Approach to cyanotic congenital heart disease

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% O 2 via hood ~10 min.. PO 2 < 150 mmHg CHD likely PO 2 >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 SVC 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 cyanosis Mild Cyanosis No 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!