The document provides guidelines and recommendations for the treatment of various congenital heart diseases, including atrial and ventricular septal defects, AV septal defects, patent ductus arteriosus, and left ventricular outflow tract obstructions. It establishes classification systems for different severity levels and provides evidence-based recommendations for intervention, follow-up care, and management of complications. Genetic testing and counseling are also recommended for certain conditions that have identified genetic causes or associations.

1. THE ROYAL COLLEGE
EXAMINATION:CARDIOLOGY
STUDY CARDS 2011:
ADULT CONGENITAL HEART DISEASE (ACHD)

2. GRADING SYSTEM FOR
GUIDELINES
Class of Recommandation
I Benefits >>> Risks Should perform
IIA Benefits >> Risks It is reasonable to perform
IIB Benefits > Risks May be considered
III Risks > Benefits Either unhelpful or may be harmful
Levels of Evidence
A Multiples RCT’s &/or Meta-analyses
B 1 RCT or 1 large non-randomized trial
C Expert opinion, small studies, retrospectives, & registries

3. GENERAL
RECOMMEDNATIONS
In the introduction, the guidelines describe the organization of the
Canadian Health Care system and the provision of ACHD care in
this context.
Please refer to the Endocarditis Section (8) of the Study Cards for
IE Prophylaxis recommendations in CHD
Essentially,
• Unrepaired cyanotic disease (including shunts)
• 1st 6 months following repair
• Residual lesions suggesting unendothelialized
prosthetic material

4. GENERAL
RECOMMEDNATIONS
Genetic diagnosis may be useful for:
• Prognosis
• Complementary Examinations (other organ involvement?)
• Reproductive counselling
• Family member screening
Evaluation of ACHD should therefore include:*
• Detailed family history for birth defects of all kinds (not just cardiac)
• Physical examination for dysmorphic facies, eye and ear abnormalities, limb defects, other
skeletal defects, other organ system involvement, neurodevelopmental delay or learning
disabilities.
• Family screening through ECG and/or Echo. The need for investigation should be guided
by genetic susceptibility when a genetic cause is known.
• Cytogenetic testing should be considered in the following situations:
• Recognizable chromosomal syndrome (eg, trisomy 21)
• Associated dysmorphic features
• Growth retardation, developmental delay or mental retardation
• Multiple congenital anomalies.
• History of multiple miscarriages and/or family history of birth defects.
Genetic consultation is recommended in the presence of
• Associated extracardiac anomalies
• Clinical suspicion of a genetic abnormality
• Positive family history of birth defects.

5. SHUNT LESIONS
• Atrial Septal Defects (ASD’s)
• Ventricular Septal Defects (VSD’s)
• Endocardial Cushion Defects (atrioventricular septal defects,
AVSD’s)
• Padent Ductus Arteriosus (PDA)
Genetics
Holt-Oram syndrome: ASD + pre-axial limb defects (TBX5 gene)
Familial ASD + progressive AVB: Nkx2.5 gene
w/o AVB: GATA4 mutations
ASD in context of FAS

6. ASD’S Complications
Residual shunting, PAH, new-onset or
Class I recommendations* recurrent arrhythmias, device embolization,
impingement of valves, veins, aorta,
tamponade, device thrombosis, RV failure
• Closure of ASD if hemodynamically significant
• Defined as an enlarged RV by MRI or Echo
• Surgical or percutaneous
• percutaneous preferred if technically feasible (< 38mm, Secundum type, and no associated
cardiac defects requiring surgical repair)
• Post Percutaneous Closure
• Systematically r/o effusion at 24hrs by TTE
• Chest Pain or Syncope -> Immediately R/O Erosion/Migration
• 3 months & 1 Year & then “periodically”
• 6 months of ASA
• 6 months prophylactic antibiotics
• Post Surgical Closure
• Risk of tamponade/post pericardiotomy syndrome for several weeks
• Peri-operative death <1%
• Follow-up
• Anticoagulation for AF/AFL according to guidelines
• reduced occurrence if closure before 40yrs
• Rate or Rhythm Control according to guidelines
• ACHD Specialist follow-up if:
• Repaired as an adult, elevated PAP, arythmia, or ventricular dysfunction at the time of
repair, coexisting heart disease, Familial ASD with risk of AVB

7. ASD’S
Class IIa & IIb recommendations*
• Closure may be indicated in the absence of RV
Enlargement in the following contexts:
• Orthodeoxia-Platypnea
• Paradoxical Emboli
• Tricuspid Valve repair or replacement
• PAH if Qp/Qs >1.5 or PAH is reversible
• Arythmia Interventions should be considered before
percutaneous intervention or at the same time as surgery
• Consult EP prior to closure in the setting of sustained atrial
arrhythmia

8. ASD’S
Class III recommendations* (i.e. don’t do it)
• NO closure if irreversible PH (refer to specialized center)
• PAP >2/3 SBP (ie PAH present)
• PVR >2/3 SVR *Transmission to infant of sporadic 2nd ASD: 5-10%
• NO pregnancy if Eisenmenger
• Otherwise well tolerated if not hemodynamically significant
or repaired
• Increased risk of paradoxical emboli during pregnancy and
post-partum
• NO IE prophylaxis if Isolated ASD or repaired ASD >6 mos
without residual shunt.
Recommended that closure be performed before 24yrs→mortality benefit
- and probably before 40yrs→arrhythmia benefit

9. Mild: Low Press sPAP/Ao Syst <0.3 or
VSD’S mPAP < 20mmHg or
sPAP< 35mmHg AND Qp/Qs<1.5
Class I recommendations* Mod: High Press Syst Pressure ratio >=0.3
mPAP>= 20mmHg or
• Closure of VSD if: sPAP>= 35mmHg AND Qp/Qs>1.2
• Significant VSD PVR/SVR<0.2
• Symptoms Large: High Press + PVR/SVR 0.2-0.7
• LV Volume overload
• LV or RV failure/deterioration
Eisenmengers: High P and Vasc Resist ratio >0.7 + Qp/Qs<1.2
• Qp/Qs > 2
• Significant RVOT Obstruction
• Cath or mean Echo Gradient > 50mmHg
• SubArterial (Type1) or PeriMb (type2) with >mild Ao Insuff
• Severe PulmHTN
• = PAPs/SBP >2/3 or PVR/SVR >2/3
• Qp/Qs 1.5 (i.e. still net significant L-to-R shunt) or evidence of reversibility on Cath
• Surgical closure by ACHD surgeon
• Device closure is IIb
• Excellent survival if normal LV&RV function
• PAP evolution variable. Therefore follow-up…
• Ventricular arrhytmias & SCD may occur, especially if late repair
• ACHD specialist follow-up if
• Residual defect (re-intervention rare)
• Elevated PVR at the time of repair
• Concomitant Ao valve surgery
• Residual RV or LV dysfunction
• Significant atrial or ventricular arythmias
• Associated cardiac lesions

10. VSD’S
Class IIa & IIb recommendations*
• Closure may be indicated if:
• History of endocarditis (esp if recurrent)
• Transvenous pacing required
• Prevention of paradoxical emboli
• Device Closure (as opposed to surgical) may be preformed if:
• Muscular or PeriMb VSD far from valves without associated
lesions.
• Risk of cAVB (1-6% early or late) higher with PeriMb device
closure than surgery (1-4%)
• Success rates are 90-95%
• Complications 10%
• Hypotension, device embolization, heart block, new AI or
TR

11. VSD’S
Class III recommendations* (i.e. don’t do it)
• NO closure if irreversible PH (refer to specialized center)
• PAP >2/3 SBP or PVR >2/3 SVR & Irreversible on Cath
• NO pregnancy if Eisenmenger
• Otherwise well tolerated if small or moderate or repaired.
• Assuming no LV dysfunction or significant cyanosis or other
lesions.
• NO IE prophylaxis if Isolated VSD without cyanosis or repaired
VSD >6 mos without residual shunt.

12. 5 leaflets: R-AS, R-inf, Superior & Inferior bridging leaflet, L-lateral wall leaflet
AVSD’S 30-60% of AVSD have DOWN’s Syndrome
70% of Down’s Syndrome have AVSD (also seen in Williams Syndrome
Smith-Lemli-Opitz, Smith-Magenis)
Class I recommendations*
• Intervention if:
• Unoperated and
• Paradoxical Emboli
• LV Dysfucntion
• RV Volume Overload
• Clinical Heart Failure
• Reversible Pulm HTN
• Operated and
• Persistent or new HD significant defects
• Left AV valve regurgitation or stenosis (from previous repair) + symptoms
• Deteriorating ventricular function
• Significant Subaortic Obstruction
• Cath or mean Echo Gradient > 50mmHg at rest or with stress (isuprel)
• Avoid TVP if residual ASD or VSD.
• Surgical closure by ACHD surgeon
• Device closure is not possible (in Primum ASD+Partial AVSD)
• If AV valve replacement, operative risk same as MVR, but higher risk of cAVB (both early and late).
• Re-Intervention 5-10%
• Pregnancy
• Well tolerated if repaired or NYHA Class I-II
• Increased risk of paradoxical emboli if unrepaired. (prophylactic closure should be considered)
• ACHD transmission 3-5% generally
• Trisomy 21 transmission is 50% (reproductive counselling recommended)
• ACHD specialist follow-up for all patients
• High risk of progressive AVB, subaortic stenosis (5%), “mitral” regurgitation and stenosis

13. AVSD’S
Class IIa & IIb recommendations*
• There are none!
Partial: Ventricular septum intact, 2 separate AV valve annuli, Primum ASD, Cleft Left AV valve
(90% of these occur in non-Downs)
Intermediate: Restrictive VSD, Primum ASD, Cleft MV. Fused anterior & post bridging leaflets
→ 2 distinct AV valves (spectrum b/w partial & complete)
Complete: Non-restrictive inlet VSD. Primum ASD (rarely atrial septum intact). Common AV orifice
(Poor prognostic features: AF/AFL, syncope, HF, hemoptysis,... eisenmengers)

14. AVSD’S
Class III recommendations* (i.e. don’t do it)
• NO closure if irreversible PH (refer to specialized center)
• PAP >2/3 SBP or PVR >2/3 SVR & Irreversible on Cath
• NO pregnancy if Eisenmenger
• Otherwise well tolerated
• Consider closure prior to pregnancy (see class I)
• NO IE prophylaxis if Isolated AVSD without cyanosis or repaired
VSD >6 mos without residual shunt or prosthetic heart valve.

15. PDA
Class I recommendations*
• No intervention for Silent PDA’s
• Device Closure is preferred (>85% success at 1 year)
• Should be planned at the same time as diagnostic cath
• The presence of Ductal Ca2+ increases surgical risk
• Surgical Closure is reserved for cases where device closure not
feasible(>95% success)
• Too large or distorted (aneurysm, post-endarteritis)
• Risk of recurrent laryngeal or phrenic nerve or thoracic duct damage.
Genetic associations:
Char Syndrome: AD; TFAP2B
- abN facies, a/hypoplasia 5th fingers
middle phalanges
↑ - must check Oximetry in hands + feet

16. PDA
Class IIa & IIb recommendations*
• Closure may is indicated for any audible PDA without
irreversible PulmHTN (ie: not Silent PDA or severe PDA)
• Clinically Silent PDA post endarteritis
• If significant Pulm HTN (sPAP>2/3 sBP or PVR >2/3 SVR) + Qp/Qs >1.5
or evidence of PA reactivity on Pulm vasodilat challenge (O2, NO,
Prostgldin)
• ACHD specialist follow-up for all
• Recanalization rare, but possible
• Natural history of device closure not completely known
• Endocarditis prophylaxis according to guidelines

17. PDA
Class III recommendations* (i.e. don’t do it)
• NO closure if irreversible PH (refer to specialized center)
• PAP >2/3 SBP or PVR >2/3 SVR & Irreversible on Cath
• NO pregnancy if Eisenmenger
• Otherwise well tolerated if small or moderate or repaired.
• Assuming no LV dysfunction or significant cyanosis or other
lesions.
• NO IE prophylaxis if Isolated PDA or repaired >6 mos without
residual shunt.

18. OBSTRUCTIVE LESIONS
& Ebstein & Marfan’s
• Left Ventricular Outflow Tract Obstruction (LVOTO)*
• Coarctation
• Right Ventricular Outflow Tract Obstruction(RVOTO)
• Ebstein Anomaly
• Marfan’s Syndrome
*Supravalvar: often diffuse, begins at superior margin of sVals, distal to coronaries
(Williams Syndrome: 7q11.23 (on FISH) microdeletion (elastin gene): neurodev delay, ‘social’ personality, ELFIN facies,
cardiac malfrmtn, HYPERCALCEMIA, skeletal, renal abN - ass. periph pulm or systemic arterial stenoses-coronary/renal)
Valvar: usually BAV + associated Coarct, PDA, Asc Aortopathy
(BAV: 1-2% of pop, AD trait with variable penetrence, 4:1 Male: Noonan, Turner, Williams Syndromes)
(Aortic atresia: Jacobsen (11q deletion), Turner, Trisomy 13, 18, Wolf-Hirschhorn (4p deletion) Syndrome)
Subvalvar: 2:1 Male Often with AI - AV damaged by subvalvr jet
Can be- discrete fibromusc ridge (partially or compl encircling LVOT
Long fibromusc narrowing, or tunnel-like narrowing of entire
LVOTO, rare: obstruction by MV insertion or accessory MV leaflet
SHONE Syndrome: LV Inflow and Outflow Obstruction
(eg: supravalvar mitral ring, parachute MV + subvalvar LVOTO, BAV, Ao coarct)

19. LVOTO
Class I recommendations*
Intervention is indicated if
• Supravalvular (discrete) and probably the same for diffuse lesions, as well.
• Sx or Mean gradient > 50 (cath or echo) orPeak gradient > 70 (echo)
• Low operative mortality and recurrence low
• Risk of aneurysms and endocarditis at the site of patches
• Valvular and
• Sx (dyspnea, angina, (pre-)syncope) and significant LVOTO
• Mean Gradient >40 (N LVEF), AVA <1.0 or <0.6/m2
• Sx and severe regurgitation
• Severe regurgitation and LVESD>55, LVEDD>75, or LVEF<50%
• AoRoot replacement if dissection, prox Ao >50, progression >5mm/yr (continued
surveillance either way)
• Subalvular and
• Sx and mean echo gradient > 50 or peak echo gradient > 70
• Gradient may be underestimated if associated VSD
• Sx and progressive Ao regurgitation
• Not indicated for prevention of regurgitation in adults!

20. LVOTO
Class I recommendations (cont’d)*
Supravalvular and Subalvular LVOTO require an ACHD surgeon
Balloon valvuloplasty and Ross procedure may be considered for
valvular in the young adult with out Ca2+
• Experienced centers only
• Valvotomy/plasty will usually require re-intervention eventually
• Pulmonary autographs may degenerate over time and there is a
risk of neo-aortic aneurysm formation
• Pulmonary homographs also require follow-up
Reintervention is indicated if
• Recurrent LVOTO (same criteria), severe AR, or restenosis with >
mild AR (esp if Sx or progressive LVEF deterioration)
• Re-intervention after subvalvular LVOTO repair is particularly
common

21. LVOTO
Class IIa & IIb recommendations*
• RVAo may be considered if Bicuspid valve and Critical Stenosis
• <0.6cm2 non-indexed
• Mean Gradient >60mmHg
• RVAo may be considered if Biscuspid valve and
• Competitive athlete
• Pregnancy desired
• Longterm follow-up following the Ross procedure
• RVAo may be considered if Biscuspid valve and
• Competitive athlete
• Pregnancy desired
• All patients should have regular follow-up
• ACHD specialist follow-up for the following:
• Williams & Shone Syndromes
• Complex lesions (with or without repair)
• Look for: recurrence, aneurysms, endocarditis, heart block, VT/VF, & SCD

22. LVOTO
Class III recommendations* (i.e. don’t do it)
• NO IE prophylaxis unless prosthetic valve.

23. COARCTATION
Class I recommendations*
• Intervention should be considered for any significant coarctation, especially in
the young with hypertension.
• 65% will have regression of hypertension
• Less straightforward are the elderly, asbence of HTN, mild coarctation, significant
comorbidity
• Either surgery (mortality <1%) or percutaneous according to experience and
patient preference
• Percutaneous preferred for recoarctation (lower mortality and lower risk of
aneurysm)
• Unsuitable anatomy for percutaneous: Long, tortuous, arch hypoplasia
• Women with significant coarctation or dilatation should undergo repair prior to
pregnancy
• All aptients require follow-up with an ACHD specialist with periodic MRI/CT
• r/o aneursym formation, late dissection
• Screen for residual/recurrent HTN (recoarct?), LV dysfn, CAD
• F/U bicuspid Ao valve and screen for ascending aortopathy
• Investigate headaches agressively (Berry aneurysms)
• Sx (dyspnea, angina, (pre-)syncope) and significant LVOTO

24. COARCTATION
Class IIa & IIb recommendations*
• There are none…

25. COARCTATION
Class III recommendations* (i.e. don’t do it)
• NO IE prophylaxis except in the first 6 months following repair.

26. RVOTO
Class I recommendations*
Percutanous Intervention (Balloon>PVR) is indicated if
• Valvular RVOTO with nice anatomy and
• Sx (dyspnea, angina, (pre-)syncope) and significant RVOTO
• Peak Gradient > 50 (mean > 30)
• Asx and significant RVOTO
• Peak Gradient > 60 (mean > 40)
Surgical Intervention is indicated if
• Valvular RVOTO with dysplasia or subvalvular or supravalvular, or f
pulmonary hypoplasia or PR
Re-intervention is indicated if
• Recurrent RVOTO (same criteria)
• Severe pulmonic regurgitation assoc’d with reduced functional
capacity or deteriorating RV function or substantial TR or sustained
atrial or ventricular arrhythmias

27. RVOTO
Class I recommendations (cont’d)*
No ACHD follow-up if trivial RVOTO (<25 Peak and Asx)
ACHD follow-up if
• > mild RVOTO
• > moderate PR
• Screen for progressive/recurrent stenosis, RV dysfn/dilatation, TR,
arrhythmias, or evidence of shunting (esp. R-to-L)

28. RVOTO
Class IIa & IIb recommendations*
• Intervention for valvular RVOTO probably also indicated for:
• Important arrhythmias (A-flutter)
• Associated ASD or VSD (esp. if R-toL shunting)
• Recurrent endocarditis
• Double-chambered RV with pullback gradient >50mmHg

29. RVOTO
Class III recommendations* (i.e. don’t do it)
• NO IE prophylaxis unless prosthetic valve.

30. DiGeorge (22q11 deletion): ToF, interrupted Ao Arch, VSD, persistent truncus
Palatal abN, hypertelorism, cleft palate
TOF
Learning disability, hearing loss,
HypoCa from hypoparathyroidism
Class I recommendations*
All patients should be followed by an ACHD specialist and all advanced
invesitgations should be performed by staff with expertise in ACHD
Surgical Intervention is indicated if
• Valvular RVOTO with dysplasia or subvalvular or supravalvular, or f
pulmonary hypoplasia or PR
Re-intervention is indicated if
• Recurrent RVOTO (same criteria)
• Severe pulmonic regurgitation assoc’d with reduced functional capacity or
deteriorating RV function or substantial TR or sustained atrial or
ventricular arrhythmias
Sustained VT and/or SCD without reversible cause should receive an ICD (2°
prevention)
All patients should have ACHD follow-up, preconception counselling and
follow-up during pregnancy.
Endocarditis prophylaxis is recommended for unrepaired TOF, 6 months
following surgery, if there is a prosthetic valve or VSD patch leak (residual
shunt)

31. TOF
Class IIa & IIb recommendations*
Complete corrective surgery should be considered for all patients, unless severe irreversible
Pulm HTN or inadequate pulmonary arteries, esp. if:
• Worsening Sx
• Cyanosis with erythrocytosis
• Reduction of absence of shunt murmur (suspected stenosis/occlusion)
• Aneurysm formation at the site of a shunt
• LV dilation due to AR or Shunt (volume overload)
Re-Intervention may be indicated for:
• Free PR with
• progressive or moderate-to-severe RV dilatation (RVEDV > 170cc/m2)
• Modereate to severe RV dysfn
• Atrial or ventricular arythmias
• Decreasing exercise tolerance
• Residual VSD with a shunt > 1.5:1
• Residual RVOTO with RVSP/SBP >2/3
• Significant AR with LV systolic dysfunction or symptoms
• AoRoot > 55mm
• RVOT aneurysm, false aneurysm, or infection
• Sustained atrial arrhythmias or monomorphic VT (eliminate hemodynamic cause and consult
EP)
• The combination of mild-moderate residual VSD, RVOTO, or PR leading to RV dilatation or
dysfn or worsening symptoms

32. TOF
Class IIa & IIb recommendations (cont’d)*
Patients with high risk (>3.5%/yr) features for SCD may benefit
from ICD (1° prevention)
• Previous palliative shunt
• QRS > 180ms
• Non-sustained VT
• Inducible VT
• Check if Hx of palpitations/syncope, older age at repair,
transannular patch, QRS>180, non-sustained VT
• LV dysfunction

33. TOF
Class III recommendations* (i.e. don’t do it)
• NO IE prophylaxis post repair, unless prosthetic valve or leak at
site of VSD patch.

34. EBSTEIN
Class I recommendations*
Intervention (ACHD surgeon, preserve native TV) is indicated for the
following:
• NYHA>II
• Cardiothoracic ratio greater than 65%
• Resting O2-Sat <90%
• Severe TR with Sx
• TIA or stroke (if associated ASD/PFO)
• NOTE: if percutaneous closure, test occlusion is mandatory!
All patients should have ACHD follow-up
• Cyanosis? Cardiomegaly? RV dysfn? TR? TS? Arrhythmias? AVB?

35. EBSTEIN
Class IIa & IIb recommendations*
There are none…

36. EBSTEIN
Class III recommendations* (i.e. don’t do it)
• NO IE prophylaxis unless cyanotic, 6 months post repair, or TV
prosthesis.

37. MARFAN’S
Class I recommendations*
Dx of Marfans should be made according to the Ghent criteria
• Skeletal Criterion, Ocular Involvement and positive FamHx or genetic testing
• Skeletal Criterion, Ocular Criterion, Cardiac Involvement
All patients with significantly dilated aortic root or malignant family history should
avoid isometric exercise, competitive or contact sports, and scuba diving (abrupt
pressure changes predispose to PTX)
Surgical Intervention for
• AoRoot/AscAo >50mm
• AoRoot/AscAo >45mm and
• Rapid progression (>5mm/yr)
• Progressive AR and valve-sparing surgery possible
• Family history of dissection @ <50mm
• Severe MR that requires surgery
• AoRoot/AscAo >44mm and pregnancy desired
• Other parts of the Aorta >50-60mm or progressive dilatation
• Severe MR with Sx or LV dilatation/dysfn (as per valve guidelines)
Longterm surveillance of the entire aorta is indicated even after repair
• AoRoot/AscAo >50mm

38. MARFAN’S
Class I recommendations (cont’d)*
Follow-up in specialized multidisciplinary setting
• Annual Echo & MRI/CT Q3yrs
• Annual MRI/CT if any dilated segment is approachingsirgical
indication
• MRI/CT within 1 year of repair
• Annual MRI/CT for minimum 3 years post-dissection, esp. if
unrepaired

39. MARFAN’S
Class IIa & IIb recommendations*
All patients with Marfan’s should receive BB therapy, especially if
dissection has occurred or root is dilated
• Strict blood pressure control paramount
• Losartan may slow the progress of root dilatation (peds
series)
All patients with Marfan’s should receive BB therapy, especially if
dissection has occurred or root is dilated
• Strict blood pressure control paramount
• Losartan may slow the progress of root dilatation (peds
series)

40. MARFAN’S
Class III recommendations* (i.e. don’t do it)
• NO IE prophylaxis unless 6 months post repair or prosthetic
valve.

41. COMPLEX CONGENITAL
LESIONS
• Complete Transposition of the Great Arteries (TGA)
• Congenitally Corrected Transposition (ccTGA)
• Fontan Circulation
• Single Ventricle Physiology
• Eisenmenger Syndrome
• Cyanotic Heart Disease

42. TGA
Class I recommendations*
All patients should be followed by an ACHD specialist (especially
surrounding pregnancy) and all advanced investigations should be
performed by staff with expertise in ACHD.
Before pacemaker or ICD lead implantation, patency and anatomy of
superior venous conduits should be assessed.
Aggressive management of atrial arrhythmias with catheter ablation.
• Catheter ablation of ventricular arrhythmias may also be warranted
• r/o reversible hemodynamic cause of arrhythmia
Sustained VT or SCD without a reversible cause -> ICD
Endocarditis prophylaxis is indicated post Rastelli (valved conduit)

43. TGA
Class IIa & IIb recommendations*
Re-intervention may be warranted if
• Atrial Switch and
• Systemic AV valve (tricuspid) regurgitation without significant RV dysfn
• SVC or IVC obstruction (IVC obstruction worse!)
• Pulmonary Venous pathway obstruction
• Baffle leak resulting in significant L-to-R shunt (>1.5), Sx, Phtn, or
progressive ventricular dilatation/dsfn
• Baffle leak resulting in R-to-L shunt & Sx
• ArterialSwitch and
• Significant RVOTO
• Coronary obstruction
• Severe neo-AR
• Severe neo-AoRoot dilatation
• Rastelli and
• Significant RV-PA conduit obstruction
• Severe RV-PA regurgitation with Sx, progressive RV dilatation, or
arrhythmias
• Severe subaortic obstruction across the tunnel (mean gradient>50)
• Significant PA branch stenosis

44. TGA
Class IIa & IIb recommendations (cont’d)*
Patients with high risk features for SCD may benefit from ICD (1°
prevention)
• Sx of arrhythmias
• Documented AF/AFL
• Systemic ventricular dysfunction
• Older Age

45. TGA
Class III recommendations* (i.e. don’t do it)
• NO IE prophylaxis post atrial or arterial switch, unless (+) Hx
endocarditis, residual VSD after patch closure, or 6 months
following repair with prosthetic patch or device implantation.

46. CCTGA
Class I recommendations*
All patients should be followed by an ACHD specialist (especially
surrounding pregnancy) and all advanced investigations should be
performed by staff with expertise in ACHD.
Pacemakers are indicated for 3°AVB or advanced 2°AVB or documented
asystole >3sec.
• Monitor ventricular function post implantation!
• Deterioration of systemic RV function reported

47. CCTGA
Class IIa & IIb recommendations*
(Re-)intervention may be warranted if
• VSD or residual VSD
• Moderate to severe SAVV regurgitation
• Hemodynamically significant RVOTO
• Significant stenosis across LV/RV-PA conduit or LV-Ao tunnel
• Deteriorating systemic RV function
Closure of intracardiac shunts should be considered prior to PM
implantation.
If closure of shunts not possible, epicardial leads should be
considered.

48. CCTGA
Class III recommendations* (i.e. don’t do it)
• NO IE prophylaxis unless cyanotic, prosthetic valve or conduit,
residual VSD or Hx of endocarditis

49. FONTAN
Class I recommendations*
All patients should be followed by an ACHD specialist (especially
surrounding pregnancy) and all advanced investigations should be
performed by staff with expertise in ACHD.
Patients with a history atrial thrombus, VTE, interatrial communication
(ASD or fenestration), or atrial arrhythmias should receive warfarin
Re-intervention isindicated for
• Obstruction of Fontan circuit
• Obstruction of PV return
• > moderate SAVV regurgitation
• Development of venous collaterals or pulmonary AVM’s resulting in
cyanosis
• Residual ASD or fenestration with R-to-L shunt
• Subaortic obstruction (>30 peak-peak)
• PLE with high systemic venous pressures or Fontan abnormality
• Recurrent/poorly tolerated atrial arrhythmias refractory to medical Tx

50. FONTAN
Class I recommendations*
Heart transplantation should be considered for severe single-ventricle
dysfunction and Sx HF despite optimal Tx
Heart transplantation should be considered for refractory PLE.
• CHD patients have poorer survival following transplantation
When arrhythmias are present, r/o hemodynamic cause & refer to EP
with expertise in CHD (very complicated and low long term arrhythmia-free
survival)
• CHD patients have poorer survival following transplantation
All women with Fontan should have ACHD consultation prior to
pregnancy.

51. FONTAN
Class IIa & IIb recommendations*
Patients with PM/ICD should receive warfarin.
All Fontan patients without a Class I indication for warfarin should
be considered for warfarin therapy.
It may be reasonable to treat ventricular dysfunction with diuretics,
ACE-inhibitors, and BB. (extension of HF data)
Serious refractory arrhythmia may warrant conversion to TCPC
with concomitant MAZE
(Re-)intervention may be warranted if
• VSD or residual VSD
• Moderate to severe SAVV regurgitation
• Hemodynamically significant RVOTO
• Significant stenosis across LV/RV-PA conduit or LV-Ao tunnel
• Deteriorating systemic RV function

52. FONTAN
Class III recommendations* (i.e. don’t do it)
• NO IE prophylaxis unless cyanotic, recent redo (6months),
prosthetic valve or extracardiac conduit material, residual patch
leaks or Hx of endocarditis

53. SINGLE VENTRICLE
Class I recommendations*
All patients should be followed by an ACHD specialist at least annually
and all advanced investigations should be performed by staff with
expertise in ACHD.
All women contemplating pregnancy should have a comprehensive
evaluation prior.
Endocarditic prophylaxis is indicated for (all) single-ventricle patients
• High rate of cyanosis
• Guidelines do not suggest room for individualization according to
resting or exercise O2-Sat

54. SINGLE VENTRICLE
Class IIa & IIb recommendations*
There are none…

55. SINGLE VENTRICLE
Class III recommendations* (i.e. don’t do it)
There are none…

56. EISENMENGER’S
Class I recommendations*
• Cardiac catheterization (if absolutely necessary) should be performed in ACHD
centers.
• All patients should be followed by an ACHD +/- PAH specialist.
• Annual comprehenisve clinical assessment, including labs
• Imaging every 2-3 years
• All women should receive preconception/contraception counselling by an
obstetrician/gynecologist with experience in high-risk pregnancy.
• Tubal ligation, intratubal stents, or progestin-only preparations
• If patient is already pregnant and wished to pursue, f/u should be with
specialized multidisciplinary team.
• All patients should be counselled not to smoke, use drugs, and to avoid
dehydration or heat exposure and excessive physical activity.
• Additionnally, the ACHD specialist should be advised if non-cardiac surgery is
proposed or if there has been a serious illness.
• Any pulmonary infection should be treated immediately
• Exception oral hygiene and regular dental check-up Q6mos
• Endocarditis prophylaxis for all (cyanotic)

57. EISENMENGER’S
Class I recommendations*
• Phlebotomy should only be performed in patients with proven Sx
due to erythrocytosis.
• Prevention of iron deficiency is important
• Sinus rhythm should be restored/maintained on an individualized basis
• Transvenous pacing should be avoided (risk of paradoxical embolism and
closure CIx)
• ICD implantation is high-risk. It may be considered for 2° prevention and
epicardial leads should be favoured.
• Patients with AF/AFL should receive warfarin

58. EISENMENGER’S
Class IIa & IIb recommendations*
• Pulmonary vasodilator therapy may improve quality of life
• Endothelin antagonists are beneficial and well tolerated
• Prostacyclins probably work, but carry risk and burden of IV therapy
• PDE5-inhibitors have been little studied in Eisenmenger’s
• Patients should likely be refered to a specialized center such
therapy

59. EISENMENGER’S
Class III recommendations* (i.e. don’t do it)
Unless unavoidable, the following should not be attempted
• Pregnancy (extremely high risk)
• Maternal and fetal mortality each approach 50% with each pregnancy
• Maternal mortality also increased post-partum (up to 3-4 weeks)
• Non-cardiac surgery, General Anesthesia
• If necessary, should have a cardiac anesthetist on hand
• Early ambulation and ICU post-op
• Hemoptysis should be aggressively investigated & ACO stopped
• Vasoldilators
• Estrogen-containing OCP’s
• NSAIDs
• Agents that impact renal function & platelet function
• Cardiac catheterization
• IV acces! (filter, filter, filter)
• Acute High altitude (>2500m)
• Strenuous Exercise
• Heat exposure & dehydration

60. CYANOTIC
Heart Disease
Class I recommendations*
• NSAIDs should be avoided.
• Platelets, FFP, cryoprecipitate, Vit K, and ddAVP can be administered
to treat severe bleeding
• Iron deficiency (anemia) should be treated
• Hydration should be prescribed prior to procedures involving
contrast media
• Symptomatic gout or hyperuricemia should be treated with
colchicine or probenacid and prophylaxis with allopurinol is
indicated.
• Patients with worsening symptoms, cyanosis, or functiona capacity
should be considered for intervention
• Complete repair should be considered in those eligible
• If you have irreversible PAH, you are ineligible
• Refer for palliative procedures/therapies or transplant

61. CYANOTIC
Heart Disease
Class I recommendations*
• All patients should have ACHD f/u including
• Annual clinical visits, incl. labs
• Imaging Q2-3yrs
• Endocarditis prophylaxis for all

62. CYANOTIC
Heart Disease
Class IIa & IIb recommendations*
• Patients may undergo phlebotomy to a target Hct<65% prior to
surgery.
• FFP’s can be substituted for volume replacement if specific
factor deficiencies are documented.

63. CYANOTIC
Heart Disease
Class III recommendations* (i.e. don’t do it)
Women with Eisenmenger’s should avoid pregnancy
• Non-Eisenmenger’s Pregnancy still high risk
• O2-Sat >85% better