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 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) 4
th
ICS, 1 cm lateral to the mid-clavicular line
Thrill palpable at (L) 2
nd
and 3
rd
intercostal space along the
sternal border
Parasternal heave not palpable
Auscultation
S
1
, S
2
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 (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)
Lung and pleural causes
Bronchiectasis
Lung abscess
Empyema
Bronchogenic carcinoma
Cystic fibrosis
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 PaO
2

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 O
2

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
TOF without
infundibular PS
Preload
PVR N- N-
SVR
HR N-
Contractility N- N-
Cardiac grid in TOF
Post-palliative
shunt
Total repair
Preload
PVR N
SVR N
HR N N
Contractility N N
Cardiac grid : Corrected TOF
Repair 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,
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, POST-
REPAIR
COAGULATION ABNORMALITIES IN TOF
INFECTIVE ENDOCARDITIS PROPHYLAXIS- NEW
CHANGES
PRE MEDICATION
INDUCTION
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