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 DefectClass 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
Ebsteins Anomaly
Marfans Syndrome
Marla Kiess, MD, FRCPC, FACC
Director, Pacific Adult Congenital Heart Clinic
Heart Centre
St. Pauls 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
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)

Presentation at Annual CCS Meeting in Edmonton 2009

L-TGA
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)

Presentation at Annual CCS Meeting in Edmonton 2009

L-TGA
The following situations may warrant surgical intervention / reinterventions: (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)

Presentation at Annual CCS Meeting in Edmonton 2009

L-TGA
Follow-up (Class I)
All patients should have regular (usually annual) cardiology follow
up by an ACHD cardiologist.
Particular attention should be paid to:
1.Ventricular function
2.Systemic (tricuspid) AV valve regurgitation
3.Progressive (or complete) AV block
(Level of Evidence C)

Presentation at Annual CCS Meeting in Edmonton 2009

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-topulmonary 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
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)

Presentation at Annual CCS Meeting in Edmonton 2009

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
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)

Presentation at Annual CCS Meeting in Edmonton 2009

Eisenmenger
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)

Presentation at Annual CCS Meeting in Edmonton 2009

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