Congenital Heart Disease

1. Congenital Heart Disease Cardiac Malpositions Approach to Diagnosis

4. Congenital Heart Disease Malpositions : Diagnosis • Identify situs • Identify chambers and vessels • Arrange chambers and vessels • Identify additional anomalies • Define route of blood flow Diagnosis = 1+ 2 + 3 + 4 + 5

6. Congenital Heart Disease Situs Solitus usual normal Inversus mirror image normal Ambiguous Indeterminate Aplenia, Polysplenia, Palpation, Percussion, X-ray

7. Congenital Heart Disease Malpositions : Asplenia • Absent spleen • Malrotation of bowels • Symmetrical liver • Complex CHD (Bilateral – right sidedness)

9. Congenital Heart Disease Malposition : Polysplenia • Multiple spleens • Malrotation of bowels • Symmetrical liver • Complex CHD (Bilateral left sidedness)

12. Atria RA LA Ventricles RV LV Great arteries PA AO

16. Congenital Heart Disease Sequential Chamber Localisation Define situs • Visceroatrial situs rule • Tracheal bifurcation • ECG • ECHO • Angiogram

17. Congenital Heart Disease Atrial Situs Morphologically right atrium • Receives IVC • Broad appendage • Crista terminalis • Fossa ovalis

18. Congenital Heart Disease Atrial situs Morphologically left atrium • Finger like appendage with narrow neck • Valve of fossa ovalis

19. Congenital Heart Disease Atrial situs • Visceroatrial situs rule • ECG – unreliable • Thoracic roentgenogram – bronchial morphology • Echo – IVC to RA Femoral vein – contrast inj. • Angiogram – shape of appendage

20. Congenital Heart Disease Atrial situs : Viscero atrial situs rule • Stomach, spleen and LA • Liver & RA Same side • Not applicable with Situs ambiguus Asplenia - bilateral RA Polysplenia - bilateral LA

21. Congenital Heart Disease Situs rule : fallacies • Thoracoabd. discordance 7.2% (No splenic abn.) • Solitus or Inversus predicted 3.6% as ambiguus • Midline liver Do no predict 30% Absent spleen S. ambiguus

22. Congenital Heart Disease Atrial situs : Situs rule 87 cases : Levo, meso, dextrocardia • Position of liver (central = ambiguus) 37% • Liver & Howell Jolly bodies 45% • Rt or Lt sided liver (no polysplenia) 59%

23. Congenital Heart Disease Atrial situs : situs rule • 3 lobed lung RA • 2 lobes lung LA Accuracy 72%

24. Congenital Heart Disease Atrial situs : Tracheal bifurcation • RA follows Rt bronchus • LA follows Lt bronchus Asymmetrical division Symmetrical – asplenia, polysplenia

26. Congenital Heart Disease Atrial situs : bronchial morphology • LA follows left bronchus – Hyparterial, horizontal, longer • RA follows right bronchus – Eparterial, vertical, shorter

28. Bronchial length (cm) 6 Left Right 5 4 3 2 1 0 2 4 6 8 10 12 14 16 Age (Yrs.)

29. Congenital Heart Disease Malpositions : Atrial situs Bronchial anatomy : Length Left bronchus :  2 cms Right bronchus :  2 cms (overlap below age 2 yrs.)

30. Congenital Heart Disease Atrial situs : bronchial anatomy Lt / Rt bronchus 2.0 or  Normal or Inversus 1.5 to 2.0 Tomography 1.5 or less Atrial isomerism

31. Congenital Heart Disease Atrial situs : ECG P  in 1, II, aVF Normal  in aVR P  in I, II aVF Inversus  in aVR

32. Congenital Heart Disease Atrial situs ‘P’ wave specificity poor i Sinus node – left side ii Ectopic atrial rhythm iii Bilateral sinus node (asplenia, polysplenia)

33. * S V C LA RA IVC LIVER

34. LA RA

35. RA CS LV RV

36. LAA LA RAA LV RA

38. Liver

39. Liver

41. Liver

44. Congenital Heart Disease Ventricular relationship d loop RV Right side LV Left side l loop RV Left side LV Right side Indeterminate : Sup. Inferior vent.

45. Congenital Heart Disease Ventricular identification – ECG QRS morphology RV : LV rS, Rs 2 : 1 rsR, rSR, rRs 8 : 1 qRS, qR 1 : 3 QRs, QR, Qr 1 : 5

48. Congenital Heart Disease Ventricular identification : Echo • Short axis – mid LV level • 4 chamber – apex – coarse trabeculation & moderator band - RV • Papillary muscle orientation of LV • MV / TV – attachment (4 chamber) • Short axis – fish mouth – MV

49. LV

50. RV LV