CASE PRESENTATION

TETRALOGY OF FALLOT
Speaker : Anil Agarwal

Moderator

Prof. Anjan Trikha

www.anaesthesia.co.in [email protected]

 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 3 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 examination within normal limits Airway assessment Mouth opening > 4 cm and sensory

Neck movement adequate

MMP class I No facial deformity noted Spine examination No abnormality detected

Investigations
Hb 15.4 CXR: Heart size (N) RV type apex Pulmonary vascularity ECG RAD 100/min, regular

TLC 8,900
Na+/K+ - 139/4.4 Urea 42 Bd. group B (+ve)

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 (Lovibonds 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 Lovibonds sign Schamroths sign

Causes Cardiac
Congenital cyanotic heart disease SBE Cardiac tumours (rare) Bronchiectasis Lung abscess Empyema Bronchogenic carcinoma Cystic fibrosis

Lung and pleural causes

Ulcerative colitis Biliary cirrhosis Intestinal causes: Crohns 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 Eisenmengers 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]

Potts shunt [descending aorta left pulmonary artery]

Waterstons 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, 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

TOF+infundibular PS Preload PVR N- TOF without infundibular PS N-

SVR
HR

N-

Contractility

N-

N-

Cardiac grid in TOF

Post-palliative shunt Preload PVR Total repair N

SVR
HR

N
N

Contractility

Cardiac grid : Corrected TOF

Repair with residual PS Preload PVR N- Residual VSD N

SVR
HR

N
N

Contractility

TOF and pregnancy

ACOG risk stafification
Uncorrected TOF: Moderate risk (5-15% mortality) Corrected TOF : Mild risk (0-1% mortality) Decreased functional capacity Thromboembolism Worsening of cyanosis Increased mortality Hyperuricemia

Maternal risk

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 Systolic murmur, < grade III Abnormal S2 Abnormal ECG Abnormal CXR Abnormal BP

Minor criteria

Presence of 1 major or 2 minor criteria significant

Airway Abnormalities in TOF

TOF with pulmonary bronchomalacia atresia: tracheomalacia,

Associated syndromes DiGeorge syndrome, CHARGE, Goldenhars syndrome, Downs 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, Downs 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, POSTREPAIR

 COAGULATION ABNORMALITIES IN TOF

INFECTIVE CHANGES
INDUCTION

ENDOCARDITIS

PROPHYLAXIS-

NEW

PRE MEDICATION

Thank You
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