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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

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CASE PRESENTATION

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Case presentation

CASE PRESENTATION

TETRALOGY OF FALLOT

Speaker:Anil Agarwal

Moderator: Prof. Anjan Trikha

www.anaesthesia.co.in

[email protected]


Case presentation

  • 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


Case presentation

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

    ¯


Case presentation

¯

  • 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


Case presentation

  • 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


Case presentation

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


Case presentation

  • 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 years4 years


Physical examination

PHYSICAL EXAMINATION

General physical examination

  • Weight – 12 kg

  • Height – 95 cm

  • Afebrile

  • Pallor – Nil

  • Cyanosis – (+), (lips, tongue, fingers)

  • Icterus – Nil

  • Clubbing – (+) (second degree)


Case presentation

  • 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

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


Case presentation

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


Case presentation

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

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


Case presentation

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


Case presentation

Provisional diagnosis

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


Clubbing

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


Case presentation

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

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


Case presentation

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

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

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

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

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

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

CCF in TOF

  • Rare

  • Exceptions:

    • Anaemia

    • Infective endocarditis

    • Major aorto-pulmonary collaterals [TOF with pulmonary atresia]


Palliative shunts concerns

Palliative shunts: Concerns

  • Reduce, but, not abolish the frequency of tet spells

    Pulmonary blood flow depends on

    Radius of shuntPVR : 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

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 in TOF


Cardiac grid in tof1

Cardiac grid in TOF


Cardiac grid corrected tof

Cardiac grid : Corrected TOF


Tof and pregnancy

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

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

Airway Abnormalities in TOF

  • TOF with pulmonary atresia: tracheomalacia, bronchomalacia

  • Associated syndromes – DiGeorge syndrome, CHARGE, Goldenhar’s syndrome, Down’s syndrome.


Tof coagulation abnormalities

TOF : Coagulation Abnormalities

  • Thrombocytopenia

  • Platelet functional defects

  • Hypofibrinogenemia

  • Elevated PT, APTT.


Tof coagulation abnormalities1

TOF : Coagulation Abnormalities

  • Thrombocytopenia

  • Platelet functional defects

  • Hypofibrinogenemia

  • Elevated PT, APTT.


Hyperviscosity syndrome symptoms

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 abnormalities2

TOF : Coagulation Abnormalities

  • Thrombocytopenia

  • Platelet functional defects

  • Hypofibrinogenemia

  • Elevated PT, APTT.


Tof associated defects

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 symptoms1

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 abnormalities3

TOF : Coagulation Abnormalities

  • Thrombocytopenia

  • Platelet functional defects

  • Hypofibrinogenemia

  • Elevated PT, APTT.


Probable questions

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

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


Case presentation

  • COAGULATION ABNORMALITIES IN TOF

  • INFECTIVE ENDOCARDITIS PROPHYLAXIS- NEW CHANGES

  • PRE MEDICATION

  • INDUCTION


Thank you

Thank You

www.anaesthesia.co.in

[email protected]


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