The approximated cost of TOF surgery in India is $8000 and it may vary according to the hospital chosen and place of choice. Success rate of TOF is reported 94%in patients within 1 year of age thus early decision of surgery might save your child from many after effects. Also the estimated stay for TOF surgery in India is approx. 30 days including pre-operative and post- operative phase apart form that this period also include the period of catheterization. To prevent the after effects of the TOF surgery care must be taken including the notice of any abnormal heat sound, regurgitation or arrhythmia.
It is a rare condition caused by combination of four heart defects that are present at birth. The symptoms may vary from none to severe at the time of birth but later cyanosis i.e. bluish coloration of skin is reported.
It is the so called conotruncal abnormality i.e. malformation of the infundibulum ( conus arteriosus) and great arteries (truncus arteriosus)that have abnormal ventriculo-arterial alignments and connections.
TOF consists of a large subaortic VSD and obstruction to the pulmonary outflow, usually at the infundibular level also. This produces right ventricular hypertrophy. In addition the aorta overrides the VSD. It can be associated with ASD, right sided aortic arch, and anomalous coronary artery.
In TOF the right ventricular pressure is same as the left ventricular pressure becauseof the large VSD. The obstruction to the pulmonary blood flow causes desaturated blood to be diverted into the aorta, which is often large; thus the degree of right ventricular obstruction determines the extent of cyanosis. Hence, in childhood the so called cyanotic TOF or pink TOF occurs when there is less obstruction to pulmonary blood flow and patients do not have cyanosis. The infundibular hypertrophy, is however, tends to be progressive; cyanosis occurs and increase with advancing age.
TOF occurs 1 in 2,000 new-borns. Male is to female ratio is equal. It is the most complex congenital heart defect.
The four defects which form the TOF are-
1 Pulmonary stenosis-
It is referred as the narrowing of the exit from the right ventricle.
2 ventricular septal defect-
It is the hole between the two ventricles.
3 right ventricular hypertrophy-
It is defined as the thickening of the right ventricular muscle.
4 overriding aorta-
Which causes the blood from both the ventricles to enter aorta.
Sign and symptoms –
- Low blood oxygen saturation.
- Heart murmur, may vary from imperceptible to very loud.
- Difficulty in feeding.
- Failure to gain weight.
- Retarded growth and poor physical development.
- Laboured breathing (dyspnoea) onexertion.
-Tiredness and limping, movement.
- Clubbing of fingers and toes.
- Polycythaemia a disease in which the haematocrit is elevated.
1 check for cyanosis – findings on physical examination include various degree of cyanosis and clubbing.
2 A right ventricular lift
3 A thrill at the left sternal edge- indicating severe pulmonary obstruction.
4 A long systolic murmur – in pulmonary area
5 Absent P2
6 Aortic regurgitation – in adult patients who have not been operated because the aorta is large and the cusps prolapse into the defect. The degree of aortic regurgitation may vary. If the aortic regurgitation jet enter the right ventricle, the right ventricular failure may develops.
1 Echocardiography- this technique is specific, can be done prenatally and is a quick method of diagnosis. TOF is confirmed by demonstrating VSD, RVH, and aortic override.
Colour Doppler measures the degree of pulmonary stenosis.
2 Chest x-ray- Shows boot- shaped heart due to right ventricular hypertrophy. Lung field s are often dark due to decreased pulmonary blood flow. Other observations include concave pulmonary bay and pulmonary oligemia.
3 Electrocardiography – ECG shows right ventricular hypertrophy along with right axis deviation. Tall peaked P waves are also noticed.
The diagnosis is confirmed by echocardiography.
Medicationgivenincludes beta – blockers such as propanolol, also acute episodes may require morphine or intranasal fentanyl, to reduce ventilatory drive.
Phenylephrine may also be given to increase systemic vascular resistance.
Total surgical repair- Total surgical repair of TOF is a curative surgery.
Completerepair of TOF may involve patch closure of the VSD and resection of a considerable amount of right ventricular myocardium, we may say that reconstruction of the right ventricular outflow tract.
Timing of surgery in asymptomatic patients is usually from the age of 2 month to one year.
Pulmonary vavlevctomy – it is also a surgical measure to treat TOF if the pulmonary annulus is small.
Palliative surgery- initially the surgery involved forming a side to end anastomosis between subclavian artery and pulmonary artery, i.e. a systemic to pulmonary atrial shunt. This method improve e pulmonary blood flow and help pulmonaryarteries to grow.
Types of shuntare;
- Blalock – Taussig (a subclavian artery –to- pulmonary artery anastomosis, which can be used on either the right side or the left side)
-Waterstone shunt- between the ascending aorta and the right pulmonary artery.
- Potts shunt (descending aorta to left pulmonary artery shunt)
-Central shunt is made up of a polytef graft.
Complications of shunts include –
- Distortion of the pulmonary arteries such as clinking,thrombosis or occlusion.
- Pulmonary vascular disease
- Patients with large shunts are at risk of left ventricular volume overload and ultimately ventricular failure.
Long term problems after surgery-
- Pulmonary regurgitation leading to right ventricular enlargement and dysfunction and ultimately to exercise limitation and fatigue.
- QRS prolongation and ventricular arrhythmia.
- Residual pulmonary stenosis.
- Right ventricular aneurysm at the site of the surgical patch.
- Residual VSD and aortic dilation and aortic regurgitation.
Late presentation of the case might face reoperation, whereas early presentation have high success rate.
1 What exactly is TOF?
- It is rare condition of heart which is a combination of four defects that are present at birth. TOF causes oxygen-poor blood to flow out of the heart and into the rest of the body.
2. What are the risk factors for TOF?
The risk factor for TOF include any viral infection such as rubella during pregnancy, maternal alcoholism or a family history of the condition.
3. What is the success rate of the TOF surgery?
Patients who have undergone TOF surgery have improved hemodynamic and often have good to excellent cardiac function after the operation with some to no exercise tolerance. The survival rate is 94% if the operation is performed at the age of 1 year and 91% at 5 years of age.
However residual post-surgical defects such as pulmonary regurgitation, pulmonary artery stenosis, residual VSD, right ventricular outflow tract obstruction and death in worst case scenario may occur.
4. What is the price of TOF surgery in India?
The estimated cost of TOF surgery in India is approx. $8000 and it may vary according to the hospital and place of treatment.
5. What is the estimated stay in India for TOF surgery?
TOF surgery performed within 1 year of age give high success rate and less period of catheterization. The estimated time for the complete stay for TOF surgery is approx. 30 days including the period of catheterization, preoperative and post-operative time period.
6. What are the after effects of TOF surgery?
The major after effect which is reported in a TOF surgery is pulmonary regurgitation, which predisposes to volume overload of the right ventricle that in turn predispose to ventricular arrhythmias.
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