TATROLOGY OF FALLOT

INTRODUCTION
Tetralogy of Fallot (teh-TRAL-uh-jee of fuh-LOW) is a rare condition caused by a combination of four heart defects that are present at birth (congenital).

These defects, which affect the structure of the heart, cause oxygen-poor blood to flow out of the heart and to the rest of the body. Infants and children with tetralogy of Fallot usually have blue-tinged skin because their blood doesn’t carry enough oxygen.

Tetralogy of Fallot is often diagnosed during infancy or soon after. However, tetralogy of Fallot might not be detected until later in life in some adults, depending on the severity of the defects and symptoms.

With early diagnosis followed by appropriate surgical treatment, most children and adults who have tetralogy of Fallot live relatively normal lives, though they’ll need regular medical care throughout life and might have restrictions on exercise.

SYMPTOMS
Tetralogy of Fallot symptoms vary, depending on the extent of obstruction of blood flow out of the right ventricle and into the lungs. Signs and symptoms may include:

A bluish coloration of the skin caused by blood low in oxygen (cyanosis)
Shortness of breath and rapid breathing, especially during feeding or exercise
Loss of consciousness (fainting)
Clubbing of fingers and toes — an abnormal, rounded shape of the nail bed
Poor weight gain
Tiring easily during play or exercise
Irritability
Prolonged crying
A heart murmur
Tet spells
Sometimes, babies who have tetralogy of Fallot will suddenly develop deep blue skin, nails and lips after crying or feeding, or when agitated.

These episodes are called tet spells and are caused by a rapid drop in the amount of oxygen in the blood. Tet spells are most common in young infants, around 2 to 4 months old. Toddlers or older children might instinctively squat when they’re short of breath. Squatting increases blood flow to the lungs.

Seek medical help if you notice that your baby has the following symptoms:

  • Difficulty breathing
  • Bluish discoloration of the skin
  • Passing out or seizures
  • Weakness
  • Unusual irritability
  • If your baby becomes blue (cyanotic), place your baby on his or her side and pull your baby’s knees up to his or her chest. This helps increase blood flow to the lungs. Call 911 or your local emergency number immediately.

CAUSES

tatrology of fallot
  Tatrology of fallot

Tetralogy of Fallot occurs during fetal growth, when the baby’s heart is developing. While factors such as poor maternal nutrition, viral illness or genetic disorders might increase the risk of this condition, in most cases the cause of tetralogy of Fallot is unknown.

The four abnormalities that make up the tetralogy of Fallot include:

  • PULMONARY VALVE STENOSIS– Pulmonary valve stenosis is a narrowing of the pulmonary valve — the valve that separates the lower right chamber of the heart (right ventricle) from the main blood vessel leading to the lungs (pulmonary artery).

Narrowing (constriction) of the pulmonary valve reduces blood flow to the lungs. The narrowing might also affect the muscle beneath the pulmonary valve. In some severe cases, the pulmonary valve doesn’t form properly (pulmonary atresia) and causes reduced blood flow to the lungs.

  • VENTRICULAR SEPTAL DEFECT- A ventricular septal defect is a hole (defect) in the wall (septum) that separates the two lower chambers of the heart -the left and right ventricles. The hole allows deoxygenated blood in the right ventricle — blood that has circulated through the body and is returning to the lungs to replenish its oxygen supply — to flow into the left ventricle and mix with oxygenated blood fresh from the lungs.

Blood from the left ventricle also flows back to the right ventricle in an inefficient manner. This ability for blood to flow through the ventricular septal defect reduces the supply of oxygenated blood to the body and eventually can weaken the heart.

  • OVERRIDING AORTA– Normally the aorta — the main artery leading out to the body — branches off the left ventricle. In tetralogy of Fallot, the aorta is shifted slightly to the right and lies directly above the ventricular septal defect.

In this position the aorta receives blood from both the right and left ventricles, mixing the oxygen-poor blood from the right ventricle with the oxygen-rich blood from the left ventricle.

  • RIGHT VENTRICULAR HYPERTROPHY- When the heart’s pumping action is overworked, it causes the muscular wall of the right ventricle to thicken. Over time this might cause the heart to stiffen, become weak and eventually fail.
    Some children or adults who have tetralogy of Fallot may have other heart defects, such as a hole between the heart’s upper chambers (atrial septal defect), a right aortic arch or abnormalities of the coronary arteries.

RISK FACTOR
While the exact cause of tetralogy of Fallot is unknown, various factors might increase the risk of a baby being born with this condition. These risk factors include:

  • A viral illness during pregnancy, such as rubella (German measles)
  • Alcoholism during pregnancy
  • Poor nutrition during pregnancy
  • A mother older than age 40
  • A parent who has tetralogy of Fallot
  • The presence of Down syndrome or DiGeorge syndrome

COMPLICATIONS
All babies who have tetralogy of Fallot need corrective surgery. Without treatment, your baby might not grow and develop properly.

Your baby may also be at an increased risk of serious complications, such as infective endocarditis — an infection of the inner lining of the heart or heart valve caused by a bacterial infection.

Untreated cases of tetralogy of Fallot usually develop severe complications over time, which might result in death or disability by early adulthood.

PHYSICAL THERAPY PHYSICAL THERAPY
PRE-OPERATIVE – Most infants with TOF will receive thoracic surgery to correct the associated abnormalities. The most important physical therapy intervention pre-operative for the patient and family is education. Many institutes are using a method of education that describes a pre-operative program that helps to decrease post-operative complications. Many institutions use a doll to explain to children about the tubes and how to care for them.

POST-OPERATIVE– Pulmonary function is a main area of concentration after thoracic surgery. The interventions vary with age but the general goal is the same, to mobilize secretions, increase aeration and increase general mobility. Atelectasis is a concern after thoracic surgery and is caused by the slowed mucous transport, altered breathing patterns, prolonged positioning in supine and diaphragmatic dysfunction in early post-operative period. The yawn maneuver and prolonged inspiration to increase inflation are ways to prevent atelectasis. The incentive spirometry is an effective tool for preventing atelectasis in the pediatric population. Other ways that can be more fun for children are activities such as blowing bubbles or blowing on a windmill. There are expiratory maneuvers but the children typically take a large breath before blowing, thus they become inspiratory maneuvers also.

Segmental expansion techniques are effective to increase segmental aeration. These techniques are performed by placing your hand over whichever segment of the lung is not effectively inflating and allowing your hand to move with the respiratory cycle. Gentle pressure may be applied to the chest at the end of the expiratory phase, just before the inhalation phase. This elicits a stretch reflex that facilitates air flow to that particular segment.

Percussion and vibration may be performed in conjunction with segmental expansion. Percussion is the rhythmic clapping with cupped hands over the involved lung segment performed throughout the respiratory cycle, with the goal of mechanically dislodging pulmonary secretions. Vibration is performed by creating a fine oscillating movement of the hands on the chest wall just before expiration begins and throughout the expiration phase. If the child does not tolerate percussion and vibration, placing the patient in side lying and gently rocking back and forth may stimulate expansion, secretion motion and relaxation. This technique is affective if the child is upset and may decrease respiratory rate.
Positioning may be useful for patients, but certain positions may be contraindicated post thoracic surgery. Check with the MD or nurse before using positioning to make sure that it is safe for the child.

POSTURAL DRAINAGE
If the child is intubated suctioning may be used to remove secretions from the airways, however children with cyanotic defects (such as TOF) tend to desaturate during suctioning and it is very important to hyperventilate these patients before and after suctioning and to monitor oxygen levels.

Coughing is an effective way to mobilize secretions if the patient is able and willing to cough. A teddy bear or pillow can be used by the child to self-splint the incision. Have the child squeeze the stuffed animal against their chest to decrease pain.

Range of motion (ROM) exercises should be initiated as soon as possible after surgery. ROM exercises are extremely important with thoracic surgery because this type of incision tends to produce more guarding. Child position should be changed regularly to avoid a pooling of secretions in the dependent part of the lungs and regular position changes reduce the risk of fevers after surgery. Ambulation should be implemented as soon as possible to decrease both pulmonary and circulatory complications. As soon as atrial lines and groin lines are removed, patients should begin to ambulate, even if only 5-10 ft.

DIFFERENTIAL DIAGNOSIS
Any other cyanotic heart defects, such as, pulmonary stenosis, transposed arterial trunks, common arterial trunk and tricuspid atresia. Patients with heart murmurs will also be tests for persistent pulmonary hypertension.

 

 

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coactaction of aorta

Coarctation (ko-ahrk-TAY-shun) of the aorta — or aortic coarctation — is a narrowing of the aorta, the large blood vessel that branches off your heart and delivers oxygen-rich blood to your body. When this occurs, your heart must pump harder to force blood through the narrowed part of your aorta.
Coarctation of the aorta is generally present at birth (congenital). The condition can range from mild to severe, and might not be detected until adulthood, depending on how much the aorta is narrowed.

Coarctation of the aorta often occurs along with other heart defects. While treatment is usually successful, the condition requires careful lifelong follow-up.

SYMPTOMS
Coarctation of the aorta symptoms depend on the severity of the condition. Most people don’t have symptoms. Children with serious aortic narrowing may show signs and symptoms earlier in life, but mild cases with no symptoms might not be diagnosed until adulthood. People may also have signs or symptoms of other heart defects that they have along with coarctation of the aorta.

Babies with severe coarctation of the aorta may begin having signs and symptoms shortly after birth. These include:

Pale skin
Irritability
Heavy sweating
Difficulty breathing
Difficulty feeding
Left untreated, aortic coarctation in babies might lead to heart failure or death.

Older children and adults with coarctation of the aorta often don’t have symptoms because their narrowing may be less severe. If you have signs or symptoms that appear after infancy, you most commonly will have high blood pressure (hypertension) measured in your arms. However, your blood pressure is likely to be lower in your legs. Signs and symptoms might include:

High blood pressure
Headache
Muscle weakness
Leg cramps or cold feet
Nosebleeds
Chest pain
When to see a doctor
Seek medical help if you or your child has the following signs or symptoms:

Severe chest pain
Fainting
Sudden shortness of breath
Unexplained high blood pressure
While experiencing these signs or symptoms doesn’t necessarily mean that you have a serious problem, it’s best to get checked out quickly. Early detection and treatment might help save your life.

CAUSES
Doctors aren’t certain what causes coarctation of the aorta (aortic coarctation). For unknown reasons, mild to severe narrowing develops in part of the aorta. Although aortic coarctation can occur anywhere along the aorta, the coarctation is most often located near a blood vessel called the ductus arteriosus. The condition generally begins before birth (congenital). Congenital heart defects are the most common of all birth defects.

Rarely, coarctation of the aorta develops later in life. Traumatic injury might lead to coarctation of the aorta. Rarely, severe hardening of the arteries (atherosclerosis) or a condition causing inflamed arteries (Takayasu’s arteritis) can narrow the aorta, leading to aortic coarctation.

Coarctation of the aorta usually occurs beyond the blood vessels that branch off to your upper body and before the blood vessels that lead to your lower body. This can often lead to high blood pressure in your arms but low blood pressure in your legs and ankles.

With coarctation of the aorta, the lower left heart chamber (left ventricle) of your heart works harder to pump blood through the narrowed aorta, and blood pressure increases in the left ventricle. This may cause the wall of the left ventricle to thicken (hypertrophy).

RISK FACTORS
Coarctation of the aorta often occurs along with other congenital heart defects, although doctors don’t know what causes multiple heart defects to form together. The condition is more common in males than in females. You or your child may be more likely to have aortic coarctation if certain heart conditions exist, including:

1.Bicuspid aortic valve. The aortic valve separates the lower left chamber (left ventricle) of the heart from the aorta. A bicuspid aortic valve has two flaps (cusps) instead of the usual three. Many people with coarctation of the aorta have a bicuspid aortic valve.
Patent ductus arteriosus. Before birth, the ductus arteriosus is a blood vessel connecting the left pulmonary artery to the aorta — allowing blood to bypass the lungs.

Shortly after birth, the ductus arteriosus usually closes. If it remains open, it’s called a patent ductus arteriosus.

Holes in the wall between the left and right sides of the heart. You may have a hole in the wall (septum) between the upper chambers of the heart (atrial septal defect) or the lower chambers of the heart (ventricular septal defect) when you’re born.

This causes oxygen-rich blood from the left side of the heart to mix with oxygen-poor blood in the right side of the heart.

2.Aortic valve stenosis. This is a narrowing of the valve that separates the left ventricle of the heart from the aorta (aortic valve). This means your heart has to pump harder to get adequate blood flow to your body.

Over time, this can cause your heart muscle to thicken and lead to symptoms such as chest pain, fainting spells and breathlessness, or heart failure.

3.Aortic valve regurgitation. This occurs when the aortic valve doesn’t close tightly, causing blood to leak backward into the left ventricle.
Mitral valve stenosis. This is a narrowing of the valve (mitral valve) between the upper left heart chamber (left atrium) and the left ventricle that lets blood flow through the left side of your heart.

In this condition, blood may back up into your lungs, causing shortness of breath or lung congestion. Like aortic valve stenosis, this condition can also lead to heart failure.

4.Mitral valve regurgitation. This occurs when the mitral valve doesn’t close tightly, causing blood to leak backward into the left atrium.
Coarctation of the aorta is also more common in people who have certain genetic conditions, such as Turner syndrome. Women and girls with Turner syndrome have 45 chromosomes, with one missing or incomplete X chromosome, instead of 46. About 10 percent of women and girls with Turner syndrome have aortic coarctation.

COMPLICATIONS
Untreated coarctation of the aorta frequently leads to complications. Some complications may be a result of long-standing high blood pressure caused by the aortic coarctation. Complications are also possible after treatment for coarctation of the aorta.

Complications of coarctation of the aorta may include:

Narrowing of the aortic valve (aortic stenosis)
High blood pressure
Stroke
Enlargement in a section of the wall of the aorta (aneurysm)
Aortic rupture or tear (dissection)
Premature coronary artery disease — narrowing of the blood vessels that supply the heart
Heart failure
A weakened or bulging artery in the brain (brain aneurysm) or bleeding in the brain (hemorrhage)
In addition, if the coarctation of the aorta is severe, your heart might not be able to pump enough blood to your other organs. This can cause damage to your heart and also can result in kidney failure or other organ failure.

If your coarctation of the aorta was treated when you were young, you have a risk of the aorta re-narrowing (re-coarctation) over time. You also have a higher risk of developing high blood pressure. You will need lifelong follow-up for coarctation of the aorta, and you may require additional treatments.

PREVENTION
Coarctation of the aorta can’t be prevented, because it’s usually present at birth (congenital). However, if you or your child has a condition that increases the risk of aortic coarctation, such as Turner syndrome, bicuspid aortic valve or another heart defect, or a family history of congenital heart disease, early detection can help.
PHYSIOTHERAPYPHYSIOTHERAPY IN PRE- AND POSTOPERATIVE PERIOD

Physiotherapy in the pre- and postoperative period is indicated in pediatric cardiac surgery in order to reduce the risk of pulmonary complications (retention of secretions, atelectasis and pneumonia) as well as to treat such complications as it contributes to the appropriate ventilation and successful extubation .

In the preoperative, physiotherapy uses techniques of clearance, reexpansion, abdominal support and guidance on the importance and objectives of physiotherapy intervention for parents or escorts, or patients able to understand such guidance . The techniques used by postoperative physiotherapy include vibration in the chest wall, percussion , compression , manual hyperinflation , reexpansion maneuver , positioning postural drainage , cough stimulation, aspiration , breathing exercises , mobilization and AEF (acceleration of expiratory flow) .

There are few current studies on the role of physiotherapy in the postoperative of pediatric cardiac surgery , especially those that approach the effectiveness of physiotherapy in the preoperative to prevent pulmonary complications after heart surgery.

Felcar et al. performed a study with 141 children with congenital heart disease, aged varying between one day old to six years, randomly divided into two groups, whereas one of them received physiotherapy in the pre- and postoperative and the other only postoperatively. The study obtained statistically significant difference regarding the presence of pulmonary complications (pneumonia and atelectasis), being more frequent in the group undergoing physiotherapy only postoperatively. Moreover, when the presence of pulmonary complications was associated with other complications regarding the time of hospital stay, such as sepsis, pneumothorax, pleural effusion and others, the group that received physiotherapy before and after surgery showed a lower risk of developing such complications. These findings demonstrate the importance of preventive action of physiotherapy preoperatively.

The study by Main et al. compared the effectiveness of aspiration with the techniques of respiratory physiotherapy (manual vibration, percussion, compression, manual hyperinflation, positioning and postural drainage) in 83 participants with a mean age of 9 months. The respiratory parameters (expiratory tidal volume, resistance and lung compliance) were measured 15 minutes before treatment and after 30 minutes, and lasting for 60 minutes after the intervention in case there was no need for clinical intervention. The duration of physiotherapy was higher than the aspiration . It was observed that physiotherapy tends to produce improvement in expiratory tidal volume, pulmonary compliance and pulmonary resistance.

According to Kavanagh , the treatment for atelectasis consists of physiotherapy, deep breathing, incentive spirometry. However, sometimes, atelectasis is difficult to reverse and it is necessary association with another method, as in the case report from Silva et al. , in which a child with congenital heart disease underwent heart surgery and developed this pulmonary complication after extubation in the postoperative period and the reversal of this presentation was achieved after the association of respiratory physiotherapy with inhalation of hypertonic saline solution with NaCl at 6%.

Chest radiographs and four physiotherapy sessions lasting 20 minutes were performed daily in this study, using maneuvers of pulmonary reexpansion and bronchial hygiene, bronchial postural drainage and tracheal aspiration. Immediately before and after physiotherapy inhalation of hypertonic saline solution with NaCl at 6% was associated. The authors found that this association was shown to be effective in this case .

Breathing exercises are indicated in cases of atelectasis due to thoracic or upper abdominal surgery, because they improve the respiratory efficiency, increase the diameter of the airways, which helps to dislodge secretions, preventing alveolar collapse, and facilitating the expansion of the lung and peripheral airways clerance .

Campos et al. analyzed the effect of increased expiratory flow (IEF) in heart rate, respiratory rate and oxygen saturation in 48 children diagnosed with pneumonia. The variables were assessed before physiotherapy, in the first and fifth minutes after physiotherapy. The authors found a statistically significant increase in oxygen saturation and statistically significant reduction in cardiac and respiratory rate after intervention with IEF and concluded that this physiotherapeutic technique for bronchial hygiene is effective in improving lung function.