Tetralogy of Fallot
You constantly question yourself asking, “Is this a heart thing or just a baby thing - Christina Schuetz
image by: Doreen Marshall
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'I know my son will never be normal'
Your baby has a life threatening heart defect." Those are some of the most frightening words you can hear during pregnancy...
And so you are catapulted into the world of congenital heart disease, where suddenly your baby is "high risk" and all the talk is of your "special child", survival rates and surgery, not morning sickness, back pain and whether to have a natural labour.
It was at the 22 week anomaly scan that my world came crashing down. The sonographer was unhappy with the ultrasound and referred us immediately to a foetal heart specialist. After a detailed scan of the baby's heart we were whisked into a small side room. It was obvious something was seriously wrong.…
Resources
What is the Tetralogy of Fallot? | 8 Things You Should Know About TOF
Tetralogy of Fallot (TOF) is a complex congenital heart defect. It is one of the most common anomalies of heart present at birth. These are the eight things you should know about this disease...
Hypercyanotic Episodes in the Newborn
A 26 day-old baby boy has been brought to the emergency department by ambulance. The past few days he has had poor feeding and recurrent episodes of respiratory distress associated with ‘turning blue’. He was diagnosed with Tetralogy of Fallot antenatally and was born at term (a normal vaginal birth). An echocardiogram 2 days after he was born confirmed the diagnosis – it also showed that his ductus arteriosus had closed. He was discharged from hospital 4 days after he was born. On presentation, the baby is agitated, tachycardic, tachypneic and profoundly cyanotic.
Hypercyanotic Spells
We are all familiar with Tetralogy of Fallot, primarily because it is a great topic for test writers to torture us with questions about. One of the cool things about pediatric cardiology is that it really makes you consider physiology and plumbing… ok… maybe I’m just a geek. But, before you judge me too harshly, let us consider the Hypercyanotic Spell (aka, ‘Tet spell’).
Shaun White's heart condition inspires an army of loyal fans
White suffers from a heart condition called Tetralogy of Fallot -- which is made up of four congential heart defects -- and has undergone three separate operations to repair his heart. White's scar from the surgeries is clearly visible in his commercial for the Super Bowl, square in the middle of his chest.
Billy Kimmel's Rare Heart Condition Explained
A number of cardiac defects turned an “easy” delivery into a race to save the life of Jimmy Kimmel’s newborn son. Tetralogy of Fallot—named after French physician Étienne-Louis Arthur Fallot—is characterized by four malformations that occur in the heart as a fetus develops.
I Haven’t Let Heart Disease Stop Me From Enjoying Life
I encourage people to adjust to heart health just as they would something like poor eyesight—but not let it define them. I may not be able to run a marathon, but I can still go out and play with my kids.
More on Ruby's Heart Defect (Tetralogy of Fallot)
Basically it is a hole in the heart that is causing other areas of the heart to become abnormal. The hole is called a VSD, or a ventricular septal defect, or a hole in the septum that divides the right and left chambers.
Tetralogy of Fallot
Patients nowadays usually present as neonates, with cyanosis of varying intensity based on the degree of obstruction to flow of blood to the lungs. The aetiology is multifactorial, but reported associations include untreated maternal diabetes, phenylketonuria, and intake of retinoic acid
Tetralogy of Fallot – Our Story
A “tet” heart can be fully repaired but the child has to be bigger in order to be able to have enough maneuverability to fix what is broken.
The Day-to-Day Life of a Child with Tetralogy of Fallot
So, allow me to talk about the day-to-day life of a kid with uncomplicated ToF (meaning he has no additional conditions like hypertension, etc.). Our day-to-day life with Cooper – now age eight and home for four years – involves a lot of the following...
'I know my son will never be normal'
Your baby has a life threatening heart defect." Those are some of the most frightening words you can hear during pregnancy.
6 Things you Should Know If Your Child is Having Surgery
Having gone through the harrowing experience that is watching your child undergo surgery, here are six things I wish someone had told me...
Bert's Box: Wired for Life
Bert's Box: Wired for Life is about Randy Hunt’s life-long adventures with a dangerous heart condition─tetralogy of fallot─and adjusting to a world that he couldn’t seem to keep up with
I Heart Sophia
The NICU doctors original diagnosis was off a little bit, but Sophia did have a pulmonary heart defect. This would be the first time of many the words Tetralogy of Fallot would be spoken in relation to our precious daughter. Plans were made to move her to the local children’s hospital the following morning.
My Miracle Anniek Who Has Tetralogy Of Fallot
I am the Mommy of a special little girl (Anniek)...she was born with a Congenital Heart Defect (Tetralogy of Fallot), has a communication based learning disability and is on a feeding tube (g-tube). She lights up my world and means everything to me.
Our Little Braveheart
William Jacob Brown – “Jake” was born on September 17, 1999. He was 7 weeks premature, but otherwise healthy….or so we thought until September 20. It was then that we discovered Jake had a congenital heart defect called Tetralogy of Fallot (TOF). Our world crumbled that day. We didn’t know why this had happened or if Jake would even survive. We vowed that we would do whatever we had to do in order for Jake to live a normal life. But getting there meant we had some hurdles to clear. We are not doctors or medical professionals. We are simply parents who have been there. We hope this blog will help give hope to other parents who are just starting the journey.
The Irvine Family
So, because this blog won’t be entirely dedicated to our child’s CHD as some others have (which isn’t a bad thing at all!) I decided to create a space for those only wanting to read our heart journey, and put all posts relating to it, into one easy-to-find area.
PedsCases
The management for Tet spells can be divided into two categories – supportive measures performed by the parent/guardian and medical interventions provided by a physician: • Supportive measures that can be performed by a parent/caregiver: 1. Place the child in a knees-to-chest position. This increases systemic resistance, reducing the right to left shunting 2. Have a caregiver hold the child. This prevents further agitation and may help him/her calm down • Medical interventions: 1. Supply oxygen. This is important because low oxygen saturation is causing the cyanosis 2. Morphine can help calm the child and also reduce pulmonary vascular resistance 3. Phenylephrine, an alpha-1 adrenergic receptor agonist, is sometimes used in the hospital setting to increase systemic vascular resistance, therefore promoting blood flow through the pulmonary trunk to the lungs for oxygenation.
CDC
This heart defect can cause oxygen in the blood that flows to the rest of the body to be reduced. Infants with tetralogy of Fallot can have a bluish-looking skin color―called cyanosis―because their blood doesn’t carry enough oxygen. At birth, infants might not have blue-looking skin, but later might develop sudden episodes of bluish skin during crying or feeding. These episodes are called tet spells.
Children’s Hospital of Philadelphia
Surgery is required to repair tetralogy of Fallot. Typically in the first few months of life we will perform open heart surgery to patch the hole (VSD) and widen the pulmonary valve or artery. In some cases, depending on the unique needs of the patient, we will perform a temporary repair until a complete repair can be done. The temporary repair involves connecting the pulmonary arteries (which carry blood from heart to lungs) with one of the large arteries that carry blood away from the heart to the body. This increases the amount of blood that reaches the lungs, and so increases the amount of oxygen in the blood.
Cincinnati Children’s
Corrective repair of tetralogy of Fallot involves closure of the ventricular septal defect with a synthetic Dacron patch so that the blood can flow normally from the left ventricle to the aorta. The narrowing of the pulmonary valve and right ventricular outflow tract is then augmented (enlarged) by a combination of cutting away (resecting) obstructive muscle tissue in the right ventricle and by enlarging the outflow pathway with a patch. In some babies, however, the coronary arteries will branch across the right ventricular outflow tract where the patch would normally be placed. In these babies an incision in this area to place the patch would damage the coronary artery so this cannot safely be done. When this occurs, a hole is made in the front surface of the right ventricle (avoiding the coronary artery) and a conduit (tube) is sewn from the right ventricle to the bifurcation of the pulmonary arteries to provide unobstructed blood flow from the right ventricle to the lungs.
Cove Point Foundation
Tetralogy of Fallot accounts for 10% of the cases of congenital heart disease. It is the most common cyanotic (blue) heart defect beyond infancy and involves four (Greek tetra = four) anomalies of the structure of the heart: 1) A large ventricular septal defect (VSD), or hole, in the septum (muscle wall) which separates the right and left ventricles 2) A narrowing (stenosis) of the outflow tract (infundibular stenosis) from the right ventricle into the pulmonary artery (2a) and/or pulmonary valve narrowing (2). 3) The aorta is enlarged and "overrides," or sits directly above, the ventricular septal defect (VSD). 4) A thickening of the muscle wall of the right ventricle resulting in a right ventricular hypertrophy (thickening). A right sided aortic arch is present in 1/4 to 1/3 of patients.
Patient
The original definition consists of four main anatomical features: a large ventricular septal defect (anterior malaligned), overriding aorta, right ventricular outflow obstruction and right ventricular hypertrophy. However, the two key abnormalities are: •A large ventricular septal defect, which allows the pressures in the two ventricles to become equal. •Right ventricular outflow obstruction. There is also right-sided aortic arch in around 20% of cases and atrial septal defect in 8-10% - pentalogy of Fallot.
The Patient Guide to Heart, Lung, and Esophageal Surgery
Some babies with TOF can have very low blood oxygen levels soon after birth. If your baby is too weak or too small to have full repair at this stage, the surgeon may perform an initial surgery (shunt procedure) to help increase blood flow to the lungs and give him or her time to grow and get strong enough for the full repair. Complete corrective surgery is done later in life.
UCSF Medical Center
Now, most babies who have tetralogy of Fallot have their defects fully repaired in infancy. However, some babies are too weak or too small to have the full repair. They must have temporary surgery first. This surgery improves oxygen levels in the blood. It also gives the baby time to grow and get strong enough for the full repair. In the temporary surgery, the surgeon places a tube called a shunt between a large artery branching off the aorta and the pulmonary artery. One end of the shunt is sewn to the artery branching off the aorta. The other end is sewn to the pulmonary artery. The shunt creates an additional pathway for blood to travel to the lungs to get oxygen. The shunt is removed when the baby's heart defects are fixed during the full repair.
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