1. Role of MRI in TOF follow-up TOF symposium October 25, 2013 Dr Edythe Tham

2. Outline Quantification of RV size & function Quantification of pulmonary regurgitation Pulmonary stenosis Branch pulmonary arteries Conduits and artificial valves

3. Goals of cardiac MRI Quantification of RV & LV volumes and function (RVEF) Quantification of pulmonary regurgitant fraction (RF) Anatomy of the RVOT & branch pulmonary arteries (and aorta) Assessment of myocardial fibrosis

4. RV volumes

8. Pulmonary regurgitation Transannular patch

9. RVOT

10. Flow Quantification: Phase contrast imaging

11. Pulmonary Regurgitation Region of interest

12. Regurgitant fraction

13. Criteria for pulmonary valve replacement RVEDV >170 ml/m 2 RVESV > 85 ml/m 2 RVEF < 45% Regurgitant Fraction >30% Therrien et al, AJC 2005

14. Relationship between RV volume and pulmonary regurgitation Samyn et al, JMRI 2007

15. Relationship between RV ESV & RVEF Geva et al, JACC 2004

16. RVEDVi 111 ml/m2 RVESVi 56 ml/m2 RVEF 50% LVEF 60% 17 year female, S/P TAP

18. Regurgitant Fraction 43%

19. RVEDVi 178 ml/m2 RVESVi 150 ml/m2 RVEF 16% LVEF 28% 11 year female with TOF/PA S/P RV-PA conduit

20. Normal septal curvature TOF

21. Regurgitant fraction 57% Peak velocity 2 m/s = Peak gradient 16 mmHg

22. Pulmonary stenosis

24. 10 year female S/P TAP Mixed disease – Mild PS: 20 mmHg Moderal PR: 34%

25. Magnetic Resonance Angiography Branch pulmonary arteries

28. 21 year male S/P TOF repair RPA 56%: LPA 44% Mild proximal LPA stenosis, PG 25 mmHg

29. 18 year old S/P TOF repair – bilateral branch PA stenosis RPA 75%: LPA 25% Peak gradients: RPA: 38 mmHg LPA: 29 mmHg

30. 12 year female with branch PA stenosis From MRI RPA 82%: LPA 18%

31. Right pulmonary artery Left pulmonary artery

32. RVOT aneurysm

34. Conduits & artificial valves

35. Artifact from prosthetic valve 12 year female Prosthetic pulmonary valve Melody valve

42. 38 year male S/P 29 mm Hancock valve RVEDVi 170 ml/m2 RVESVi 98 ml/m2 RVEF 42% RF 20% Peak velocity 3 m/s = PG 36 mmHg

43. Melody valve

44. Circulation, 2006;113:405-413

47. RVEF 33%

48. Indications for cardiac MRI Baseline post-TOF repair at 7-10 years (no sedation required) Follow up every 1-3 years depending on clinical status Yearly MRI if: symptomatic or evidence of RV dysfunction

49. Cardiac MRI: Disadvantages Not portable Contraindications: pacemaker/AICD Affected by metallic artifacts eg prosthetic valves, stents

50. Advantages of MRI No radiation Does not require sedation in older children Independent of acoustic windows Capability for 3D reconstruction Quantifies ventricular function Flow quantification