Presentation at Annual CCS Meeting in Edmonton 2009

1. CANADIAN CARDIOVASCULAR SOCIETY 2009 CONSENSUS CONFERENCE UPDATE ON THE GUIDELINES FOR THE MANAGEMENT OF ADULTS WITH CONGENITAL HEART DISEASE Presentation at Annual CCS Meeting in Edmonton 2009

2. Section Editors Ariane Marelli (Section Editor) - Montreal, Canada Luc Beauchesne (Section Editor) – Ottawa, Canada Annie Dore (Section Editor) - Montreal, Canada Marla Kiess (Section Editor) – Vancouver, Canada Omid Salehian (Section Editor) – Hamilton, Canada Presentation at Annual CCS Meeting in Edmonton 2009

3. Timothy Bradley – Toronto, Canada Jack Colman – Toronto, Canada Michael Connelly – Calgary, Canada Louise Harris – Toronto, Canada Paul Khairy – Montreal, Canada Seema Mital – Toronto, Canada Koichiro Niwa - Ichihara, Japan Erwin Oechslin– Toronto, Canada Nancy Poirier – Montreal, Canada Markus Schwerzmann – Bern, Switzerland Dylan Taylor – Edmonton, Canada Isabelle Vonder Muhll – Edmonton, Canada Writing Panel Members Presentation at Annual CCS Meeting in Edmonton 2009

4. International Panel Members Helmut Baumgartner – Muenster, Germany Lee Benson – Toronto, Canada David Celermajer – Sydney, Australia Matthias Greutmann – Zurich, Switzerland Eric Horlick – Toronto, Canada Mike Landzberg – Boston, USA Folkert Meijboom – Utrecht, The Netherlands Barbara Mulder – Amsterdam, The Netherlands Carole Warnes – Rochester, USA Gary Webb – Philadelphia, USA Presentation at Annual CCS Meeting in Edmonton 2009

5. Speciality Panel Members Epidemiology Paul Khairy – Montreal, Canada Interventional Lee Benson – Toronto, Canada Eric Horlick – Toronto, Canada Dylan Taylor – Edmonton, Canada Arrhythmia Paul Khairy – Montreal, Canada Louise Harris – Toronto, Canada Genetics Seema Mital– Toronto, Canada Chantal Morel – Toronto, Canada Pregnancy Jack Colman– Toronto, Canada Samuel Siu – London, Canada Mathew Sermer– Toronto, Canada CV Surgery Christo Tchervenkov – Montreal, Canada Ivan Rebeyka– Edmonton, Canada

7. Outline • Epidemiology and Scope of the Problem, Antibiotic prophylaxis,Genetic Evaluation • PART I - Shunts • PART II - Outflow Tract Obstructions, Ebstein Anomaly and Marfan Syndrome • PART III - D-TGA, L-TGA, Single Ventricle/Fontan, Eisenmenger Syndrome Presentation at Annual CCS Meeting in Edmonton 2009

8. PART IASDVSDAVSDPDA Dylan A. Taylor MD FRCPC FACC Director, Northern Alberta Adult Congenital Heart Clinic University of Alberta Mazankowski Alberta Heart Institute Edmonton, Alberta, Canada Presentation at Annual CCS Meeting in Edmonton 2009

9. Atrial Septal Defect – Class I • Surgical or percutaneous closure of an atrial septal defect (ASD) is indicated in the presence of a hemodynamically significant ASD with or without resulting symptoms. (Level of Evidence: B) • In patients with large secundum ASDs (>38 mm) not amenable to device closure, surgical closure should be undertaken (Level: B) • Patients with a sinus venosus defect or ostium primum ASD cannot be closed by percutaneous devices and should be surgically repaired by congenital heart surgeons (Level: C) Presentation at Annual CCS Meeting in Edmonton 2009

10. Atrial Septal Defect – Class I • If atrial fibrillation/flutter occurs, anticoagulation is indicated in accordance with existing guidelines (Level: A) • Atrial arrhythmias can be managed with either rate or rhythm control strategies and the approach should be tailored to the individual patient (Level: B) Presentation at Annual CCS Meeting in Edmonton 2009

11. Atrial Septal Defect – Class III • If PAH is present and there is irreversible PAH, the ASD should not be closed. Such patients should receive care from a specialist with expertise in PAH. (Level: C) • pulmonary artery pressure (PAP) > 2/3 systemic arterial blood pressure (SABP) • pulmonary arteriolar resistance > 2/3 systemic arteriolar resistance Presentation at Annual CCS Meeting in Edmonton 2009

12. Ventricular Septal Defect – Class I • The following situations warrant closure: • a) The presence of a “significant” VSD [symptomatic; LV volume overload; deteriorating ventricular function due to volume (LV) or pressure (RV) overload, Qp/Qs >2:1; pulmonary artery systolic pressure > 50 mmHg] (Level: B) • b) Significant right ventricular outflow tract obstruction (cath gradient or mean echo gradient greater than 50 mmHg) (Level: B) • A perimembranous or sub arterial VSD with more than mild aortic incompetence (Level: B) Presentation at Annual CCS Meeting in Edmonton 2009

13. Ventricular Septal Defect – Class I • In the presence of severe pulmonary hypertension there must be a net left-to-right shunt of ≥ 1.5:1; or evidence of pulmonary artery reactivity when challenged with a pulmonary vasodilator (e.g. oxygen, nitric oxide and/or prostaglandins)(Level: B) • PAP > 2/3 SABP or pulmonary arteriolar resistance greater than 2/3 systemic arteriolar resistance • Patients with an isolated VSD with or without associated lesions (right ventricular outflow tract obstruction, aortic valve prolapse, subaortic stenosis or infective endocarditis) should be repaired by congenital heart surgeons. (Level: C) Presentation at Annual CCS Meeting in Edmonton 2009

14. Ventricular Septal Defect – Class III • If irreversible PAH is present the VSD should not be closed. Such patients should receive care from a specialist with expertise in PAH. (Level: C) • PAP > 2/3 SABP • pulmonary arteriolar resistance > 2/3 systemic arteriolar resistance Presentation at Annual CCS Meeting in Edmonton 2009

15. Atrioventricular Septal Defect – Class I • The following situations warrant intervention or re-intervention: • An unoperated AVSD with: • a) Presumed paradoxic embolism (Level: B) • b) Left ventricular dysfunction (Level: B) • c) Right ventricular volume overload (Level: B) • d) Clinical heart failure (Level: B) • e) Reversible pulmonary hypertension (Level: B) Presentation at Annual CCS Meeting in Edmonton 2009

16. Atrioventricular Septal Defect–Class I • An unoperated AVSD with: • a) Presumed paradoxic embolism (Level: B) • b) Left ventricular dysfunction (Level: B) • c) Right ventricular volume overload (Level: B) • d) Clinical heart failure (Level: B) • e) Reversible pulmonary hypertension (Level: B) • f) Significant subaortic obstruction (cath gradient or mean echo gradient >50mmHg at rest or on provocative testing with isoproterenol) (Level: B) • g) Transvenous pacing should be avoided if there are residual inter-atrial or inter-ventricular communications since paradoxical emboli may occur (Level: B) • Patients with an AVSD should be repaired by congenital heart surgeons (Level: C) Presentation at Annual CCS Meeting in Edmonton 2009

17. Atrioventricular Septal Defect – Class III • If irreversible PAH is present the AVSD should not be closed. Such patients should receive care from a specialist with expertise in PAH. (Level: C) • PAP > 2/3 SABP • pulmonary arteriolar resistance > 2/3 systemic arteriolar resistance Presentation at Annual CCS Meeting in Edmonton 2009

18. Patent Ductus Arteriosus – Class I • No intervention is indicated if a small silent PDA is detected (Level: C) • Surgical closure should be reserved for those in whom the PDA is too large for device closure (Level: B) • Operative repair should be undertaken by congenital heart surgeons (Level: C) Presentation at Annual CCS Meeting in Edmonton 2009

19. Patent Ductus Arteriosus – Class III • If irreversible PAH is present the AVSD should not be closed. Such patients should receive care from a specialist with expertise in PAH. (Level: C) • PAP > 2/3 SABP • pulmonary arteriolar resistance > 2/3 systemic arteriolar resistance Presentation at Annual CCS Meeting in Edmonton 2009

20. Follow up • Uncomplicated or simple lesions require infrequent clinical & imaging follow up by a cardiologist familiar with these lesions • closed ASD, restrictive or closed VSD, silent or closed PDA • Complicated or complex lesions require more frequent clinical & imaging follow up in a centre with special expertise with these lesions • repaired or unrepaired VSD with associated lesions • repaired or unrepaired AVSD • repaired or unrepaired PDA • PAH associated with any lesion Presentation at Annual CCS Meeting in Edmonton 2009

21. PART IIOutflow Tract Obstructions Ebstein‘s Anomaly Marfan‘s Syndrome Marla Kiess, MD, FRCPC, FACC Director, Pacific Adult Congenital Heart Clinic Heart Centre St. Paul‘s Hospital Vancouver, BC, Canada Presentation at Annual CCS Meeting in Edmonton 2009

22. LEFT VENTRICULAR OUTFLOW TRACT OBSTRUCTION (LVOTO) AND BICUSPID AORTIC VALVE DISEASE Presentation at Annual CCS Meeting in Edmonton 2009

23. SUPRAVALVULAR LVOTO Class I • Operative intervention is recommended for patients with supravalvular LVOTO with symptoms and/or a mean echo or a mean catheter gradient of > 50 mmHg or a peak instantaneous echo gradient > 70 mmHg if the obstruction is discrete (Level of Evidence: C) • Patients who require operation for supravalvar LVOTO should be operated on by congenital heart surgeons (Level of Evidence: C) Presentation at Annual CCS Meeting in Edmonton 2009

24. VALVULAR LVOTO Class I • Valvular LVOTO requires intervention for symptoms (dyspnea, angina, presyncope, or syncope) and significant left sided outflow obstruction (mean echo gradient of >40 mmHg or aortic valve area <1.0 cm2 or <0.6 cm2/m2). Gradients may be lower if there is significant LV systolic dysfunction. (Level of Evidence: C) • Patients with bicuspid aortic valves require intervention for symptoms and severe regurgitation OR severe aortic regurgitation with left ventricular end systolic dimensions of >55 mm, end diastolic diameter >75 mm or left ventricular ejection fraction < 50% (Level of Evidence: B) Presentation at Annual CCS Meeting in Edmonton 2009

25. VALVULAR LVOTO Class I • Aortic root replacement is required for ascending aortic dissection and should be considered prophylactically for proximal aortic dilation (> 50 mm) or progressive dilation of >5 mm/year (Level of Evidence: B) • Pulmonary autograft (Ross procedure) and balloon valvuloplasty for valvar LVOTO should be performed in centres and by physicians with substantial experience in these procedures(Level of Evidence: C) Presentation at Annual CCS Meeting in Edmonton 2009

26. Re-interventions for Valvular LVOTO Class I – Re-operation is indicated after valvotomy or after surgery for: • Recurrent LVOTO (same criteria as above) (Level of Evidence: C) • Severe aortic regurgitation (Level of Evidence: C) • Combined restenosis with moderate or greater regurgitation especially if symptoms or progressive LV dilation is present (Level of Evidence: C) Presentation at Annual CCS Meeting in Edmonton 2009

27. SUBVALVULAR LVOTO Class I • Intervention is indicated for patients with subvalvular LVOTO with symptoms and a peak instantaneous echo gradient of >50 mmHg or a mean echo gradient of >30 mmHg, or if combined with progressive aortic regurgitation (Level of Evidence: C) • Patients who require operation for subvalvar LVOTO should be operated on by congenital heart surgeons (Level of Evidence: C) Presentation at Annual CCS Meeting in Edmonton 2009

28. COARCTATION OF THE AORTA Class I • All patients with significant coarctation (native or re-coarctation post-repair) should be considered candidates for treatment (Level of Evidence: C) • For significant native aortic coarctation, a surgical or a percutaneous approach (if the anatomy is suitable) is reasonable. The preferred approach should reflect center expertise and patient preference (Level of Evidence: B) • For significant re-coarctation post repair, a percutaneous approach (if the anatomy is suitable) is the preferred initial intervention (Level of Evidence: B) • Surgeries and percutaneous interventions should be performed in centres and by individuals with expertise in the procedure (Level of Evidence: C) Presentation at Annual CCS Meeting in Edmonton 2009

29. RIGHT VENTRICULAR OUTFLOW TRACT OBSTRUCTION (RVOTO) Class I • In symptomatic patients with valvular RVOTO, a domed pulmonary valve and peak instantaneous Doppler gradients > 50 mmHg or mean echo gradients > 30 mmHg, balloon valvotomy is recommended (Level of Evidence: C) • In asymptomatic patients with valvular RVOTO, a domed pulmonary valve and peak instantaneous Doppler gradients > 60 mmHg or mean gradients > 40 mmHg, balloon valvotomy should be considered (Level of Evidence: C) Presentation at Annual CCS Meeting in Edmonton 2009

30. RIGHT VENTRICULAR OUTFLOW TRACT OBSTRUCTION (RVOTO) Class I • The surgical approach is recommended for those patients with significant RVOTO and dysplastic pulmonary valves, subvalvular or supravalvular pulmonary stenosis, associated pulmonary hypoplasia or severe pulmonary regurgitation (Level of Evidence: C) • Balloon valvuloplasty is the treatment of choice for valvar RVOTO. Occasionally valve replacement may be necessary (Level of Evidence: B) • Balloon valvuloplasty for valvar RVOTO should still be performed only in centres and by teams with experience in this technique (Level of Evidence: C) • Patients who require operation for RVOTO should be operated on by congenital heart surgeons (Level of Evidence: C) Presentation at Annual CCS Meeting in Edmonton 2009

31. RIGHT VENTRICULAR OUTFLOW TRACT OBSTRUCTION (RVOTO) Class I - Re-interventions for RVOTO • Recurrent RVOTO after prior surgery or balloon valvotomy (same criteria as above) (Level of Evidence: C) • Severe pulmonic regurgitation associated with reduced exercise capacity of cardiovascular cause or deteriorating right ventricular function or substantial tricuspid regurgitation or sustained atrial flutter/fibrillation or sustained ventricular tachycardia (Level of Evidence: C) Presentation at Annual CCS Meeting in Edmonton 2009

32. TETRALOGY OF FALLOT Class I • In patients with sustained ventricular tachyarrhythmia and/or resuscitated from sudden cardiac death with no clear identified reversible cause, ICDs are indicated for secondary prevention.(Level of Evidence: B) • Patients who require operation for TOF should be operated on by congenital heart surgeons (Level of Evidence: C) Presentation at Annual CCS Meeting in Edmonton 2009

33. TETRALOGY OF FALLOT Class IIa Following palliative surgery, complete intracardiac repair should be considered in all patients, in the absence of severe irreversible pulmonary hypertension or unfavourable anatomy (inadequate pulmonary arteries). In palliated patients the following situations particularly warrant complete repair: • Worsening symptoms (Level of Evidence: C) • Cyanosis with erythrocytosis (Level of Evidence: C) • Reduction or absence of the continuous shunt murmur (suspected shunt stenosis or occlusion) (Level of Evidence: C) • Aneurysm formation in the shunt • Left ventricular dilation due to aortic regurgitation or a residual shunt (Level of Evidence: C) Presentation at Annual CCS Meeting in Edmonton 2009

34. TETRALOGY OF FALLOT Class IIa - Re-interventions for Tetralogy of Fallot The following situations may warrant intervention following repair: • Free pulmonary regurgitation associated with progressive or moderate to severe RV enlargement (right ventricular end diastolic volume > 170 cc/m2), moderate to severe right ventricular dysfunction, important tricuspid regurgitation, atrial or ventricular arrhythmias, or symptoms such as deteriorating exercise performance (Level of Evidence: C) • Residual VSD with a shunt > 1.5:1 (Level of Evidence: C) • Residual pulmonary stenosis with RV pressure  2/3 of systemic pressure (either the native right ventricular outflow or valved conduit if one is present) (Level of Evidence: C) • Significant aortic regurgitation associated with symptoms and/or progressive left ventricular systolic dysfunction (Level of Evidence: C) Presentation at Annual CCS Meeting in Edmonton 2009

35. TETRALOGY OF FALLOT Class IIa - Re-interventions for Tetralogy of Fallot The following situations may warrant intervention following repair: • Aortic root enlargement  55 mm in diameter (Level of Evidence: C) • A large right ventricular outflow tract aneurysm or evidence of infection or false aneurysm (Level of Evidence: C) • Sustained clinical arrhythmias, most commonly either atrial flutter or fibrillation, or sustained monomorphic ventricular tachycardia. When any of these arrhythmias occur, the patient should also be evaluated for a treatable hemodynamic cause of the arrhythmia (Level of Evidence: C) • The combination of residual VSD, and/or residual pulmonary stenosis and regurgitation, all mild-moderate but leading to substantial RV enlargement, reduced RV function or symptoms (Level of Evidence: C) Presentation at Annual CCS Meeting in Edmonton 2009

36. TETRALOGY OF FALLOT Class IIa • Patients deemed to be at particularly high risk for sudden cardiac death may benefit from ICDs for primary prevention(Level of Evidence: B) • Patients who require re-operation for tetralogy of Fallot should be operated on by congenital heart surgeons (Level of Evidence: B) Presentation at Annual CCS Meeting in Edmonton 2009

37. EBSTEIN ANOMALY Class I The following situations warrant intervention: • Limited exercise capacity (New York Heart Association class greater than II) (Level of Evidence: B) • Increasing heart size (cardiothoracic ratio greater than 65%) (Level of Evidence: B) • Important cyanosis (resting oxygen saturations < 90%) (Level of Evidence: B) • Severe tricuspid regurgitation with symptoms (Level of Evidence: B) • Transient ischemic attack or stroke (Level of Evidence: B) Patients who require operation for Ebstein anomaly should be operated on by congenital heart surgeons who have substantial specific experience and success with this operation. Every effort should be made to preserve the native TV. (Level of Evidence: C) Presentation at Annual CCS Meeting in Edmonton 2009

38. MARFAN SYNDROME Class I The following situations warrant surgical intervention: • A maximal aortic root/ascending aorta diameter > 50 mm (Level of Evidence: B) • A maximal aortic root/ascending aorta diameter > 45-50 mm with: 1. Rapid aortic root growth > 5 mm per year, 2. Progressive aortic regurgitation, especially if the surgeon believes the aortic valve can be spared and an aortic valve-sparing procedure is planned, 3. Family history of premature aortic dissection< 50 mm, 4. Severe mitral valve regurgitation that requires surgery (Level of Evidence: B) • A maximal aortic root/ascending aorta diameter > 44 mm if pregnancy is desired (Level of Evidence: B) Presentation at Annual CCS Meeting in Edmonton 2009

39. MARFAN SYNDROME Class I The following situations warrant surgical intervention: • A maximal dimension of other parts of the aorta of 50-60 mm or progressive dilation (Level of Evidence: B) • Severe mitral regurgitation with symptoms or progressive left venticular dilation/dysfunction as per the current guidelines on valvular heart disease (Level of Evidence: B) Patients who require operation for Marfan syndrome should be operated on by congenital heart surgeons (Level of Evidence: C) Presentation at Annual CCS Meeting in Edmonton 2009

40. MARFAN SYNDROME Class IIa • All patients with Marfan syndrome should be advised to take beta-blockers and to remain on them unless side effects preclude their use. This is especially true, usually in association with other blood pressure lowering agents, if dissection has occurred. (Level of Evidence: B) Presentation at Annual CCS Meeting in Edmonton 2009

41. PART IIID-TGAL-TGASingle Ventricle/FontanEisenmenger Syndrome Omid Salehian MSc, MD, FRCPC, FACC, FAHA Director of Echocardiography Laboratory McMaster University, Hamiton Presentation at Annual CCS Meeting in Edmonton 2009

42. D-TGA

43. D-TGA

44. D-TGA

45. D-TGA

46. D-TGA Presentation at Annual CCS Meeting in Edmonton 2009

47. L-TGA Presentation at Annual CCS Meeting in Edmonton 2009

48. L-TGA Presentation at Annual CCS Meeting in Edmonton 2009

49. L-TGA Presentation at Annual CCS Meeting in Edmonton 2009

50. Fontan