CASE PRESENTATION

CASE PRESENTATION TETRALOGY OF FALLOT Speaker : Anil Agarwal Moderator : Prof. Anjan Trikha www.anaesthesia.co.in anaesthesia.co.in@gmail.com

A four year old male child from Bihar Presenting complaints • Bluish discolouration of lips since 1 month of age • Breathlessness on exertion since 6 months of age

H/o present illness • Asymptomatic in the first month of life ¯ • Gradual, bluish discolouration of lips and skin ¯ • H/o episodes of increasing bluish discolouration and rapid breathing on exertion (playing, excess crying) since 2-3 months of age ¯ • Episodes lasted for 3-5 minutes ¯

¯ • Resolved with squatting posture or on calming by mother ¯ • No h/o loss of consciousness/seizures during such episodes ¯ • Initially, increased frequency of such episodes ¯ • Decreased frequency over last 1 year

H/o breathlessness on exertion (NYHA gr. II) x last 3½ years Ordinary activity like playing with other children, climbing stairs cause shortness of breath ¯ Relieved on taking rest • No H/o frequent respiratory tract infections • No H/o feeding difficulties • No H/o fever, headache, vomiting, convulsions, lethargy or focal motor weakness • No H/o headache, dizziness, blurred vision, somnolence, fatigue, paresthesia of fingers, toes, lips

Treatment history • No history of previous surgery • Receiving T. propranolol (10 mg) OD x last 6 months (irregular) Birth history • Full term normal delivery • Birth weight 4 kg • No significant maternal illness in antenatal period • No h/o bluish discolouration/jaundice/cyanosis in the neonatal period

Skeletal abnormalities like • 6 fingers in (R) hand • 4 fingers in (L) hand, noticed since birth • No other obvious deformity noticed since birth Developmental history • Developmental milestones attained within normal limits Family history • No history of similar complaints in the family 7 years 4 years

PHYSICAL EXAMINATION General physical examination • Weight – 12 kg • Height – 95 cm • Afebrile • Pallor – Nil • Cyanosis – (+), (lips, tongue, fingers) • Icterus – Nil • Clubbing – (+) (second degree)

Oedema – Nil • Lymphadenopathy – Nil • Neck veins – not engorged • Pulse – 88/min, regular, no radio-radial or radiofemoral delay • All peripheral pulses palpable • BP – 84/60 mmHg (left arm, supine position) • Peripheral venous access - adequate

CARDIOVASCULAR SYSTEM Inspection • Precordium normal on inspection • No visible apical impulse • No visible pulsation • No scar mark visible Palpation • Apex • Palpable at (L) 4th ICS, 1 cm lateral to the mid-clavicular line • Thrill palpable at (L) 2nd and 3rd intercostal space along the sternal border • Parasternal heave not palpable

Auscultation • S1, S2 audible • Systolic murmur, grade IV, best heard at left upper sternal border harsh in quality. Not radiating to back/axilla • No continuous murmur over the lung fields Respiratory system • No chest wall deformity on inspection • Respiratory rate 20/min, regular, accessory muscles not working • Auscultation: • B/L air entry equal • No added sounds

Central nervous system • Higher functions – normal • Cranial nerves, cerebellum, motor and sensory examination – within normal limits Airway assessment • Mouth opening > 4 cm • Neck movement adequate • MMP class I • No facial deformity noted Spine examination • No abnormality detected

Investigations • Hb – 15.4 • TLC – 8,900 • Na+/K+ - 139/4.4 • Urea – 42 • Bd. group B (+ve) CXR: • Heart size – (N) • RV type apex • Pulmonary vascularity ¯ ECG • RAD • 100/min, regular

Echo • Severe infundibular + valvular PS • Confluent good sized pulmonary arteries • Large VSD with aortic override (perimembranous, R  L) • No additional VSD • No ASD, coarctation of aorta, PDA • (N) RV/LV function Impression • CCHD, ¯ pulmonary blood flow, TOF, confluent good sized pulmonary arteries

Provisional diagnosis • Cyanotic congenital heart disease with no evidence of infective endocarditis or congestive cardiac failure

CLUBBING Degree of clubbing 1° Increased fluctuation of the nail bed with loss of onychodermal angle (Lovibond’s sign) 2° Increased anteroposterior and transverse diameter of the nails, loss of longitudinal ridges (+1°) 3° Increased pulp tissue (+2°) 4° Swelling in wrist and ankle due to hypertrophic osteoarthropathy Examination for clubbing • Fluctuation of nail bed • Lovibond’s sign • Schamroth’s sign

Causes • Cardiac • Congenital cyanotic heart disease • SBE • Cardiac tumours (rare) • Lung and pleural causes • Bronchiectasis • Lung abscess • Empyema • Bronchogenic carcinoma • Cystic fibrosis • Ulcerative colitis • Biliary cirrhosis • Intestinal causes: Crohn’s disease and Coeliac disease • Idiopathic (in normal persons) • Genetic

CYANOSIS Definition • Bluish discolouration of the skin and mucous membranes due to presence of increased amount of reduced haemoglobin in the blood (> 5 gm/dl) • Types: • Central • Peripheral

Central cyanosis • Due to marked decrease in arterial PaO2 • Sites: • Tongue • Inner aspect of lips • Mucous membrane of gum, soft palate, cheeks • Lower palpebral conjunctiva • Sites for peripheral cyanosis [tip of nose, ear lobule, tips of fingers and toes, outer aspects of lips]

CAUSES OF CENTRAL CYANOSIS Respiratory • Cardiac • Cyanotic congenital heart disease • Left ventricular failure • Eisenmenger’s syndrome • Respiratory • Acute severe asthma • COPD • Lobar pneumonia • Tension pneumothorax • Acute laryngeal oedema • Pulmonary thromboembolism • High altitude

SQUATTING Mechanisms of improvement in oxygenation • Angulation and compression of the femoral arteries with squatting in SVR, [pulm. stenosis remains constant] ¯ in PBF ¯ in arterial saturation • ¯ venous return of desaturated blood from lower extremities

PALLIATIVE SHUNTS IN TOF • Blalock-Taussig shunt [anastomosis of subclavian artery and pulmonary artery] • Modified B-T shunt [Goretex graft used] • Pott’s shunt [descending aorta  left pulmonary artery] • Waterston’s shunt [ascending aorta  right pulmonary artery]

BREATH HOLDING SPELLS • Reflexive events • Provoking event e.g. anger, frustration Crying  Crying stops at full expiration  Apneic, pale/cyanotic  Loss of consciousness (some cases) • 4-5% of paediatric population • Treatment: • Parental reassurance • Iron therapy

Management of hypercyanotic spells • Knee chest position • Supplemental O2 • Morphine 0.1-0.2 mg/kg • Correct acidosis, sodium bicarbonate • Beta blockers • Vasopressors: phenylephrine • Correction of anaemia, dehydration • Indication for surgical correction

CCF in TOF • Rare • Exceptions: • Anaemia • Infective endocarditis • Major aorto-pulmonary collaterals [TOF with pulmonary atresia]

Palliative shunts: Concerns • Reduce, but, not abolish the frequency of tet spells Pulmonary blood flow depends on Radius of shunt PVR : SVR Too smallhypoxia, cyanosis Too large – Pulmonary edema CHF • BP monitoring not accurate in upper extremity ipsilateral to shunt • Antibiotic prophylaxis required

Post-total correction of TOF: Concerns • Residual RVOT obstruction • Residual VSD • Pulmonary insufficiency • Ventricular arrhythmias • Risk of sudden death

Cardiac grid in TOF

Cardiac grid : Corrected TOF

TOF and pregnancy • ACOG risk stafification • Uncorrected TOF: Moderate risk (5-15% mortality) • Corrected TOF : Mild risk (0-1% mortality) • Maternal risk • Decreased functional capacity • Thromboembolism • Worsening of cyanosis • Increased mortality • Hyperuricemia • Fetal risk • Hypoxia • Intrauterine death • Prematurity • Congenital heart disease (0-4%)

Nadas’ criteria • Assessment for presence of heart disease in children • Major criteria • Systolic murmur > grade III • Diastolic murmur • Cyanosis • CCF • Minor criteria • Systolic murmur, < grade III • Abnormal S2 • Abnormal ECG • Abnormal CXR • Abnormal BP • Presence of 1 major or 2 minor criteria  significant

Airway Abnormalities in TOF • TOF with pulmonary atresia: tracheomalacia, bronchomalacia • Associated syndromes – DiGeorge syndrome, CHARGE, Goldenhar’s syndrome, Down’s syndrome.

TOF : Coagulation Abnormalities • Thrombocytopenia • Platelet functional defects • Hypofibrinogenemia • Elevated PT, APTT.

Hyperviscosity Syndrome : Symptoms • CNS – headache, dizziness, blurred vision, depressed mentation • General – fatigue, myalgia, weakness, paresthesia of fingers, toes, etc. • Management – indicated when Hct > 65% • Correction of dehydration • Erythropheresis or exchange transfusion.

TOF : ASSOCIATED DEFECTS • Cardiac – PDA, multiple VSDs, right sided aortic arch, anomalous origin of LAD artery. • Extra cardiac – CHARGE association, Di George syndrome, Goldenhar syndrome, Down’s syndrome.

Hyperviscosity Syndrome : Symptoms • CNS – headache, dizziness, blurred vision, depressed mentation • General – fatigue, myalgia, weakness, paresthesia of fingers, toes, etc. • Management – indicated when Hct > 65% • Correction of dehydration • Erythropheresis or exchange transfusion.

Probable questions • Cyanosis- causes, sites, d\d of cyanotic CHD • Clubbing- causes, grades, examination • Squatting • Hypercyanotic spells- management • Breath holding spells • NYHA CLASSIFICATION • Nadas’ criteria of heart disease in children

Questions • Palliative shunts- timing, types • Anesthetic concerns post- palliaton • Total correction • Sequelae of total correction • Hyperviscosity syndrome • Hemodynamic goals of TOF, POST-SHUNT, POST- REPAIR

COAGULATION ABNORMALITIES IN TOF • INFECTIVE ENDOCARDITIS PROPHYLAXIS- NEW CHANGES • PRE MEDICATION • INDUCTION

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CASE PRESENTATION