The document summarizes guidelines from the 2009 Canadian Cardiovascular Society Consensus Conference for the management of adults with congenital heart disease. It discusses recommendations for interventions or follow up for various lesions including atrial and ventricular septal defects, atrioventricular septal defects, patent ductus arteriosus, left ventricular outflow tract obstructions, Ebstein's anomaly and Marfan syndrome. The recommendations are based on the presence of symptoms, hemodynamic parameters and degree of pulmonary hypertension. Surgical repair is indicated for complex lesions and those not amenable to percutaneous closure, and should be performed by congenital heart surgeons.

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

8. PART I ASD VSD AVSD PDA 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

21. PART II Outflow 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

41. PART III D-TGA L-TGA Single Ventricle/Fontan Eisenmenger 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 Class I Pacemaker insertion for symptomatic bradycardia or anti-tachycardia pacing for some atrial arrhythmias may be required. Prior to transvenous lead implantation, the superior baffle must be evaluated for stenosis and/ or baffle leaks with appropriate intervention undertaken. (Level of Evidence: B) Given the association between rapidly conducting atrial arrhythmias and sudden death, an aggressive management strategy that includes catheter ablation is often recommended (Level of Evidence: C) 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) Ablation and device implantation should be undertaken by an electrophysiologist with appropriate training/experience in the ACHD population (Level of Evidence: C) Patients who require intervention or re-intervention should be treated by ACHD cardiologists and congenital heart surgeons with appropriate experience (Level of Evidence: C)

43. D-TGA The following situations may warrant re-intervention following the atrial switch procedures : (Class IIa) Significant systemic (tricuspid) AV valve regurgitation without significant ventricular dysfunction (Level of Evidence: C) Superior or inferior vena cava pathway obstruction (Level of Evidence: C) Pulmonary venous pathway obstruction (Level of Evidence: C) Baffle leak resulting in a significant left to right shunt (Qp:Qs >1.5), symptoms, pulmonary hypertension or progressive ventricular enlargement/dysfunction (Level of Evidence: C) Baffle leak resulting in a significant right to left shunt and symptoms (Level of Evidence: C) Symptomatic bradyarrythmias or tachyarrhythmias (Level of Evidence: C)

44. D-TGA The following situations may warrant re-intervention following the arterial switch procedure : (Class IIa) Significant pulmonary artery stenosis (subvalvular, pulmonary trunk or branch pulmonary artery) (Level of Evidence: C) Coronary arterial obstruction (Level of Evidence: C) Severe neo-aortic valve regurgitation (Level of Evidence: C) Severe neo-aortic root dilatation (Level of Evidence: C)

45. D-TGA The following situations may warrant re-intervention following the Rastelli procedure : (Class IIa) Significant right ventricle to pulmonary artery conduit obstruction (Level of Evidence: C) Severe right ventricle to pulmonary artery conduit regurgitation with symptoms, progressive RV enlargement, the occurrence of atrial or ventricular arrhythmia (Level of Evidence: C) Severe subaortic obstruction across the left ventricle to aorta tunnel (mean gradient >50 mmHg) (Level of Evidence: C) Significant branch pulmonary artery stenosis (Level of Evidence: C) Residual VSD resulting in a Qp:Qs > 1.5, pulmonary hypertension or progressive left ventricular enlargement / dysfunction (Level of Evidence: C) Patients deemed to be at particularly high risk for sudden cardiac death may benefit from ICDs for primary prevention (Level of Evidence: C) (Class IIb)

46. D-TGA Presentation at Annual CCS Meeting in Edmonton 2009 Follow-up (Class I) All patients should be followed regularly (usually annual) by an ACHD cardiologist including appropriate imaging, laboratory, and arrhythmia assessment. (Level of Evidence: C)

47. L-TGA Presentation at Annual CCS Meeting in Edmonton 2009 Class I Pacemakers are indicated in patients with spontaneous or postoperative third-degree and advanced second-degree AV block or documented periods of asystole (≥3.0 seconds) (Level of Evidence: C) Ablation and device implantation should be undertaken by an electrophysiologist with appropriate training/experience in the ACHD population (Level of Evidence: C) Patients who require intervention should be treated by ACHD cardiologists and congenital heart surgeons with appropriate experience (Level of Evidence: C)

48. L-TGA Presentation at Annual CCS Meeting in Edmonton 2009 The following situations may warrant surgical intervention / re-interventions: (Class IIa) Presence of VSD or residual VSD (Level of Evidence: C) Moderate to severe systemic AV valve regurgitation (Level of Evidence: B) Hemodynamically significant pulmonary or subpulmonary obstruction (Level of Evidence: B) Significant stenosis across a left ventricle to pulmonary artery conduit (Level of Evidence: C) Deteriorating systemic (right) ventricular function (Level of Evidence: C)

50. Fontan Re-intervention after Fontan procedure is warranted in the following situations: (Class I) Obstruction to systemic venous return in the Fontan circuit (Level of Evidence: C) Obstruction of pulmonary venous return (Level of Evidence: C) Significant (moderately severe or greater) systemic AV valve regurgitation (Level of Evidence: C) Development of venous collateral channels or pulmonary arterio-venous malformations resulting in symptomatic cyanosis (Level of Evidence: C) Residual atrial septal defect or fenestration resulting in significant right-to-left shunt Residual shunt secondary to a previous palliative surgical shunt or residual ventricle-to-pulmonary artery connection causing a hemodynamically significant volume or pressure load (Level of Evidence: C) Subaortic obstruction with a peak-to-peak gradient of >30mmHg (Level of Evidence: C) Protein losing enteropathy that is associated with high systemic venous pressures or Fontan abnormality (Level of Evidence: C) Recurrent or poorly tolerated atrial arrhythmias refractory to medical therapy (Level of Evidence: C)

51. Fontan Class I (continued) Fontan patients with a history of atrial thrombus, thromboembolic event, interatrial communication or atrial arrhythmias should be therapeutically anticoagulated with warfarin (Level of Evidence: C) When arrhythmias are present, an underlying hemodynamic cause should always be sought, and in particular, obstruction of the Fontan circuit, thrombus formation, or ventricular dysfunction need to excluded by comprehensive imaging (Level of Evidence: C) Patients with arrhythmias should be referred for consultation with an electrophysiologist with expertise in congenital heart disease (Level of Evidence: C) Electrophysiologic studies in Fontan patients should be performed in centres with expertise in congenital heart disease (Level of Evidence: C) Patients who require intervention or re-intervention should be treated by ACHD cardiologists and congenital heart surgeons with appropriate experience (Level of Evidence: C)

52. Fontan Class IIa Fontan patients with intracardiac pacemaker or defibrillator leads should be therapeutically anticoagulated with warfarin (Level of Evidence: C) Anticoagulation may be considered in Fontan patients without atrial thrombus or arrhythmias (Level of Evidence: C) Patients with serious refractory atrial arrhythmias may be considered for Fontan conversion to a total cavopulmonary connection with concomitant atrial maze procedure (Level of Evidence: C) Class IIb When clinical situations or hemodynamics warrant therapy, it may be reasonable to treat ventricular dysfunction in Fontan patients with diuretics, angiotensin converting enzyme inhibitors and beta-blockers as tolerated (Level of Evidence: C)

53. Fontan Presentation at Annual CCS Meeting in Edmonton 2009 Follow-up (Class I) All patients who have had a Fontan operation should be followed yearly by an ACHD cardiologist including appropriate imaging, laboratory, and arrhythmia assessment. (Level of Evidence: C)

54. Eisenmenger Class I Advanced pulmonary vascular obstructive disease with a resistance, which is fixed, in combination with the absence of left-to-right shunting render a patient ineligible for cardiac repair (Level of Evidence: C) The main interventions in patients with Eisenmenger syndrome are directed towards preventing complications (e.g. influenza and pneumococcal vaccination) or restoring physiologic balance (e.g. iron replacement for iron deficiency). (Level of Evidence: C) Phlebotomy with fluid replacement and iron supplementation should be performed only in patients who are symptomatic from secondary erythrocytosis. Prevention of iron deficiency is important. (Level of Evidence: C) Platelet transfusions, fresh frozen plasma, vitamin K, cryoprecipitate and desmopressin can be used to treat severe bleeding (Level of Evidence: C) If iron deficiency anemia is confirmed, iron replacement should be prescribed. (Level of Evidence: C) Symptomatic hyperuricemia and gouty arthritis can be treated as necessary with colchicine, probenecid or sulfinpyrazone; and with Allopurinol for prophylaxis. (Level of Evidence: C)

55. Eisenmenger Class I Sinus rhythm should be restored promptly and maintained whenever possible (Level of Evidence: C) Symptomatic arrhythmias should be treated with individualized antiarrhythmic therapy (Level of Evidence: C) Patients with atrial fibrillation/flutter should receive warfarin therapy with judicious monitoring of INR levels (sodium citrate adjusted to hematocrit) (Level of Evidence: C) Insertion of an implantable defibrillator is a high-risk endeavour. It may be considered in patients with syncope and documented concurrent ventricular arrhythmia. Epicardial approaches should be used (Level of Evidence: C) Transvenous pacing leads are not recommended and must be avoided in the presence of intracardiac shunts due to risk of paradoxical embolization (Level of Evidence: B) Patients with Eisenmenger syndrome should be treated by ACHD cardiologists who understands and has experience in management of the Eisenmenger syndrome (Level of Evidence: C) Patients with Eisenmenger syndrome benefit from the involvement of other specialists (nursing, respirology, psychology/psychiatry, hematology, gynecology, anesthesia, intensive care, social work) (Level of Evidence: C)

56. Eisenmenger Presentation at Annual CCS Meeting in Edmonton 2009 Class IIa Cyanotic patients having surgery may undergo prophylactic phlebotomy to reduce the hematocrit to less than 65% (Level of Evidence: C) Pulmonary vasodilator therapy may help to improve quality of life in patients in Eisenmenger syndrome. (Level of Evidence: B)

57. Eisenmenger Presentation at Annual CCS Meeting in Edmonton 2009 Follow-up (Class I) Annual clinical visits with comprehensive, systematic assessment and laboratory evaluation for potential complications are recommended. (Level of Evidence: C) All patients with cyanotic congenital heart disease should be cared for by an ACHD cardiologist. They may also benefit from the involvement of other specialists within such an ACHD centre (nursing, respirology, psychology/psychiatry, hematology, gynecology, anesthesia, intensive care, social work). (Level of Evidence: C) Imaging tests should be performed every 2 to 3 years in a stable patient. (Level of Evidence: C)

58. 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