspnea on exertion, clubbing of the fingers and toes, and polycythemia.

Tet spells are characterized by a sudden, marked increase in cyanosis, syncope, and may result in hypoxic brain injury and death.


[edit] Diagnosis
Often a simple chest x-ray is enough to determine the presence of this condition. The heart may present with a "boot-like" appearance, rather than the symmetric appearance of a normal heart.


[edit] Treatment
Tetralogy of Fallot is treated on two levels: with immediate emergency care for hypoxic or "tet" spells and with corrective surgery.


[edit] Emergency management of tet spells
Consequential acute hypoxia may be treated with beta-blockers such as propranolol, but acute episodes may require rapid intervention with morphine to reduce ventilatory drive and phenylephrine to increase blood pressure. Oxygen is ineffective in treating hypoxic spells because the underlying problem is lack of blood flow through the pulmonary circuit and not alveolar oxygenation. There are also simple procedures such as the knee-chest position which increases aortic wave reflection, increasing pressure on the left side of the heart, decreasing the right to left shunt thus decreasing the amount of deoxygenated blood entering the systemic circulation.[5]


[edit] Palliative surgery
The condition was initially thought untreatable until surgeon Alfred Blalock, cardiologist Helen B. Taussig, and lab assistant Vivien Thomas at Johns Hopkins University developed a surgical procedure, which involved forming an anastomosis between the subclavian artery and the pulmonary artery. It was actually Helen Taussig who convinced Alfred Blalock that the shunt was going to work. This redirected a large portion of the partially oxygenated blood leaving the heart for the body into the lungs, increasing flow through the pulmonary circuit, and greatly relieving symptoms in patients. The first Blalock-Taussig shunt surgery was performed on 15-month old Eileen Saxon on November 29, 1944 with dramatic results.

The Pott shunt and the Waterson procedure are other shunt procedures which were developed for the same purpose.

Currently, Blalock-Taussig shunts are not normally performed on infants with TOF except for severe variants such as TOF with pulmonary atresia.


[edit] Total surgical repair
The Blalock-Taussig procedure was the only surgical treatment until the first total repair was performed in 1954. Between 1944 and when total repair became available at major surgical centers in the early 1960s, many infants and children were treated with Blalock-Taussig procedures.

The total repair was performed by C. Walton Lillehei at the University of Minnesota in 1954 on a 10-month boy. Total repair initially carried a high mortality risk which has consistently improved over the years. Surgery is now often carried out in infants 1 year of age or younger with a <5% perioperative mortality. The surgery generally involves making incisions into the heart muscle, relieving the right ventricular outflow tract stenosis by careful resection of muscle, and repairing the VSD using a Gore-Tex or Dacron patch or a homograft. Additional reparative or reconstructive work may be done on patients as required by their particular anatomy.

Patients who have undergone "total" repair of tetralogy of Fallot often have good to excellent cardiac function after the operation with some to no exercise intolerance and have the potential to lead normal lives. Surgical success and long-term outcome greatly depends on the particular anatomy of the patient and the surgeon's skill and experience with this type of repair.


[edit] Prognosis
Untreated, tetralogy of Fallot results in progressive right ventricular hypertrophy and dilatation due to the increased resistance on the right ventricle. The dilated cardiomyopathy progresses to right heart failure, usually with accompanying left heart failure. Actuarial survival for untreated tetralogy of Fallot is approximately 75% after the first year of life, 60% by four years, 30% by ten years, and 5% by forty years.

Patients with repaired tetralogy of Fallot have the potential to lead normal lives with continued excellent cardiac function, with some considerations:

Current techniques for total surgical repair greatly improve the hemodynamic function of the heart with tetralogy of Fallot but do not provide a lifetime correction of the defect. Ninety percent of patients with total repair as infants develop a progressively leaky pulmonary valve as the heart grows to its adult size. Patients also may have some degree of residual right outflow stenosis and damage to the electrical system of the heart from surgical incisions, causing abnormalities as detected by EKG and/or arrhythmias.

Long-term follow up studies show that this patient population is at risk for sudden cardiac death and for heart failure. Therefore, lifetime follow-up care by an adult congenital cardiologist is recommended to monitor these risks and to recommend treatment, such as interventional procedures or re-operation, if it becomes necessary.

As with patients that have undergone any heart surgery, antibiotic prophylaxis is indicated during dental treatment in order to prevent infective endocarditis.


go to: http://heart.seattlechildrens.org/home/  (+ info)

Tetralogy of Fallot- heart valve replacement is necessary?


I'm 25, according to what my pediatric-cardiac doctor suggests, she thought i might have another open-heart surgery to replace my old mechanical heart valve (which has been using for 20 years since my first surgery when i was 3). Does anyone here have same condition? having an artificial heart valve and need to replace it after decades of using?
btw, i also have some syptoms that cardiologist can't explain or ignore, such as i can't swallow very well as usual (easily get choking). you people with malfunctional heart valves have what symptons? let's share. i live in Taipei, taiwan. my hospital is good at CHD surgery, it could be the best or -next-to-best in Asia. After you opinons, i think i have to be serious about my condition.
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Vongirl,

I don't blame you for asking questions on that one. Open heart surgery is a major surgery.

I googled mechanical heart valve but did not find anything related to your case. Some said a mechanical valve was designed to last forever, but face it 20 years is a long time for a mechanical device that cannot repair itself and the data was probably related to mechanical valves being put into old farts like me. At an average pulse rate of 75, 20 years would be 788,400,000 operations- 788 million. That's' a lot of flapping.

You are dealing with something that has both high cost and high consequences. What going to happen if the valve fails? If it failed would you be in good enough condition to or live long enough to replace it? Is it the valve proper size for you now? ......

I suggest that you tell your cardiologist that you would like to get a second opinion. I'm sure he'll understand and will likely appreciate it. You will need some of your records to take to the next guy.

Good Luck.  (+ info)

I have tetralogy of fallot. Should I be taking the contraceptive pill?


I have been taking it for a month, I'm on the off-week right now. I've only had normal side-effects, and my ECG was fine yesterday.
Shall I continue, ask my GP, maybe?

I had repair surgery at 18 months, and will be having replacement surgery as soon as I need it. I will stop taking it before the surgery, obviously.
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that is a serious heart defect and this is a question for only licensed healthcare providers since the pill can cause circulation issues.

you NEED to ask your GP, cardiologist AND surgeon. get at least 3 opinions from different doctors. i would ask about safer options instead of the pill  (+ info)

Congenital heart disease: Tetralogy of Fallot a maybe- my 1yr old?


my daughter who is 13 months old has been having blue episodes (with out any other symptoms: no breathing problems, no choking, no crying.)
nothing unordinary but the blueness and sometimes cranky.

but the doctor is sending her to a ped. cardiologist for an EKG & echiocardigram.

she has already had 2 heart/chest x-rays.
wouldnt it have showed up in an x-ray?
im not sure at all. just wondering.

thank you.
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A chest x-ray is the most basic of diagnostic tools. It shows only a basic outline of the heart.

I would think that if they are looking at issues that involves heart muscle and valves then they would need to do ultrasounds, echos and EKGs in order to determine whether or not she possessed the malformations described in this disorder. Or to pinpoint where the issue is if they do not find them. They may also want to do MRIs and hopefully you will get answers quickly.  (+ info)

Why do children with Tetralogy of Fallot squat?


It makes it easier for them to breath and decreases the workload on the heart.  (+ info)

tetralogy of fallot and having children?


I was born with Tetralogy Of Fallot. I have not have any problems since i was 2 yrs old. The doctor says i have no problems with my heart Thank God. Me and my boyfriend have been thinking that we should start having children and im scared if that will put me and the baby at risk. Is there anybody out there with this disease and has had children. If you have,please email me because i am very confused!!
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I've had a patient before that actually had this (repaired during childhood) and she had 2 healthy, uneventful pregnancies! She did see a Perinatologist (high risk pregnancy doctor) and her Cardiologist (heart doctor) throughout the entire pregnancy. She also delivered at a "Level 3" Hospital (equiped to handle sick babies and sick mommies). Just make sure that your OB/GYN and Cardiologist are informed about your desire to become pregnant and follow their advice! Also be prepared to have some activity restrictions placed on you during the pregnancy and immediate post-partum period (bedrest, work restrictions, etc.) if any problems do arise.

Good Luck to you!  (+ info)

is the life expectancy of ppl with Tetralogy of Fallot normal if they had a successful surgery as a baby?


Thank you so much for all of your comments I appreciate it! And littleba...you are right, live life to the fullest and appreciate everyday we are here on earth. life is too short.
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Only the surgeon can answer this. My prayers are with you if you have a baby with heart problems. My little girl had heart surgery at 31/2. She is fine now, but I thought for sure I would die and her too.  (+ info)

Is tetralogy of fallot hereditary?


My fiance has tetralogy of fallot. He has had 2 valve replacements in his life, but is 35 and overall healthy. Nobody in his family ever had this. He is very hesitant about us having children because of his battle with it (scared he won't be around) and his fear he will pass it on. He is not interested in adopting, so if the hereditary chance is high, then we will not have children at all. Any advice would be helpful. Thank you.
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It's not hereditary ... it's congenital

Hmmm... chromosome 22 deletions

I guess some forms can be inherited.

Ah - there you go - DiGeorge syndrome - genetic basis.

Dr Trisha McNair:
The cause of Fallot's tetralogy isn't fully understood. While the baby is in the womb, something interferes with the development of the heart and major blood vessels. Although no specific single genetic abnormality has yet been found to explain every case, genetics often do play a part in these types of malformations (known as conotruncal abnormalities), and in some children a particular genetic problem can be identified (for example, DiGeorge syndrome).

Some researchers have suggested that Fallot's tetralogy is caused by an autosomal recessive gene which has yet to be identified, and which has variable penetrance (that is, doesn't always cause disease). But this is far from proven, and Fallot's tetralogy has also been linked to certain medications and also to alcohol taken by the mother while pregnant. Whatever the cause, in those families who have a child with Fallot's the risk of a second child being born with the condition is only increased very slightly.  (+ info)

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