Atrioventricular Septal Defect Echocardiographic Imaging

Transcription

1 Atrioventricular Septal Defect Echocardiographic Imaging Dr Choo Tze Liang Jonathan MBBS(S pore), FRCPCH (UK), DRCOG (UK), FRCP (Edin), FAMS (Paeds) Senior Consultant Cardiology Service KK Women s and Children s Hospital

2 Welcome to Singapore!

3 Introduction Most common form of CHD diagnosed prenatally (17%) Allan et al 9 th most common form of CHD from postnatal series

4 CHD in Euploid Fetuses Dharnadhono et al: SMJ 2012

5 Prevalence Allan et al 17% of CHD 198 without heterotaxy 174 with heterotaxy

6 Differences in Prevalence? Readily detected on prenatal scanning High association with Chromosomal abnormalities High risk of fetal demise

7 Associations 50% of AVSD associated with T21 50% of fetuses with T21 have CHD (50% of these are AVSD) Other associations include heterotaxy, T18, 8p deletion, EVC syndrome, CHARGE association

8 Atrioventricular Septal Defect Hallmark of a AVSD is the presence of a common AV valve

9 Normal Atrioventricular Junction Paediatric Cardiology: RH Anderson 2010

10 The Hallmark of Atrioventricular Septal Defects Paediatric Cardiology: RH Anderson 2010

11 Common Atrioventricular Junction Paediatric Cardiology: RH Anderson 2010

12 Unwedging of the Aorta Paediatric Cardiology: RH Anderson 2010

13 Dual or Single Orifices Paediatric Cardiology: RH Anderson 2010

14 Level of Shunting Paediatric Cardiology: RH Anderson 2010

15 Types of Atrioventricular Septal Defects No universally unified nomenclature

16 Types of Atrioventricular Septal Defects? Complete common AV canal defect? Partially common AV canal defect? Intermediate defect? Transitional defect? Rastelli classification

17 Key Considerations Having determined the presence of a common atrioventricular junction: Consider then: 1) Single atrioventricular orifice verses two orifices 2) Shunting across septums 3) Arrangement of leaflets

18 Types of Atrioventricular Septal Defects Anderson Atrioventricular septal defect with Common atrioventricular junction Common atrioventricular valve Atrial and ventricular shunting Atrioventricular septal defect with Common atrioventricular junction Dual orifices with bridging leaflets attached to crest of the interventricular septum Atrial shunting Van Praagh Complete Common Atrioventricular Canal defect Partially Common Atrioventricular Canal Defect

19 Types of Atrioventricular Septal Defects Anderson Atrioventricular septal defect with Common atrioventricular junction Common atrioventricular valve Atrial and ventricular shunting (ventricular shunt small because of chordal attachments to ventricular septal crest) Atrioventricular septal defect with Common atrioventricular junction Dual orifices with bridging leaflets Inter atrial and interventricular shunting Van Praagh Transitional type Atrioventricular Canal defect (Jesse Edwards) Intermediate type Atrioventricular Canal Defect (Maurice Lev)

20 Echocardiographic diagnosis atrial situs ventricular size common atrioventricular valve/ junction shunt degree of left or right atrioventricular valve regurgitation exclusion of other cardiac anomalies (heterotaxy)

21 Complete AVSD

22 Complete AVSD

23 Common AV Valve

24 Common AV Valve

25 Common AV Valve

26 Common AV Valve

27 AV Valve Regurgitation Moderate RAVV regurgitation

28 Common AV Valve

29 Left AV Valve Regurgitation

30 Left AV valve

31 Outflow

32 Common AV Valve Rastelli Classification

33 Case #1: Transitional AV Canal

34 Case #2: Transitional AV Canal

35 Case #3: Complete AVSD

36 Case #4: Complete AVSD

37 Case #5: Unbalanced AVSD

38 Antenatal Spectrum Antenatally diagnosed AVSD cases are towards the more severe end of spectrum High association with chromosomal abnormalities with risk of fetal demise Overall prognosis remains lower than that expected from surgical series

39 Outcome Huggon et al 2000 N=301 51% isolated AVSD, 20% LAI, 12% RAI T21 40%, other chromosomal abnormalities 10% Live births 82% 1/12 survival 55% 3 year survival 38%

40 Echocardiographic diagnosis atrial situs ventricular size common atrioventricular valve/ junction shunt degree of left or right atrioventricular valve regurgitation exclusion of other cardiac anomalies (heterotaxy)

41 Key Considerations Having determined the presence of a common atrioventricular junction: Consider then: 1) Single atrioventricular orifice verses two orifices 2) Shunting across septums 3) Arrangement of leaflets

42 Conclusion The hallmark of the Atrioventricular Septal Defect is a common atrioventricular junction Echocardiographic imaging defines the anatomy and function of these hearts and is sufficient for diagnosis and surgical management in the majority of cases

43 Acknowledgements Cardiology Team KKH: A/P Tan TH, Chen CK, Sree Raghavan, Monika Kotecha, Jun, Wai Lin, Xu Ming, Alicia, Xin Yi, Yi Peng, Katrina, Jay, Kim

44 Yankin Children s Hospital Programme

45 DSGH Paediatric Cardiology Team

46 Enjoy your stay in Singapore!