CASE PRESENTATION

TETRALOGY OF FALLOT

Speaker : Anil AgarwalModerator : Prof. Anjan Trikha

www.anaesthesia.co.in

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• 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 yearsOrdinary 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 birthDevelopmental history• Developmental milestones attained within normal

limitsFamily 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 SYSTEMInspection• Precordium normal on inspection• No visible apical impulse• No visible pulsation• No scar mark visiblePalpation• 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 fieldsRespiratory 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 limitsAirway assessment• Mouth opening > 4 cm• Neck movement adequate• MMP class I• No facial deformity notedSpine 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 functionImpression• 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

CLUBBINGDegree of clubbing1° 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

osteoarthropathyExamination 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 CYANOSISRespiratory• 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

SQUATTINGMechanisms 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 smallhypoxia, cyanosisToo large – Pulmonary edemaCHF

• 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 TOFTOF+infundibula

r PSTOF without

infundibular PSPreload

PVR N- N-

SVR

HR N-

Contractility N- N-

Cardiac grid in TOFPost-palliative

shuntTotal repair

Preload

PVR N

SVR N

HR N N

Contractility N N

Cardiac grid : Corrected TOFRepair with residual PS

Residual VSD

Preload

PVR N- N

SVR N

HR N N

Contractility N N

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.

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 : Coagulation Abnormalities

• Thrombocytopenia• Platelet functional defects• Hypofibrinogenemia• Elevated PT, APTT.

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.

TOF : Coagulation Abnormalities

• Thrombocytopenia• Platelet functional defects• Hypofibrinogenemia• Elevated PT, APTT.

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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