Cyanotic congenital heart diseases

1. Cyanotic congenital
heart disease

2. Congenital Heart Diseases
 Congenital heart disease-6-8 /1000 live births
 CHD- Cyanotic/Acyanotic
 Further subdivided based on CxR- evidence of
increased (Plethoric)/decreased pulmonary vascular
marking (Oligemic)
 ECG- to determine R/ L / biventricular hyertrophy
 Character of HS/murmur
 Final diag-by echocardiography &/or cardiac
catheterization

3. Assessment of a child for the
presence of heart disease-
Nadas criteria
 Major
1. Systolic murmur
grade III or more
2. Diastolic murmur
3. Cyanosis
4. CCF
 Minor
1. Systolic murmur less
than grade III
2. Abnormal 2nd HS
3. Abnormal ECG
4. Abnormal –x-ray
5. Abnormal BP
1 major or 2 minor criteria

5. Fetal Circulation
• For the fetus the placenta is the oxygenator so the
lungs do little work
• RV & LV contribute equally to the systemic
circulation and pump against similar resistance
• Shunts are necessary for survival
• Ductus venosus (bypasses liver)
• Foramen ovale (R→L atrial level shunt)
• Ductus arteriosus (R→L arterial level shunt)

9. Transitional Circulation
• With first few breaths lungs expand and
serve as the oxygenator (and the placenta is
removed from the circuit)
• Foramen ovale functionally closes
• Ductus arteriosus usually closes within first
1-2 days

11. Neonatal Circulation
• RV pumps to pulmonary circulation and LV
pumps to systemic circulation
• Pulmonary resistance (PVR) is high; so initially
RV pressure ~ LV pressure
• By 6 weeks pulmonary resistance drops and
LV becomes dominant

13. Normal Pediatric Circulation
• LV pressure is 4-5 x RV pressure (this is
feasible since RV pumps against lower
resistance than LV)
• RV is more compliant chamber than LV

14. Classification- Cyanotic CHD
 Decreased PBF
1. Tetralogy of Fallots
2. Pulmonary atresia with
VSD
3. Tricuspid atresia
4. Double outlet RV with
Pulm Stenosis
5. Ebstein anomaly of the
tricuspid valve
 Increased PBF
1. Transposition of the great
arteries(TGA)
2. DORV without Pulm
Stenosis
3. Total anomalous
Pulmonary venous return
4. Truncus arteriosus
5. Hypoplastic left heart
syndrome

15. Tetralogy of Fallots
 Commonest CCHD in
children >2 yr
 Contituents-
i-VSD
ii-pulmonic stenosis
iii –overriding or
dextroposed aorta
iv-Right ventricular
hypertrophy

17. TOF- hemodynamics
 Pulmonary stenosis-concentric RV hypertrophy
without cardiac enlargement & increase in RV
pressure
 Increase in RV pressure- leads R-L shunt
 Once RV &LV pressure equals ,increasing severity of
pulmonic stenosis reduce pulm blood flow
 VSD-silent
 Flow across the pulmonary stenosis – ejection
systolic murmur

18. TOF- hemodynamics
 The more severe PS –The shorter the ejection systolic
murmur and more the cyanosis
 The VSD of TOF is always large-effectively decompress RV
and so CCF never occurs in TOF
 Exceptions-anemia,infective endocarditis, systemic HTN,
Myocarditis and associated AR
 The late and soft P2 –inaudible and 2nd HS is single and due to
aortic component
 RV HT –large a wave

19. TOF- Clinical features
 Anoxic spells-paroxysmal attack of dyspnea any time
after birth
 Cyanosis-since birth or may present some yrs after
 Commonest presentation-dyspnoea on exertion and
exercise intolerance--- squatting position
 Anoxic spells occur predominantly after waking up or
following exertion-starts crying,dyspneic, may loose
consciousness. Convulsion may occur.

20. TOF- Clinical features
 Cyanosis,clubbing, slightly prominent ‘a’ waves
 Others: growth retardation, chest pain, headache,
tachypnoea
 ECG-right axis deviation with RV hypertrophy. ‘T’
waves usually inverted in right precordial leads.
 CxR-N sized heart with upturned apex .
Oligemic lung fields. Absence of main pulmonary
artery segment, boot shaped heart or “cor-en –
sabot”

21. X-ray picture of TOF

22. TOF- complication
 Anoxic infarction, embolism and venous
thrombosis-hemiplegia
 Brain abscess
 Infective endocarditis
 Polycythemia: gout, renal failure, gall stones
 Cyanotic spells

23. Treatment
 Oral beta-blockers to prevent cyanotic spells
 Iron supplementation to prevent anaemia
(Hb>12)
 Palliative Sx: Blalock –Taussig shunt
 Definitive SX: closure of VSD and relief of
RVOT obstruction

24. Management of anoxic spells
1. Knee chest position
2. Humidified oxygen
3. Morphine - 0.1-0.2mg/kg subcutaneous
4. Correct acidosis- soda bicarbonate iv
5. Propranolol-o.1mg/kg/IV(during spell) o.5 to
1mg/kg 4-6hrly orally
6. Vasopressors-Methoxamine IM/IV Drip
7. Correct hypovolemia, anemia

25.  Pink TOF:PS not severe enough to result in
decreased pulmonary flow.
 Pentalogy of fallot: TOF + ASD
 Trilogy of fallot: PS, RVH and ASD

26. Tricuspid atresia
 Congenital absence of the tricuspid valve
 RA LA LV………..RV
 Symptoms- same as TOF
 Features suggestive of TA
1.LV type of apical impulse
2.Prominent a wave in the JVP
3.Enlarged liver with presystolic pulsation
4.ECG-left axis deviation with LV HT
 Palliative T/t—GLENN shunt(SVC-RPA)
-FONTAN OPERATION (all systemic return
to pulmonary artery)

27. Ebstein’s anomaly
 Posterior as well as the septal leaflet of the
tricuspid valve is displaced downwards---
arterialized RV
 Heart sounds: split S1, S2; S3,S4; pansystolic
murmur (TR)
 ECG-`p’ pulmonale as well as `p’ mitrale;
prolonged PR interval; RBBB
 CXR: enlarged cardiac size ( enlarged RA, RV)

28. Cyanotic CHD -Increased PBF
 Abnormal mixing of pulmonary venous blood and
absence of pulmonary blood flow obstruction
 Clinical presentation-
-Cyanosis
- CHF
- Recurrent chest infection
-Failure to thrive
 Prognosis-Poor-80% of Pts expire within 3 months

29. Transposition of great vessels
 5% 0f CHDs
 M/C CCHD presenting in the NB
 M:F –3:1
 RA…………LA RA LA
RV…….. LV LV RV
AO……….PA PA AO
Complete corrected

30. Transposition of great vessels
 C/F- Cyanosis-at birth or 1st week of life
Congestive Heart Failure
 ECG-Rt axis deviation& Rt ventricular hyertrophy
 CxR- Cardiomegaly with a narrow base and
plethoric lung fields.(EGG ON side apearance)
 Treatment-Rx-CHF &acidosis
If hypoxemia –prostaglandin infusion
 Operative- septostomy-- Jatene’ s switch over
-Mustard operation

31. TRANSPOSITION OF
GREAT ARTERIES

32. Total Anomalous Pulmonary
venous connection
All the pulmonary veins instead of joining
LA are connected anomolously to RA -
There is complete mixing of the two
venous returns

33. TAPVC

34. Flow Chart for DD
Cyanosis
 PBF  PBF
RVH LVH BVH RVH LVH BVH
1.TOF
2.Complex
lesions
with PS
1.TA 1.Complex
transpositions
2.Double outlet
ven.
1.TGA
2.TAPVC
3.Hypoplastic
left heart
Synd.
1.TA with
VSD
2.Truncus
arteriosus
1.TGA with
VSD
2.Truncus
arteriosus

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