Doctor insights on:
Pentalogy Of Fallot
What is the odds of passing Tetroligy of fallot to a child? And what is the odds of passing Tetroligy of fallot to a second child?
Genetic factors: Depends on which parent was affected. In general, a mother with congenital heart disease (CHD) will have ~5% chance of transmitting CHD to a child--not necessarily TOF though. The risk of TOF will also be significantly higher than the population baseline. If there is some other underlying genetic condition (e.g., DiGeorge syndrome), the risk may be significantly higher though. ...Read more
TOF: Tetrology of Fallot is a congenital heart defect consisting of defect in the wall between the ventricles, stenosis of the pulmonary artery, enlargement of the right ventricle, and an abnormally placed aorta. It occurs in about 5 out of every 10, 000 babies. The defect affects boys and girls equally. It is corrected with open heart surgeries. ...Read more
Depends on variables: Most cases of complex congenital heart disease happen in a sporadic manner. The general risk of a parent with a chd having a child in these sporadic forms is about 6%.This is not specific to the same defect but includes all defects. When it is part of an autosomal disorder or chromosome deletion syndrome, the risks are higher. ...Read more
Heart surgery: This is a congenital heart defect knows at "tetralogy" because the initial description discussed four findings: 1) ventricular septal defect, 2) overriding aorta, 3) pulmonary stenosis, 4) right ventricular hypertrophy. The cause is abnormal conotruncal septation, giving too much to the outflow to the aorta and not enough to the outflow to the lungs. This defect always requires surgical repair. ...Read more
Congenital heart dis: This is a congenital heart defect knows at "tetralogy" because the initial description discussed four findings: 1) ventricular septal defect, 2) overriding aorta, 3) pulmonary stenosis, 4) right ventricular hypertrophy. The cause is abnormal conotruncal septation, giving too much to the outflow to the aorta and not enough to the outflow to the lungs. This defect always requires surgical repair. ...Read more
Heart Surgery: Tof is a complex type of congenital heart disease characterized by a large hole in the heart (vsd) and obstruction of pulmonary blood flow (pulmonary stenosis). Babies with tof require heart surgery during infancy. The surgery involves closing the hole and establishing unobstructed pulmonary blood flow. Survival rates are very good. ...Read more
Microdeletion of chromosome 22q11 (digeorge syndrome) is a genetic abnormality that has been linked to tof and other forms of congenital heart defects. It also can occur with Down syndrome (trisomy 21), and in patients with no identified genetic abnormality. Heart defects occur in 0.8% of babies, but in 3-5% of those with 1st degree relatives with heart defects.
There is a genetic predisposition. ...Read more
Not typically.: Most cases are isolated and sporadic. It accounts for 5-10% of all congenital heart defects. 15% of tof cases are part of known genetic syndromes (down, digeorge, alagille, phenylketonuria) or associations (charge/vacterl) and some are linked to poorly controlled preconceptional maternal diabetes mellitus. Recurrence risk: 2.5% with one prior affected child, 8% with two. Folic acid may prevent it! ...Read more
We don't know: In most cases tetralogy of fallot is thought to be "multifactorial", meaning that there are possibly genes, and also environmental factors which might increase a child's potential of having the defect. ...Read more
Depends quite a bit: For children who have have non-emergency surgery at < 1 year old at a hospital that does at least 200 childhood heart surgeries per year, the risk of death is probably about 1%. Other severe risks such as stroke, severe infection or need for pacemaker are uncommon, but occur. The surgeon will give you more accurate info. Without surgery 50% of children die by 6 years of age. ...Read more
Tetralogy of Fallot: The answer depends on the type of operation. Typically tof surgery involves ventricular septal defect closure, right ventricular muscle bundle division/resection, and relief of any pulmonary valve stenosis (with a transannular patch or right ventricle to pulmonary artery conduit). Risks of the surgery include residual vsd, pulmonary stenosis, pulmonary insufficiency, and ventricular dysfunction. ...Read more
TOF: Tof is a condition where there is a vsd (hole between bottom two pumping chambers or ventricle), a displaced aorta, right ventricular hypertrophy and restriction of blood flow to lungs. The early course is usually determined by amount of blood flow to lungs. If restricted, the child will appear blue (cyanotic), breathe fast, be very irritable. A child at baseline does not have pain. ...Read more
TETRALOGY OF FALLOT: The tet spells are sudden lethal episodes of severe cyanosis and dyspnea or hyperpnea leading to seizures and occacionally death. ...Read more
Condition with this American Heart Association definition: heart defect that features four problems.
a hole between the lower chambers of the heart
an obstruction from the heart to the lungs
The aorta (blood vessel) lies over the hole in the lower chambers
The muscle surrounding the lower right chamber becomes overly thickened ...Read more
If your son has: A tetrology of fallot, he should be followed closely by a pediatric cardiologist pre and post op. Speak first with your peds cardiologist about any medications you would consider using. ...Read more
Fairly accurate: In experienced centers, a fetal diagnosis of tetralogy of fallot should be reasonably accurate, although some of the specific anatomic details that may have an impact on the timing and type of surgical approach may not be readily apparent until after birth. ...Read more
Several hours: The thinking nowadays is to try and correct the defect in the newborn period if possible or at least within the first 2-3 months. In the past tetralogy of fallot was repaired in two steps, a simple surgery within the first several months to connect blood from a branch of the aorta to the lung arteries followed by complete repair at 3-5 years of age. ...Read more
If my daughter had tetralogy of fallot, is it likely that my future children will have similar problems?
A 9 year old boy has undergone vsd closure for the tetrology of fallot (now his age is 32)what complications may occur in his future? How they manage?
Multiple Issues: Patients with repaired tof have many potential issues including heart rhythm problems, recurrent pulmonary artery obstruction, and pulmonary valve issues. The specific problems depend on the types of surgery as well as the individual. Please discuss these issues with the cardiologist to understand the specific issues in this man's case. ...Read more
Genetics and TOF: The short answer is yes. TOF is the most common of presenting congenital heart disease and multiple studies have been done trying to determine and isolate various genetic mutations. In a paper published in 2006, 20% of the patients with TOF were identified with genetic mutations. ...Read more
Tetralogy of fallot:
Is a congenital malformation. Uneven septation of the embryological troncus: small PA, larger Aorta with overriding the VSD, RV hypertrophy, presence of a VSD,
Obstruction of the RV outflow, diminished blood flow to the lungs, usually requires an arterial shunt to the PA to increase blood flow in to the lungs, the surgery is done as neonatal. Total repair by the age of 4-5 years. ...Read more
YES: Tetralogy of fallot is repaired surgically and patients do very well. ...Read more