practical approach to cyanotic congenital heart disease

1. Practical approach to
Cyanotic Congenital Heart
Disease

2. Diagnosing Heart Disease
• Suspecting it
• If you are waiting for the child to
present to you with cyanosis, you are
likely to miss majority of the cases
• History and clinical clues
• Role of Chest X Ray, ECG,
Echocardiography

3. CHD: Traditional Clinical
Diagnostic Approach
pulmonary
blood flow
Cyanotic
 pulmonary
blood flow
PS PAH
Acyanotic
L-R Shunts Obstructive
lesions
Miscellaneous

4. CHD: Diagnostic approach
• Age oriented approach
– Neonates
– Early and mid infancy
– Late infancy and older children
• ‘Physiological’ diagnosis rather than
anatomical diagnosis
– Functional effects of the heart disease: Cyanosis,
Pulmonary blood flow, CCF, Shock
– Assessing the need for early intervention

5. Hemodynamic Classification
• Duct dependent lesions
– Duct dependent pulmonary circulation
– Duct dependent systemic circulation
• Left to right shunts (Pre and Post tricuspid)
• Tetralogy of Fallot physiology
• Admixture physiology
• Miscellaneous
– Valvular diseases
– Obstructive lesions
– Cardiomyopathies

6. Neonates
• Duct dependent lesions
– Duct dependent pulmonary circulation
– Duct dependent systemic circulation
• Transposition of great arteries
• Total anomalous PV drainage (Obstructed)
• Admixture lesions
• Large PDA (Preterms), AP Window, Trucus
arteriosus

7. Duct Dependent Pulmonary Circulation
• Discontinuity between pulmonary ventricle and pulmonary artery
• Typical presentation: 24-72 hours .

8. Pulmonary Atresia with intact IVS

9. Ebstein’s Anomaly
( Functional Pulmonary Atresia)

10. Clinical features
• Development of cyanosis, rapid
worsening
• Single S2
• Unremarkable otherwise (no murmur)
• Sick looking and acidotic
• Misdiagnosed as sepsis commonly

11. Detection of cyanosis
• Cyanosis indicates presence of R-L shunt
• Can be easily missed: Poor lighting, dark skin
and anemia
• Absence of h/o cyanosis does not r/o cyanotic
heart disease
• At times cyanosis is evident only during
activity or crying

12. Detection of cyanosis
• Clinical cyanosis is apparent
only if the SO2 is <85%
• Any value <95% is abnormal
after 48 hours of birth
• Pulse oximetry: invaluable tool
to aid clinical diagnosis (detects
subclinical cyanosis)

13. Work up of the Cyanotic Newborn:
The Hyperoxia Test
100% O2 via hood
~10 min..
PO2
< 150 mmHg
CHD likely
PO2
>200mmHg,
CHD
unlikely
150 to
200

< 70
mmHg
CHD very
likely

14. Duct dependent systemic circulation
• Obstruction to left
heart outflow:
– Aortic atresia,
– Severe coarctation
– Hypoplastic left heart
syndrome
• Circulation maintained
by flow through the
PDA (R-L shunt)
• When PDA constricts,
systemic perfusion is
compromised

15. • Present with shock like state
• Pulse disparity, SO2 disparity (Difference of >5%)
• Single S2, no murmur

16. Signs of Low Cardiac Output
• Poor perfusion, bradycardia, hypotension
• Acidosis
• Cyanosis
• Arrhythmias
• Altered sensorium
• Temperature instability
• Renal and Liver dysfunction

17. Clinical clue
• Femoral pulsations: often the only
clue to the presence of coarctation;
Careful palpation and comparison with
brachials
• Ideally four limb BP measurement
should be made (automated NIBP
preferred )
SHOCK WITH DIFFERENTIAL CYANOSIS: EXCLUDE CHD

18. Mode of presentation
A relatively well child presenting dramatically
between 2 days to 1 week of life strongly
suggests duct dependent lesion

19. Transposition of great arteries

20. Transposition of Great Arteries
• Two parallel
circuits
• Early presentation
with intact IVS
• Large ASD or VSD
will delay the
presentation
• Single S2
• Short ESM

21. Obstructed TAPVC
• Pathway from PVs to LA
obstructed
• Results in Severe PVH
and PAH
• Variable presentation
depending of severity of
obstruction
• S2 variable
• ESM at PA
RA
RV
LV
LA
PA
Ao
Inn
Obstructed
TAPVC

22. • If there is a murmur
• It there is cardiomegaly in the CXR
• If there is pulse discrepancy
– We all know that ….
– We already knew that …..
So… when to suspect heart disease?

23. So… when to suspect heart disease?
• Any child who does not fit clearly to
your initial clinical diagnosis
– Think if this could be heart disease and
look out for some more clues
– Read the CXR again, take an ECG
– When in doubt, do a simple echo: A4CV

24. • Any child with significant desaturation
(assuming that we are doing pulse oxymetry in every child. If
we have not started this practice, we should start it today)
– Think if this could be heart disease and
look out for some more clues
– Read the CXR again, take an ECG
– Don’t hesitate to ask for an echo
So… when to suspect heart disease?

25. Screening
Clinical Examination and Pulse oximetry
Pre-discharge
Repeat 6-8 weeks
Any one
abnormal
Refer for echo and pediatric cardiology
evaluation

26. Role of Chest X Ray

27. Role of Chest x Ray
 Situs
 Cardiac position
 Chamber enlargement
 Arch sidedness
 Lung vasculature
 Lung parenchyma
 Bony cage and diaphragm

28. Situs Solitus
Bronchial
situs
Visceral
situs
Arch

29. Situs solitus, dextrocardia

30. Stomach Liver
Shorter
More Horiz.
Bronchus
Situs Inversus

31. Situs Ambiguous, mesocardia

32. Right arch

33. Low SO2, intubated at admn
Decreased PBF

34. Ground-
glass Haze
Obstructed TAPVC
Low SO2, intubated, no improvement

35. Obstructed TAPVC

36. 8 years old; minimally symptomatic
Supracardiac TAPVC
Left
vertical
vein
Dilated
SVC

37. TAPVC @ 7 days

38. Transposition of Great Arteries

39. No ‘egg’, had TGA
‘egg’ appearance, had
Truncus arteriosus

40. Neonatal Ebstein’s anomaly

41. Role of ECG
• It is normal in many of the serious CHD.
Hence, a normal ECG does not rule out a
heart disease
• An abnormal ECG, almost always points
towards a serious heart disease
• Answer three questions:
– Is the QRS axis rightward
– Is there RV dominance
– Are there q waves in II, III and aVF

42. QRS axis… simplified
• Look at lead I and aVF
• Calculate the mean QRS voltage
I
aVF
+
+
-
-

43. I
aVF
+
+
-
-

44. I
aVF
+
+
-
-

45. I
aVF
+
+
-
-

46. 3 weeks to 3 months
‘Physiological / Functional’ approach
Answer two questions
• Is there cyanosis / systemic
desaturation?
• Is the pulmonary blood flow is
normal/decreased or increased?

47. Large ASD vs large VSD
• Volume overload
• RV vs LV
• Pressure overload

48. Assessment of PBF
History
• Excessive precordial activity noted by parents
• Poor feeding and interrupted feeding
• Excessive forehead sweating
• Orthopnea equivalent
• Respiratory infections that are frequent,
prolonged and difficult to treat
• Failure to thrive

49. Assessment of PBF
Clinical features
• Intercostal and sub-costal retractions
• Cardiomegaly
• Visible precordial activity
• Ejection murmur in the pulmonary area
• Diastolic flow murmur in the apical area
Absence of these findings mean that the PBF is
normal or decreased

50. Hemodynamic Classification
• Duct dependent lesions
– Duct dependent pulmonary circulation
– Duct dependent systemic circulation
• Left to right shunts (Post tricuspid)
• Admixture physiology
• TOF physiology
• Miscellaneous
– Valvular diseases
– Obstructive lesions
– Cardiomyopathies

51. Normal Heart

52. L – R shunts (pre tricuspid)
Atrial Septal Defect
• Increased PBF
• No cyanosis
• No PAH
• Volume overload
without pressure
overload
Partial anomalous
pulmonary venous
connection

53. L – R shunts (post tricuspid)
Ventricular Septal
Defect
• Increased PBF
• No cyanosis
• Volume and
pressure overload
Complete AV canal defect
Patent ductus arteriosus
Aorto pulmonary window

54. Admixture physiology
Single ventricle
• PBF increased
• Mild systemic
desaturation (very
mild/ no cyanosis)
Tricuspid atresia with VSD
Mitral atresia with VSD
Double outlet RV
TAPVC

55. Tricuspid Atresia Single Ventricle

56. TOF physiology
Tetralogy of Fallot
• PBF reduced
• Significant
cyanosis
Single ventricle with PS
DORV with PS
Tricuspid atresia with
restrictive VSD

57. To simplify…
• Acyanotic + active chest = simple L-R shunt
• Cyanotic + active chest = admixture
physiology
• Cyanotic + quite chest = TOF Physiology

58. ‘Physiological / Functional’ approach
Significant cyanosisMild CyanosisNo Cyanosis
No h/o CCF
Quiet precordium
TOF physiology
Heart failure
Hyperactive precordium
Murmur
Admixture
physiology
L – R shunts
(usually
post tricuspid)

59. ‘Physiological / Functional’ approach
No cyanosis
No CCF
No active precordium
+
Prominent murmur
Small L-R shunts
Valvular HD
AS, PS, MR
No cyanosis
H/o CCF
Active precordium
+
No/short murmur
Cardiomyopathies

60. Infants (after 3 months)
• Ventricular Septal Defects (Moderate to large)
• PDA / AP window
• Tetralogy of Fallot physiology
• Admixture physiology
• Outflow tract obstructions, esp PS
• Congenital AV valve regurgitation
• Cardiomyopathies, ALCAPA

61. Older children
• Moderate to small VSD (can be large)
• Small PDA (can be mod to large)
• Fallot and its variants
• PS, AS
• RHD

62. Summary
• Hemodynamic understanding of CHD is very
important
• Clinical, CXR and ECG clues
• Neonatal period: Duct dependent lesions,
cyanosis or shock like status
• Infancy: approach based on systemic
desaturation and pulmonary blood flow
• To have a low threshold for ordering an
echocardiography if clinically indicated

63. Thank you for your attention!