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TETRALOGY OF FALLOT (TOF) DEFINITION, COMPONENTS, ETIOLOGY, HEMODYNAMICS, SYMPTOMS, TREATMENT

@lovemedics TETRALOGY OF FALLOT INTRODUCTION Common cyanotic congenital heart disease Presenting above 2 yrs. It is characterized by the classic tetrad: Severe right ventricle outflow obstruction (pulmonary stenosis), Large malaligned VSD, Right ventricular hypertrophy, Overriding of Aorta. Components of tetrAlogy of Fallot Severe right ventricle outflow obstruction, Large malaligned VSD, Aorta that overrides the VSD Right ventricular hypertrophy. ETIOLOGY Extra cardiac manifestation in 10% Syndrome associated with Aperts anomaly Silver anomaly Goldenhar syndrome De Lange sydrome Maternal drug and disease associated Trimethadone Thalidomide HEMODYNAMICS – TOF(PHYSIOLOGY) Pulmonic stenosis RVH without cardiac enlargement & in RV pressure. When the Rt ventricular pressure is as high as the Lt ventricular or aortic pressure, the right to left shunt appears to decompress the RV. It reduces the blood flow into the PA & Increases the right-to-left shunt. As the systolic pressures b/w 2 ventricles are identical there is little or no left-to-right shunt & the VSO is silent Right to left shunt is also silent since it occurs at the insignificant difference in pressure b/w the RV & aorta HEMODYNAMICS (MURMUR) The flow from the RV into PA occurs across the pulmonic stenosis producing an Ejection Systolic Murmur HEMODYNAMICS Severe the pulmonic stenosis Less the flow into the pulmonary artery Shorter the ejection systolic murmur Bigger the right to left shunt. More the cyanosis. HEMODYNAMICS severity of cyanosis is directly proportional to severity of pulmonic stenosis, But Intensity of the systolic murmur is inversely related to severity of pulmonic stenosis. CLINICAL FEATURES Symptomatic -Any time after birth. Anoxic spells - Neonates ,infants (paroxysmal attacks of dyspnea). Cyanosis may be present from birth or make its appearance some years after birth Commonest symptoms: Dyspnea on exertion & exercise intolerance ANOXIC SPELL It occurs predominantly after waking up or following exertion. The child starts crying, becomes dyspneic, and bluer than before Loss of consciousness & Convulsions may occur. The frequency varies from once in a few days to numerous attacks every day SQUATTING The patients assume a sitting posture-squatting as soon as they get dyspneic. PHYSICAL EXAMINATION Cyanosis Clubbing 1st sound: Normal Single 2nd sound & Ejection systolic murmur which ends before the audible single 2nd sound Normal-sized heart with a mild parasternal impulse A systolic thrill in less than 30% pts INVESTIGATION: Chest x-ray ECG Echocardiography (prenatally) Cardiac catheterization (occasionally) CHEST X-RAY boot-shaped heart ECG Right ventricular hypertrophy The diagnosis of TOF is confirmed by echocardiography; Cardiac catheterization is seldom necessary MANAGEMENT OF CYNOTIC SPELL check airway deliver O2 by face mask / nasal cannula knee-chest position Morphine (0.2 mg/kg subcutaneously) or ketamine (3-5 mg/kg/ dose intramuscular) MANAGEMENT OF CYANOTIC SPELL Sodium bicarbonate at 1-2 ml/kg (diluted 1:1 or in 10 ml/kg N/5 in 5% dextrose) Correct hypovolemia (10 ml/kg of DNS) Keep child warm Transfuse packed red cell if anemic (hemoglobin less than12 g/ dl) Beta blockers unless contraindicated by bronchial asthma / ventricular dysfunction; metoprolol is given at 0.1 mg/kg IV slowly over 5 min & repeated every 5 min for maximum of 3 doses; may be followed by infusion at 1-2 µg/kg/min Monitor saturation, heart rates & BP; keep heart rate below 100/minute PALLIATIVE PROCEDURE GOAL : To increase pulmonary blood flow independent of ductal patency & to allow pulmonary artery growth & even total correction SYSTEMIC TO PULMONARY SHUNT Central shunt connecting the ascending aorta & the main pulmonary artery. Waterston shunt connecting the Rt P and the ascending aorta Potts shunt connecting the Lt PA to the descending aorta DEFINITIVE SURGERY OF TOF Involves closure of the VSD & Relief of RVOT obstruction. RVOT - placement of a transannular patch across the pulmonary valve valvectomy resulting in severe pulmonary regurgitation. COMPLICATION pulmonic stenosis - Dyspnea Increasing exercise intolerance, limit Patient activities. Anoxic spell Anemia Cardiac enlargement & CCF Infective endocarditis. #lovemedics #tamilmedico #cardiology #physiology