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Adult Congenital Guidelines from Canada

Adult Congenital Guidelines from Canada

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Canadian 2009 guidelines Canadian 2009 guidelines Presentation Transcript

  • 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
    • 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
    • Writing Panel Members
    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 Presentation at Annual CCS Meeting in Edmonton 2009
    • 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
    • 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
  •  
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • LEFT VENTRICULAR OUTFLOW TRACT OBSTRUCTION (LVOTO) AND BICUSPID AORTIC VALVE DISEASE Presentation at Annual CCS Meeting in Edmonton 2009
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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)
  • 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)
  • 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)
  • 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)
  • 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)
  • 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)
  • 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)
  • L-TGA Presentation at Annual CCS Meeting in Edmonton 2009 Follow-up (Class I)
    • All patients should have regular (usually annual) cardiology follow up by an ACHD cardiologist.
    • Particular attention should be paid to:
    • Ventricular function
    • Systemic (tricuspid) AV valve regurgitation
    • Progressive (or complete) AV block
    • (Level of Evidence C)
  • 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)
  • 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)
  • 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)
  • 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)
  • 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)
  • 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)
  • 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)
  • 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)
  • 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