1.08.2012

Tetralogy of Fallot

Posted on 1:12 AM by know about heart


Tetralogy of Fallot described by Fallot in 1988.It has four feature:-
   1. Pulmonary stenosis
   2. VSD (ventricular disease)
   3. Overriding of aorta
   4. Right ventricular hypertrophy
There is a failure of the bulbus cordis to rotate properly so that the aorta lies more anterior to the right (dextroposed) than normal. The aorta moves nearer to the tricuspid valve and overrides the septum with a “malignment” VSD beneath the aortic valve. Infundibular stenosis develops ,with hypertrophy of the septal  and parietal bands of infundibular muscle that form part of crista.Obs6truction of  right ventricular outflow is usually due to combination of infundibular  and valve stenosis, but may be either alone.
This is the most common cyanotic congenital heart disease presenting after 1 year
Diagnosis
History/Symptom:
§ Increased cyanozed after crying or feeding
§  Dyspnoea on exertion
§ Paroxysmal hypercyanotic attacks(Fallot’s spellor bluve spell)-Patient goes on squatting position (knee elbow position) during crying, playing & after feeding to relieve Fallot’s spell is called Fallot’s sign’s.
§ Growth and development failure
§Patient may become apnoeic of unconscious
§ Delayed puberty
On examination:
a.     On general exam: Short of stature
§ cyanosis
§ Congested red eyes
HEART
§ Polycythaemia
§ Clubbing
b.     Systemic examination:
Precordial Examination:
§ Right ventricular heave/ left parasternal heave-present
§ Systolic thrill felt along the left sternal edge in 3rd on 4th intercojfal space.
§ First heart sound is normal
§ Second heart sound is either soft or single
§ Loud ejection systolic murmur in the pulmonary area (Due to pulmonary stenosis)
(iii) Investigation:
(a)   Blood (Routinely):
§ Hb%: Increased
§ PCV-Increased (Polycythaemia)
§ Full Blood count (FBC)
§ ESR-Reduced
§ Peripheral Blood film: Immature RBC
(b)Diagnostic
X-ray chest:
§ Boot shaped heart with normal transverse diameter
§ Small pulmonary artery
ECG:
§ Evidence of RVH
§ Evidence of right axis deviation
Echocardiography-diagnostic (To find 4 components of Fallot’s teratology) § Cardiac cheterization
B. Treatment:
i) Medical treatment:
(a) Treatment of cyanotic attack
§ Patient should be propped up position on patient goes on squatting position (knee elbow position)
§ 02 should be given
§ Propranolol is given
§ Decreased agitation by giving pathediane injection 1-2 mg/kg 1M
§ Correction of acidosis by NaHCO3 (8.5%) IV
b.Maintenance of hydratias: Intake of large amount of fluid
c. Iron & vit-B complex supplementation.
d. Prophylaetic antibiotic for infective endocarditic.

(ii) Surgical treatment: Correction should be done before 5 years.
(a) Palliative shunt procedure: Anastomosis between pulmonary & systemic circulation.
§ Black Taussing’s shunt
§ Warson’s shunt
§ Pot’s shunt
(b)Total corrective surgery:
Total correction of the defect by surgical relief of pulmonary stenosis & closure of the VSD.

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