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Blue Babies

Tetralogy of Fallot

What is the Tetralogy of Fallot?

We have learned the normal structure of heart and its functioning. Tetralogy of Fallot is a congenital problem of the
babies. The wall between the two ventricles has a large hole (ventricular septal defect) and at the same time, passage
of right heart leading to lung artery (pulmonary artery) is narrow and obstructive. Systemic artery (aorta) is pushed
towards the right ventricle. As the effect of the changes in anatomy cardiac hemodynamics also changes. Due to
obstructive RV outflow the impure blood mixes with the pure blood and distributed in the body through the aorta.
The RV becomes thickened due to overwork done against the narrow outflow. The lungs are deprived of share of
blood flow what they usually receive. Hence , amount of total desaturated blood is increased in comparison to the
saturated blood and also the desaturated blood can easily enter in the body through VSD, giving rise to classical
blue tinge of body (central cyanosis) and thickening of finger tips (Clubbing).

What is the classical component of tetralogy of Fallot?

Classically a baby with the TOF has a large VSD with overriding aorta, severe obstruction at RVOT and
hypertrophied RV. There might be additional problems like additional VSD, ASD, small or absent pulmonary
arteries. It is essential to recognize the additional problems for the proper management.

What are the classical clinical features of tetralogy of Fallot?

A baby born with the classical TOF may be diagnosed in first few days due to duskiness or detection of murmur.
However, the babies with classical TOF do not become symptomatic very early. These babies may suffer from
gradual deepening of cyanosis, growth failure, delayed walking. Most catastrophic presentation of TOF babies is
known as hypercyanotic or tet spell which can be life threatening also. The untreated infants may suffer from
thickening of blood (polycythemia)

What are the tet or hypercyanotic spells?

The spells are the alarm bell in a baby with TOF. The spells are characterized by irritability, crying, and sudden
deepening of cyanosis, deep breathing (hyperventilation), exhaustion, sleep or loss of consciousness. Usually it
occurs in infants. It may be life threatening or may cause long term brain damage.

What one must do in presence of tet spell?

Mother should take the baby in lap or keep him in bed at knee chest posture. Bending at knee and hip helps the child
in improving the blood flow to the lungs. Baby must be taken to the hospital as he would need sedation, intra venous
fluid, oxygen, drugs like betablocker infusion and sometimes ventilator support.
A baby with the predisposition must be given iron to keep hemoglobin above 13 gm/dl, must be on regular
betablockers. A single episode of spell in absence of any other factor like anemia, dehydration or fever is an
indication for surgery.

Why the tetralogy of Fallot occur? How we can prevent it?

The cause is usually unknown. Genetic factors can sometimes play a role. The consanguinity may play a major role
in exposing the disease in genetically predisposed population. The drugs, radiation or some other factors also have
some role to play. The fetal echo done at early gestation and early detection of disease may play some role in
management.

Is natural or medical cure is possible in TOF patients?

No. TOF is essentially a complex disease and unlike the VSD, ASD or PDA natural process can not heal it. The
medical therapy is to support the baby from ill effects of physiological response of body like use of beta blockers.
But they are not curative.

Why the un-operated TOF babies have higher risk for long term problems?

TOF patients are essentially lacking in adequate oxygen supply in relation to the demand. Hence there growth,
development and exercise capacities are grossly abnormal. Not uncommonly, there may be neurological problems
secondary to transfer of infective embolus from right side to the brain. Babies with thickened blood (polycythemia)
may also have neurological problems.

Some babies may have associated chromosomal anomalies or involvement of other organs like kidneys and may
suffer because of that also.

Is surgical correction is unavoidable?

In absence of natural cure and an effective medical therapy, surgical correction is mandatory.

Can surgery be performed in a single stage?

Typically, the single staged surgical correction of TOF for a classical anatomy done around fifth months or above in
our country with good long term results. If baby is symptomatic early or some variations in anatomy exist, the two
staged surgery can be planned.

What is the palliative surgery for the TOF? What is the Blalock Taussig shunt?

As explained before in certain circumstance, palliative surgery is planned as a first stage surgery. This is done by
putting an alternative channel from systemic artery to lung artery to improve the blood supply of lung. This is also
known as modified Blalock Taussig (BT) Shunt. In later stage when total correction is achieved, this shunt is
closed surgically.

Can the shunt surgery abolish the blueness of body completely?

Shunt surgery is done to optimize the systemic saturation at or above 80%. Hence complete abolition of cyanosis is
not the goal. If we try to achieve a higher saturation by putting a larger shunt we can harm the lungs by causing
flooding of lungs (Pulmonary edema).

For how long a shunt can work?

A shunt has limited duration of functioning. The size of shunt is weight appropriate and as the weight and
accordingly blood volume of baby increases, efficiency of shunt goes down. Therefore baby outgrows the shunt.
Sometimes shunt may get blocked particularly in smaller babies.

What are the precautions to be taken for the babies with modified BT shunt?

These babies are kept on small doses of aspirin, a known antiplatelet drug. IT helps in decreasing the chance of clot
formation inside the shunt. Proper hydration must be maintained for these babies particularly during the episodes of
the diarrhea and fever. Time to time pediatrician / cardiologists consultation must be taken to know about the
proper functioning of shunt. Routine saturation monitoring with pulse oxymeter and hematocrit levels are good
indicator of status of shunt. Sudden deepening of cyanosis, distress and cranky child must be attended in emergency
and shunt failure must be suspected.

What is the corrective surgery foe the TOF?

The corrective surgery of TOF is done as an open heart procedure on bypass machine. The VSD is closed by sewing
in an artificial material -Dacron patch, and by ressecting the muscle of RV out flow. Mostly, a patch is put across the
RVOT and its valve. Pulmonary valve is deformed and is removed in most of the cases. In severe TOF with
inadequate lumen of main pulmonary artery and its branches, a patch repair is done.

What is the conduit repair of TOF?

In cases where RVOT is not repairable, an artificial valve with a tubular structure resembling the RVOT is sewn
between the RV and pulmonary artery. This is known as conduit. Usually in these babies initial palliative surgery is
done and subsequently after 2 years of age conduit repair is planned. Conduit surgery has disadvantage as more
reintervention may be needed.

How much time is taken for the surgery? How much blood is needed?

The usual operating time for the TOF is 3-4 hours. Usually, the donation of 4-6 units of blood is required before the
surgery. Nowadays blood of any group can be donated and hospital can arrange the appropriate blood group.
Specific blood group donors are required in cases of rare blood group or when fresh blood is needed.

What is the role of repeat echocardiography in management of TOF?

The primary diagnosis of TOF is done by echocardiography. Once the primary diagnosis is made, the follow up
echocardiography is important in deciding the timing of intervention.

What is the role of CT angiography in TOF?

For an excellent surgery, prior detailed information regarding the lesion must be given to the surgeon by all possible
means. CT angiography has helped greatly in presurgical evaluation of lung arteries and anomalous arteries like
aorto-pulmonary collaterals in less amount of time. It can be used judiciously whenever required.

What is the role of conventional angiography in TOF?

The CT angio can provide anatomical details but we cannot get hemodynamic data or chance for intervention. The
conventional angiography provides us exactly that. In many patients the abnormal channels can be closed in cath lab
before the surgery or sometimes after the surgery. Some residual lesions can also be handled in the cath lab.

What is the role of ECG and X-ray chest in TOF babies?

The base-line ECGs and X-rays are required in all babies. These simple investigations provide enormous
information to attending physician. They also help in post op patients long term follow up.

What are the chances of getting re-interventions after the corrective surgery of TOF?

The baby may need reintervention in immediate post op period or later. Immediate interventions are done either for
residual holes or RVOT obstruction. There might be some other indications also.

Pacemaker implantation can also be needed in an operated TOF.

Late interventions are done for residual holes, RVOT obstruction or lung artery stenosis. An important cause of
intervention is presence of severe pulmonary leak causing RV dilatation and dysfunction. In grown-up children
nowadays, pulmonary valve implantation is done per-cutaneously or by surgery.

What is the prognosis of un-operated TOF Babies?

The longevity of TOF patients is better amongst the other cyanotic cardiac ailments. But quality of life as a rule is
not good. These kids are prone for morbidity and mortality as mentioned above.

What is the prognosis of operated TOF Babies?

Timely operated TOF babies enjoy a good life and can carry on their profession as well as married life efficiently.
Few of them may need some medications. However, sudden life threatening events due to arrhythmias is prevalent
in this population. There is some restriction on athletic activities.

How often an operated TOF must go for a follow-up check-up?

Usually first few follow-up are planned more frequently like 15 days, 1month, 3 months then 6 months. Later these
visits can be reduced to 1-3 year depending on the follow-up findings.

What are the recommendations for live vaccination in these kids?

According to current norms followed by various tertiary centres world over- no specific protocol to be followed for
closed heart shunt procedures. Live vaccines are to be avoided for 4-6 weeks in case of open heart surgery.
What are the recommendations for endocarditis Prevention in operated TOF babies?

A lifelong endocarditis prophylaxis is recommended for these patients!

Is pregnancy is contraindicated in operated TOF patients?

Once the TOF is repaired successfully, the risk with pregnancy is very low. The risk from a pregnancy goes up if
theres a residual lesion.

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