Professional Documents
Culture Documents
management of adult
congenital heart disease
(ACHD)
2020 ESC Guidelines for the management of
adult congenital heart disease (ACHD)
Task Force Members:
1Representing the Association for European Paediatric and Congenital Cardiology (AEPC)
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554) 2
2020 ESC Guidelines for the management of
adult congenital heart disease (ACHD)
ESC entities having participated in the development of this document:
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554) 3
ESC Classes of recommendations
Definition Wording to use
Class I Evidence and/or general agreement that a
Is recommended
given treatment or procedure is beneficial,
or is indicated
useful, effective.
Class II Conflicting evidence and/or a divergence of opinion about the
usefulness/efficacy of the given treatment or procedure.
Class IIa Weight of evidence/opinion is in favour
Should be considered
of usefulness/efficacy.
Class IIb Usefulness/efficacy is less well
May be considered
established by evidence/opinion.
Class III Evidence or general agreement that the given
treatment or procedure is not useful/effective, Is not recommended
©ESC
and in some cases may be harmful.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
ESC Levels of evidence
©ESC
evidence C studies, registries.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Selected revised recommendations (R), new recommendations (N),
and new concepts (1)
Arrhythmia
N There were no formal recommendations on arrhythmia in the 2010 edition – these have
now been included.
Summary of the most important items:
• Emphasizing the importance of understanding the cause, the mechanism of the
arrhythmia, and the anatomy of the underlying CHD
• Emphasizing the importance of a multidisciplinary approach for optimal arrhythmia
treatment prior to, or concomitant with, percutaneous or surgical interventions
• Consideration of early catheter ablation as an alternative to long-term medical
treatment for symptomatic SVT and VT provided that the procedure is performed in
©ESC
experienced centres
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Selected revised recommendations (R), new recommendations (N),
and new concepts (2)
Arrhythmia (continued)
N • Targeting VT-related anatomical isthmuses in repaired tetralogy of Fallot patients
with sustained VTs before percutaneous or surgical reintervention in the RVOT, as
these procedures may lead to inaccessibility of the VT substrates
• Recognizing the association between bradycardia and IART and the potential benefit
©ESC
of PM implantation.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Selected revised recommendations (R), new recommendations (N),
and new concepts (3)
Eisenmenger sydrome /pulmonary arterial hypertension
N It is recommended that patients with CHD and confirmed pre-capillary PH are counselled
against pregnancy.
N Risk assessment is recommended in all patients with PAH-CHD.
N In low- and intermediate-risk patients with repaired simple lesions and pre-capillary PH,
initial oral combination therapy or sequential combination therapy is recommended and
high-risk patients should be treated with initial combination therapy including parenteral
prostanoids.
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Selected revised recommendations (R), new recommendations (N),
and new concepts (4)
Eisenmenger sydrome /pulmonary arterial hypertension (continued)
R Emphasize the importance of sequential In Eisenmenger patients with reduced
PAH therapy strategy in Eisenmenger exercise capacity (6MWT distance <450 m),
syndrome and use of 6MWT for the a treatment strategy with initial endothelin
decision to initiate therapy. receptor antagonist monotherapy should be
considered followed by combination therapy
©ESC
if patients fail to improve.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Selected revised recommendations (R), new recommendations (N),
and new concepts (5)
Shunt lesions
N In patients with shunt lesions and non-invasive signs of PAP elevation, invasive
measurement of PVR is mandatory.
N/R Adjusted recommendations for shunt closure (when Qp:Qs >1.5) according to calculated PVR:
<3 WU: class I for ASD, VSD, and PDA
3–5 WU: class IIa for ASD, VSD, and PDA
≥5 WU but decreasing to <5 WU after targeted PAH treatment: class IIb for ASD
(fenestrated closure only)
≥5 WU for VSD and PDA (careful individual decision in expert centres); class IIb
≥5 WU despite targeted PAH treatment: class III for ASD.
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Selected revised recommendations (R), new recommendations (N),
and new concepts (6)
Shunt lesions (continued)
N In patients with ASD and LV disease, it is recommended to perform balloon testing and
carefully weigh the benefit of eliminating L–R shunt against the potential negative impact
of ASD closure on outcome due to increase in filling pressure (taking closure, fenestrated
closure, and no closure into consideration).
N Take older age into account in the In elderly patients not suitable for device closure,
decision for surgical ASD closure. it is recommended to weigh the surgical risk
against the potential benefit of ASD closure.
R Transcatheter VSD closure has Transcatheter VSD closure has become an
become an alternative to surgery in alternative, particularly in residual VSDs, VSDs
selected patients, particularly in poorly accessible by surgery, and in muscular VSDs
©ESC
residual VSD. centrally located in the interventricular septum.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Selected revised recommendations (R), new recommendations (N),
and new concepts (7)
Shunt lesions (continued)
R Specify the requirement for a Surgical closure is recommended in patients with
congenital cardiac surgeon for significant RV volume overload and should only be
partial AVSD closure. performed by a congenital cardiac surgeon.
R Specify the presence of AF or PH as In asymptomatic patients with severe left-sided AV
requirements for valve repair in valve regurgitation, preserved LV function
AVSD. (LVESD <45 mm and/or LVEF >60%), high likelihood
of successful valve repair, and low surgical risk,
intervention should be considered when atrial
©ESC
fibrillation or systolic PAP >50 mmHg is present.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Selected revised recommendations (R), new recommendations (N),
and new concepts (8)
Shunt lesions (continued)
R Specify the option of fenestrated In patients with PVR ≥5 WU, fenestrated ASD
ASD closure. closure may be considered when PVR falls below
5 WU after targeted PAH treatment and significant
L–R shunt is present (Qp:Qs >1.5).
R Include desaturation on exercise as a Shunt closure is not recommended in patients with
contraindication for ASD, VSD, AVSD, severe PAH (PVR ≥5 WU) presenting with
and PDA closure. desaturation on exercise.
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Selected revised recommendations (R), new recommendations (N),
and new concepts (9)
Left ventricular outflow tract obstruction and aortopathies
R Increase the recommendation class Intervention is indicated in patients with severe
from IIa to I in low-flow, low- low-flow, low-gradient (mean gradient <40 mmHg)
gradient AS for intervention. AS with reduced EF and evidence of flow
(contractile) reserve excluding pseudosevere AS.
R Lower mean Doppler gradient In symptomatic patients with valvular, subvalvular,
threshold for LVOTO intervention or supravalvular AS and mean Doppler gradient
from 50 to 40 mmHg. ≥40 mmHg surgery is recommended.
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Selected revised recommendations (R), new recommendations (N),
and new concepts (10)
Left ventricular outflow tract obstruction and aortopathies (continued)
R Include BNP levels and increased Intervention should be considered in
PAP in the indication for asymptomatic patients with normal EF and no
intervention in valvular AS. exercise test abnormalities if the surgical risk is low
and one of the following findings is present:
• Markedly elevated BNP levels (>3-fold age- and
sex-corrected normal range) confirmed by
repeated measurements without other
explanation
• Severe PH (systolic PAP at rest >60 mmHg
confirmed by invasive measurement) without
other explanation.
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Selected revised recommendations (R), new recommendations (N),
and new concepts (11)
Left ventricular outflow tract obstruction and aortopathies (continued)
R Confirm pressure gradients by Repair of coarctation or re-coarctation (surgically
invasive measurement and prefer or catheter based) is indicated in hypertensive
stenting in coarctation and re- patients with an increased non-invasive gradient
coarctation when technically between upper and lower limbs confirmed by
feasible. invasive measurement (peak-to-peak ≥20 mmHg),
with preference for catheter treatment (stenting)
when technically feasible.
N Catheter treatment (stenting) of coarctation should be considered in normotensive
patients with an increased non-invasive gradient confirmed with invasive measurement
(peak-to-peak ≥20 mmHg) when technically feasible.
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Selected revised recommendations (R), new recommendations (N),
and new concepts (12)
Left ventricular outflow tract obstruction and aortopathies (continued)
N In aortopathies, aortic valve repair using the reimplantation or remodelling with aortic
annuloplasty technique is recommended in young patients with Marfan syndrome or
related HTAD with aortic root dilation and tricuspid aortic valves, when performed by
experienced surgeons.
N Surgery should be considered in patients with a TGFBR1 or TGFBR2 mutation (including
Loeys–Dietz syndrome) who have aortic root disease with maximal aortic sinus diameter
≥45 mm.
N In Turner syndrome, elective surgery for aneurysms of the aortic root and/or ascending
aorta should be considered for women who are >16 years of age, have an ascending ASI
>25 mm/m2, and have associated risk factors for aortic dissection.
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Selected revised recommendations (R), new recommendations (N),
and new concepts (13)
Left ventricular outflow tract obstruction and aortopathies (continued)
N In Turner syndrome, elective surgery for aneurysms of the aortic root and/or ascending
aorta may be considered for women who are >16 years of age, have an ascending aortic
size index >25 mm/m2, and do not have associated risk factors for aortic dissection.
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Selected revised recommendations (R), new recommendations (N),
and new concepts (14)
Right ventricular outflow tract obstruction / tetralogy of Fallot /Ebstein
R Adjust recommendations for If surgical valve replacement is the only option, it is
surgical intervention in indicated in patients with severe stenosis who are
RVOTO according to symptomatic.
symptoms.
If surgical valve replacement is the only option in patients
with severe stenosis who are asymptomatic, it is indicated
in the presence of one or more of the following:
• Objective decrease in exercise capacity
• Decreasing RV function and/or progressive TR
to at least moderate
• RVSP >80 mmHg
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• R–L shunting via an ASD or VSD.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Selected revised recommendations (R), new recommendations (N),
and new concepts (15)
Right ventricular outflow tract obstruction / tetralogy of Fallot /Ebstein (continued)
R Include preference for catheter In patients with no native outflow tract, catheter
intervention for pulmonary valve intervention (TPVI) should be preferred if
replacement in TOF. anatomically feasible.
R Specify RV dilatation in the setting Pulmonary valve replacement should be
of pulmonary valve replacement for considered in asymptomatic patients with severe
TOF and for RVOT conduits. PR and/or RVOTO, in the presence of progressive
RV dilation to RVESVi ≥80 mL/m2, and/or RVEDVi
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≥160 mL/m2, and/or progression of TR to at least
moderate.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Selected revised recommendations (R), new recommendations (N),
and new concepts (16)
Right ventricular outflow tract obstruction / tetralogy of Fallot /Ebstein (continued)
R In case of ASDs in Ebstein In the case of documented systemic embolism probably
anomaly, add caution about caused by paradoxical embolism, isolated device closure
RA pressure rise or fall in of ASD/PFO should be considered but requires careful
cardiac output. evaluation before intervention to exclude induction of RA
pressure increase or fall in cardiac output.
If cyanosis (oxygen saturation at rest <90%) is the leading
problem, isolated device closure of ASD/PFO may be
considered but requires careful evaluation before
intervention to exclude induction of RA pressure increase
or fall in cardiac output.
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Selected revised recommendations (R), new recommendations (N),
and new concepts (17)
Transposition of the great arteries
R Downgrade level of In patients with severe systemic (tricuspid) AV
recommendation for AV valve repair valve regurgitation without significant ventricular
in TGA/atrial switch from I to IIa in systolic dysfunction (EF >40%), valve repair or
symptomatic patients. replacement should be considered regardless of
symptoms.
R In patients with TGA/atrial switch In patients with a baffle leak who require a
requiring PM/ICD implantation: pay PM/ICD, closure of the baffle leak should be
attention to the presence of baffle considered, when technically feasible, prior to
leaks. insertion of transvenous leads.
N In ccTGA, biventricular pacing should be considered in case of complete AV block or >40%
©ESC
ventricular pacing requirement.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Selected revised recommendations (R), new recommendations (N),
and new concepts (18)
Transposition of the great arteries (continued)
R Revise indications for TV In symptomatic patients with severe TR and
replacement in ccTGA, according to preserved or mildly impaired systemic RV systolic
symptoms and systemic ventricular function (EF >40%), TV replacement is indicated.
function.
(Upgrade level of recommendation In asymptomatic patients with severe TR and
for symptomatic ccTGA patients progressive systemic RV dilatation and/or mildly
from IIa to I). impaired systemic RV systolic function (EF >40%),
TV replacement should be considered.
In symptomatic patients with severe TR and more
than mildly reduced systemic RV systolic function
(EF ≤40%), TV replacement may be considered.
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Selected revised recommendations (R), new recommendations (N),
and new concepts (19)
Transposition of the great arteries (continued)
R Anatomic repair (atrial + arterial
switch) for ccTGA removed from the
©ESC
recommendations.
Univentricular heart
N It is recommended that adults with unoperated or palliated UVHs undergo careful
evaluation in specialized centres including multimodality imaging as well as invasive work-
up to decide whether they may benefit from surgical or interventional procedures.
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Selected revised recommendations (R), new recommendations (N),
and new concepts (20)
Fontan circulation
N Sustained atrial arrhythmia with rapid AV conduction is a medical emergency and should
promptly be treated with electrical cardioversion.
N Anticoagulation is indicated in the presence, or with a history, of atrial thrombus, atrial
arrhythmias, or thromboembolic events.
N It is recommended that women with a Fontan circulation and any complication are
counselled against pregnancy.
N Cardiac catheterization is recommended at a low threshold in cases of unexplained
oedema, exercise deterioration, new-onset arrhythmia, cyanosis, and haemoptysis.
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Selected revised recommendations (R), new recommendations (N),
and new concepts (21)
Fontan circulation (continued)
N In patients with arrhythmias, a proactive approach of electrophysiological evaluation and
ablation (where appropriate) should be considered.
N Regular liver imaging (ultrasound, computed tomography, magnetic resonance) should be
considered.
N Endothelin receptor antagonists and phosphodiesterase-5 inhibitors may be considered in
selected patients with elevated pulmonary pressures/resistance in the absence of
elevated ventricular end diastolic pressure.
N In selected patients with significant cyanosis, device closure of a fenestration may be
considered but requires careful evaluation before intervention to exclude induction of
©ESC
systemic venous pressure increase or fall in cardiac output.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Selected revised recommendations (R), new recommendations (N),
and new concepts (22)
Coronary artery anomalies
N Non-pharmacological functional imaging (e.g. nuclear study, echocardiography or CMR
with physical stress) is recommended in patients with coronary anomalies to
confirm/exclude myocardial ischaemia.
A) Anomalous coronary artery from the pulmonary artery (ACAPA)
N Surgery is recommended in patients with ALCAPA.
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dysfunction, or myocardial ischaemia attributable to coronary anomaly.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Selected revised recommendations (R), new recommendations (N),
and new concepts (23)
Coronary artery anomalies (continued)
B) Anomalous aortic origin of a coronary artery (AAOCA)
N Surgery is recommended for AAOCA in patients with typical angina symptoms who
present with evidence of stress-induced myocardial ischaemia in a matching territory or
high-risk anatomy.
N Surgery should be considered in asymptomatic patients with AAOCA (right or left) and
evidence of myocardial ischaemia.
N Surgery should be considered in asymptomatic patients with AAOLCA and no evidence
of myocardial ischaemia but a high-risk anatomy.
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Selected revised recommendations (R), new recommendations (N),
and new concepts (24)
Coronary artery anomalies (continued)
B) Anomalous aortic origin of a coronary artery (AAOCA)
N Surgery may be considered for symptomatic patients with AAOCA even if they have
neither evidence of myocardial ischaemia nor high-risk anatomy.
N Surgery may be considered for asymptomatic patients with AAOLCA without myocardial
ischaemia and without high-risk anatomy when they present at young age (<35 years).
N Surgery is not recommended for AAORCA in asymptomatic patients without myocardial
ischaemia and without high-risk anatomy.
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Selected revised recommendations (R), new recommendations (N),
and new concepts (25)
New concepts
Staffing requirements for an ACHD expert centre. Categories of high-risk pregnancies in accordance
with the pregnancy guidelines.
Emerging role of biomarkers in follow-up of ACHD. Expand Marfan syndrome section to aortopathies
(and include HTAD, Turner syndrome, and bicuspid
aortic disease).
©ESC
More specific and adjusted recommendations for
PAH treatment.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Classification of congenital heart disease complexity (1)
Mild
• Isolated congenital aortic valve disease and bicuspid aortic disease
• Isolated congenital mitral valve disease (except parachute valve, cleft leaflet)
• Mild isolated pulmonary stenosis (infundibular, valvular, supravalvular)
• Isolated small ASD, VSD, or PDA
• Repaired secundum ASD, sinus venosus defect, VSD, or PDA without residuae or sequellae,
such as chamber enlargement, ventricular dysfunction, or elevated PAP.
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Classification of congenital heart disease complexity (2)
©ESC
• Ebstein anomaly
• Marfan syndrome and related HTAD, Turner Syndrome
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Classification of congenital heart disease complexity (3)
©ESC
• VSD with associated abnormalities (excluding pulmonary vascular disease) and/or moderate or
greater shunt.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Classification of congenital heart disease complexity (4)
©ESC
heterotaxy syndromes, ventricular inversion).
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Congenital heart disease
A lifelong chronic condition
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Staff requirements for specialist ACHD centres
©ESC
(Modified from Baumgartner H, et al. European Heart Journal. 2014)
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Staff requirements for specialist ACHD
©ESC
(Modified from Baumgartner H, et al. European Heart Journal. 2014)
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Indications for cardiovascular magnetic resonance imaging in
ACHD patients (1)
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• Collaterals and arteriovenous malformations (CCT may be superior)
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Indications for cardiovascular magnetic resonance imaging in
ACHD patients (2)
©ESC
• Quantification of systemic and pulmonary blood flow to calculate Qp:Qs
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Indications for cardiovascular magnetic resonance imaging in
ACHD patients (3)
Indications for CMR in ACHD patients
• Quantification of perfusion distribution to the right/left lung
• Measurement of pulmonary blood flow in patients with multiple sources of blood supply
(i.e. with major aorto-pulmonary collateral arteries)
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Risk estimates for arrhythmic events and bradycardias in ACHD (1)
supraventricular
arrhythmias with
rapid AV conduction.
cVT higher estimated
risk in complex
dextro-TGA.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Risk estimates for arrhythmic events and bradycardias in ACHD (2)
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for treatment of arrhythmias in ACHD (1)
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for treatment of arrhythmias in ACHD (2)
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for treatment of arrhythmias ACHD (3)
©ESC
reasonable alternative.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for treatment of arrhythmias in ACHD (4)
©ESC
electrical stimulation.
*Considering
the broad spectrum of ACHD with LV pathologies that may differ from acquired heart diseases, the potentially high er risk of ICD-related complications in
ACHD and the paucity of data on the benefit of ICDs for primary prevention of SCD in ACHD, a personalized approach seems appr opriate.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for treatment of arrhythmias in ACHD (5)
©ESC
risk factors.*
*Data are sparse and risk factors may be lesion specific, including non-sustained VT, NYHA II/III, severe AV valve regurgitation, wide QRS ≥140 ms (TGA).
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for treatment of arrhythmias in ACHD (6)
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Definitions of pulmonary hypertension subtypes and their
occurrence in ACHD (1)
©ESC
segmental PAH)
*The most recent definition of PH lowers mean PAP from ≥25 mmHg to >20 mmHg but additionally requires a PVR ≥3 WU for pre-capillary PH
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Definitions of pulmonary hypertension subtypes and their
occurrence in ACHD (2)
©ESC
capillary PH in combination with
shunt lesions/complex CHD
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for treatment of pulmonary arterial
hypertension associated with congenital heart disease
Recommendations Class Level
It is recommended that patients with CHD and confirmed pre-capillary PHa
I C
are counselled against pregnancy.
Risk assessment is recommended in all patients with PAH-CHD.b I C
In low- and intermediate-risk patients with repaired simple lesions and
pre-capillary PH, initial oral combination therapy or sequential combination
I A
therapy is recommended and high-risk patients should be treated with initial
combination therapy including parenteral prostanoids.c
In Eisenmenger patients with reduced exercise capacity
(6MWT distance <450 m), a treatment strategy with initial endothelin
IIa B
receptor antagonist monotherapy should be considered followed by
©ESC
combination therapy if patients fail to improve.
aThe risk of pregnancy in patients with pre-capillary PH is very high. It may be less in pregnancies of patients with post-capillary PH, therefore, a right heart catheterization
is necessary in all patients with a suspicion of pre-capillary PH to confirm the diagnosis. - bFor details see 2015 ESC/ERS Guidelines for diagnosis & treatment of PH. - cFor
details about choice of drugs and the recommended risk-adjusted treatment algorithm see 2015 ESC/ERS Guidelines for diagnosis & treatment of PH.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Risk reduction strategies in patients with cyanotic congenital
heart disease (1)
Prophylactic measures are the mainstay of care to avoid complications.
The following exposures/activities should be avoided:
• Pregnancy in patients with Eisenmenger syndrome and in cyanotic patients without PAH,
but arterial oxygen saturation <90%
• Iron deficiency and anaemia (no routine, inappropriate phlebotomies to maintain a
pre-determined haemoglobin) – treat iron deficiency and iron-deficient anaemia
• Inappropriate anticoagulation
• Dehydration
• Infectious disease: administer influenza and pneumococcal vaccines
• Cigarette smoking, recreational drug abuse including excessive alcohol
©ESC
• Transvenous PM/ICD leads
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Risk reduction strategies in patients with cyanotic congenital
heart disease (2)
Prophylactic measures are the mainstay of care to avoid complications.
The following exposures/activities should be avoided:
• Strenuous exercise
• Acute exposure to heat (sauna, hot tub, or hot shower) or extreme cold
• Oestrogen-containing contraception
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Risk reduction strategies in patients with cyanotic congenital
heart disease (3)
Other risk reduction strategies include:
• Use of an air filter in an intravenous line to prevent air embolism
• Consultation with an ACHD cardiologist before administration of any agent and performance
of any surgical/interventional procedure
• Prompt antibiotic therapy of upper respiratory tract infections
• Cautious use or avoidance of agents that impair renal function
• Contraceptive advice at each clinic visit
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Congenital heart disease with high risk and extremely high risk
for pregnancy (1)
Significantly increased risk of maternal Extremely high risk of maternal mortality or
mortality or severe morbidity (mWHO class severe morbidity (mWHO class IV)*
III) (cardiac event rate 19–27%) (cardiac event rate 40–100%)
Unrepaired cyanotic heart disease Pulmonary arterial hypertension
Moderate LV impairment (EF 30–45%) Severe LV impairment (EF <30% or NYHA class
III–IV)
Systemic RV with good or mildly decreased Systemic RV with moderate or severely
ventricular function decreased ventricular function
Fontan circulation. If the patient is otherwise Fontan with any complication
well and the cardiac condition uncomplicated
Severe asymptomatic AS Severe symptomatic AS
©ESC
*Pregnancy should definitely be avoided in women with these conditions.
Modified from the ESC Guidelines for the management of cardiovascular disease during pregnancy.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Congenital heart disease with high risk and extremely high risk
for pregnancy (2)
Significantly increased risk of maternal Extremely high risk of maternal mortality or
mortality or severe morbidity (mWHO class severe morbidity (mWHO class IV)*
III) (cardiac event rate 19–27%) (cardiac event rate 40–100%)
Moderate mitral stenosis Severe mitral stenosis
Moderate aortic dilatation (40–45 mm in Severe aortic dilatation (>45 mm in Marfan
Marfan syndrome or other HTAD; 45–50 mm syndrome or other HTAD, >50 mm in BAV,
in BAV, 20–25 mm/m2 in Turner syndrome) >25 mm/m2 in Turner syndrome)
Mechanical valve Severe (re-)coarctation
©ESC
*Pregnancy should definitely be avoided in women with these conditions.
Modified from the ESC Guidelines for the management of cardiovascular disease during pregnancy.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recurrence rates for various congenital heart lesions according
to the sex of the affected parenta
Recurrence rate (%)* Recurrence rate (%)*
Women Men
Women Men
RVOTO (PS) 4–6.5 2–3.5
ASD 4–6 1.5–3.5
Eisenmenger 6 NR
VSD 6–10 2–3.5 syndrome
AVSD 11.5–14 1–4.5 TOF 2–2.5 1.5
PDA 3.5–4 2–2.5 Pulmonary NR NR
atresia/VSD
CoA 4–6.5 2–3.5 TGA 2b
Marfan/HTAD 50b ccTGA 3–5b
LVOTO 8–18 3–4 UVH (HLHS) 21b
©ESC
©ESC aModified from Pierpont ME et al. Circulation 2018;138:e653-e711
* Except for Marfan syndrome, rates apply to patients with isolated cardiac
bSex-specific data not available or not relevant.
lesions in whom known genetic/syndromic entities have been excluded
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for intervention in atrial septal defect
(native and residual) (1)
Recommendations Class Level
In patients with evidence of RV volume overload * and no PAH (no non-
invasive signs of PAP elevation or invasive confirmation of PVR <3 WU in case
I B
of such signs) or LV disease, ASD closure is recommended regardless of
symptoms.
Device closure is recommended as the method of choice for secundum ASD
I C
closure when technically suitable.
In elderly patients not suitable for device closure, it is recommended to
I C
carefully weigh the surgical risk against the potential benefit of ASD closure.
In patients with non-invasive signs of PAP elevation, invasive measurement
I C
©ESC
of PVR is mandatory.
*RV enlargement with increased stroke volume.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for intervention in atrial septal defect
(native and residual) (2)
Recommendations Class Level
In patients with LV disease, it is recommended to perform balloon testing
and carefully weigh the benefit of eliminating L–R shunt against the potential
negative impact of ASD closure on outcome due to an increase in filling I C
pressure (taking closure, fenestrated closure, and no closure into
consideration).
In patients with suspicion of paradoxical embolism (exclusion of other
causes), ASD closure should be considered regardless of size providing there IIa C
is absence of PAH and LV disease.
In patients with PVR 3–5 WU, ASD closure should be considered when
IIa C
©ESC
significant L–R shunt is present (Qp:Qs >1.5).
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for intervention in atrial septal defect
(native and residual) (3)
Recommendations Class Level
In patients with PVR ≥5 WU, fenestrated ASD closure may be considered
when PVR falls below 5 WU after targeted PAH treatment and significant L–R IIb C
shunt is present (Qp:Qs >1.5).
ASD closure is not recommended in patients with Eisenmenger physiology,
patients with PAH and PVR ≥5 WU despite targeted PAH treatment, or III C
©ESC
desaturation on exercise.*
*Thereare limited data available for a precise cut-off, but by clinical experience, this would definitely be given by a fall of arterial oxygen saturation
below 90%.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
aRV enlargement with
increased stroke volume.
bProviding there is no PAH
or LV disease.
cIn elderly patients not
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for intervention in ventricular septal
defect (native and residual) (1)
Recommendations Class Level
In patients with evidence of LV volume overload * and no PAH (no non-
invasive signs of PAP elevation or invasive confirmation of PVR <3 WU in case I C
of such signs), VSD closure is recommended regardless of symptoms.
In patients with no significant L–R shunt but a history of repeated episodes of
IIa C
IE, VSD closure should be considered.
In patients with VSD-associated prolapse of an aortic valve cusp causing
IIa C
progressive AR, surgery should be considered.
In patients who have developed PAH with PVR 3–5 WU, VSD closure should
IIa C
©ESC
be considered when there is still significant L–R shunt (Qp:Qs >1.5).
*LV enlargement with increased stroke volume.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for intervention in ventricular septal
defect (native and residual) (2)
Recommendations Class Level
In patients who have developed PAH with PVR ≥5 WU, VSD closure may be
considered when there is still significant L–R shunt (Qp:Qs >1.5), but careful IIb C
individual decision in expert centres is required.
VSD closure is not recommended in patients with Eisenmenger physiology
and patients with severe PAH (PVR ≥5 WU) presenting with desaturation on III C
©ESC
exercise.*
*There are limited data available for a precise cut-off, but by clinical experience, this would definitely be given by a fall of
arterial oxygen saturation below 90%.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
aLV enlargement with increased stroke volume.
©ESC
bIncludes all patients with desaturation at rest (Eisenmenger physiology) or on exercise
cCareful individual decision in expert centres is required.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for intervention in atrioventricular
septal defect (1)
Recommendations Class Level
Complete AVSD
Surgical repair is not recommended in patients with Eisenmenger physiology
and patients with PAH (PVR ≥5 WU) presenting with desaturation on III C
exercise.*
For recommendations on intervention see also recommendations for intervention in VSD.
Partial AVSD (primum ASD)
Surgical closure is recommended in patients with significant RV volume
I C
overload and should only be performed by a congenital cardiac surgeon.
©ESC
For further details see recommendations for intervention in ASD.
*There are limited data available for a precise cut-off, but by clinical experience, this would definitely be given by a fall of arterial oxygen saturation below 90%.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for intervention in atrioventricular
septal defect (2)
Recommendations Class Level
AV Valve regurgitation
Valve surgery, preferably AV valve repair, is recommended in symptomatic
patients with moderate to severe AV valve regurgitation and should be I C
performed by a congenital cardiac surgeon.
In asymptomatic patients with severe left-sided AV valve regurgitation, valve
surgery is recommended when LVESD ≥45 mm * and/or LVEF ≤60% provided I C
other causes of LV dysfunction are excluded.
In asymptomatic patients with severe left-sided AV valve regurgitation,
preserved LV function (LVESD <45 mm * and/or LVEF >60%), high likelihood of
IIa C
successful valve repair, and low surgical risk, intervention should be
considered when atrial fibrillation or systolic PAP >50 mmHg is present.
©ESC
Left ventricular outflow tract obstruction
See recommendations for intervention in SubAS.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
*Cut-off refers to average-sized adults and may require adaption in patients with unusually small or large stature .
Recommendations for intervention in patent ductus arteriosus
©ESC
aLV enlargement with increased stroke volume.
bThere are limited data available for a precise cut-off, but by clinical experience, this would definitely be given by a fall of arterial oxygen saturation below 90%.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
aLV enlargement with increased stroke volume.
bIncludes all patients with lower limb desaturation at rest (Eisenmenger physiology) or on exercise.
cCareful individual decision in expert centres is required.
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Diagnostic criteria for degree of aortic stenosis severity
©ESC
*At normal transvalvular flow.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for intervention in valvular aortic stenosis (1)
©ESC
systolic LV dysfunction (LVEF <50%) not due to another cause.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for intervention in valvular aortic stenosis (2)
©ESC
measurement) without other explanation.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for intervention in valvular aortic stenosis (3)
©ESC
additional valve replacement.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
aThere are fundamental
differences in management
decisions compared to valvular
AS, particularly because a valve
substitute with its consequences
is generally not required.
bPeak velocity >5.5m/s, severe
©ESC
explanation).
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for intervention in supravalvular
aortic stenosis
Recommendations Class Level
In patients with symptoms (spontaneous or on exercise test) and mean
I C
Doppler gradient ≥40 mmHg, surgery is recommended.
In patients with mean Doppler gradient <40 mmHg, surgery is recommended
when one or more of the following findings are present:
• Symptoms attributable to obstruction (exertional dyspnoea, angina,
I C
syncope)
• LV systolic dysfunction (EF <50% without other explanation)
• Surgery required for significant CAD or valvular disease.
Patients with mean Doppler gradient ≥40 mmHg * – but without symptoms,
LV systolic dysfunction, LVH, or abnormal exercise test – may be considered IIb C
for repair when the surgical risk is low.
©ESC
*Doppler-derived gradients may overestimate the obstruction and may need confirmation by left heart catheterization .
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for intervention in subaortic stenosis (1)
©ESC
below baseline on exercise.
aDoppler-derived gradients may overestimate the obstruction and may need confirmation by cardiac catheterization.
bSee 2017 ESC Guidelines on the management of valvular heart disease.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for intervention in subaortic stenosis (2)
©ESC
prevent further progression).
*Doppler-derived gradients may overestimate the obstruction and may need confirmation by cardiac catheterization.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for intervention in coarctation and
re-coarctation of the aorta (1)
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for intervention in coarctation and
re-coarctation of the aorta (2)
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
aRight arm ambulatory blood pressure should be considered for diagnosis.
bInvasively confirmed measurement
cRelative to the aortic diameter at the diaphragm .
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for aortic surgery in aortopathies (1)
©ESC
hypertension, and/or aortic size increase >3 mm/year (on repeated measurements using the same ECG-gated imaging technique measured at the same level of the
aorta with side-by-side comparison and confirmed by another technique).
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for aortic surgery in aortopathies (2)
the same ECG-gated imaging technique, measured at the same level of the aorta with side-by-side comparison and confirmed by another technique).
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for aortic surgery in aortopathies (3)
©ESC
*BAV, elongation of the transverse aorta, CoA, and/or hypertension.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for intervention in right ventricular
outflow tract obstruction (1)
Recommendations Class Level
In valvular PS, balloon valvuloplasty is the intervention of choice, if
I C
anatomically suitable.
Provided that no valve replacement is required, RVOTO intervention at any
level is recommended regardless of symptoms when the stenosis is severe I C
(Doppler peak gradient is >64 mmHga).
If surgical valve replacement is the only option, it is indicated in patients with
I C
severe stenosis who are symptomatic.b
©ESC
aRVSP estimated from TR velocity should confirm severe PS.
bThe threshold for intervention is higher when a valve substitute is required because long-term risks such as endocarditis and re-intervention for prosthetic valve
failure need to be taken into account.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for intervention in right ventricular
outflow tract obstruction (2)
Recommendations Class Level
If surgical valve replacement is the only option * in patients with severe
stenosis who are asymptomatic, it is indicated in the presence of one or more
of the following:
• Objective decrease in exercise capacity I C
• Decreasing RV function and/or progression of TR to at least moderate
• RVSP >80 mmHg
• R–L shunting via an ASD or VSD.
Intervention in patients with a Doppler peak gradient <64 mmHg should be
considered in the presence of one or more of the following:
©ESC
• Symptoms related to PS IIa C
• Decreasing RV function and/or progressive TR to at least moderate
• R–L shunting via an ASD or VSD.
*The threshold for intervention is higher when a valve substitute is required because long-term risks such as endocarditis and re-intervention for prosthetic valve
failure need to be taken into account.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for intervention in right ventricular
outflow tract obstruction (3)
©ESC
RVSP >50 mmHg and/or related reduced lung perfusion is present.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
©ESC
aIn peripheral PS, regardless of symptoms, catheter interventional treatment should be considered if >50% diameter narrowing
and RVSP >50 mmHg and/or related reduced lung perfusion is present.
bIn valvular PS, balloon valvuloplasty is the intervention of choice if anatomically suitable.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for intervention in Ebstein anomaly (1)
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for intervention in Ebstein anomaly (2)
©ESC
IIb C
before intervention to exclude induction of RA pressure increase or fall in
cardiac output.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Management of repaired tetralogy of Fallot: long-term
complications to address during follow-up
©ESC
Ao = aorta; LV = left ventricle;
RV = right ventricle; PA =
Pulmonary artery; TV =
tricuspid valve; RA = right
atrium; RV = right ventricular;
LA = left atrium; PA =
pulmonary artery; PS =
pulmonary stenosis; SCD =
sudden cardiac death; VSD =
ventricular septal defect.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for intervention after repair of tetralogy
of Fallot (1)
Recommendations Class Level
Pulmonary valve replacement is recommended in symptomatic patients with
I C
severe PRa and/or at least moderate RVOTO.b
In patients with no native outflow tract,c catheter intervention (TPVI) should
I C
be preferred if anatomically feasible.
Pulmonary valve replacement should be considered in asymptomatic
patients with severe PR and/or RVOTO when one of the following criteria is
present:
• Decrease in objective exercise capacity
IIa C
• Progressive RV dilation to RVESVi ≥80 mL/m2 and/or RVEDVi ≥160mL/m2d
and/or progression of TR to at least moderate
• Progressive RV systolic dysfunction
©ESC
• RVOTO with RVSP >80 mmHg.
aRegurgitantfraction by CMR >30–40%. - bPeak velocity >3 m/s - cPatients with previous RVOT surgery using homografts, bovine jugular vein grafts,
bioprostheses/conduits - dConfirmed by repeated measurements.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for intervention after repair of tetralogy
of Fallot (2)
Recommendations Class Level
VSD closure should be considered in patients with residual VSD and
significant LV volume overload or if the patient is undergoing pulmonary IIa C
valve surgery.
In patients with sustained VT who are undergoing surgical pulmonary valve
replacement or transcutaneous valve insertion, pre-operative catheter
IIa C
mapping and transsection of VT-related anatomical isthmuses before or
during the intervention should be considered.
Electrophysiologic evaluation, including programmed electrical stimulation,
should be considered for risk stratification for SCD in patients with additional
IIa C
risk factors (LV/RV dysfunction; non-sustained, symptomatic VT; QRS
©ESC
duration ≥180 ms, extensive RV scarring on CMR).
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for intervention after repair of tetralogy
of Fallot (3)
Recommendations Class Level
ICD implantation should be considered in selected TOF patients with multiple
risk factors for SCD, including LV dysfunction, non-sustained, symptomatic
IIa C
VT, QRS duration ≥180 ms, extensive RV scarring on CMR, or inducible VT at
programmed electrical stimulation.
Catheter ablation or concomitant surgical ablation for symptomatic
monomorphic sustained VT may be considered in those with a preserved
biventricular function as an alternative to ICD therapy, provided that the
IIb C
procedure is performed in highly experienced centres and that established
ablation endpoints have been reached (e.g. non-inducibility, conduction
©ESC
block across ablation lines).
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Management of transposition of the great arteries:
long-term complications to address during follow-up
Ao = aorta; AV = atrioventricular; IVC = inferior caval vein; LA = left atrium; LV = left ventricle; LVOT = left ventricular (subpulmonary) outflow tract; PS = pulmonary stenosis
©ESC
(supravalvular/pulmonary artery branch); PV = pulmonary vein; PVA = pulmonary venous atrium; RA = right atrium; RV = right ventricle; SCD = sudden cardiac death; SN-dysf. = sinus node
dysfunction; SVA = systemic venous atrium; SVC = superior vena cava; TV = tricuspid valve.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for intervention in transposition of the great
arteries after atrial switch operation (1)
Recommendations Class Level
Indications for surgical intervention
In symptomatic patients with pulmonary venous atrium obstruction, surgical
I C
repair (catheter intervention rarely possible) is recommended.
In symptomatic patients with baffle stenosis not amenable to catheter
I C
intervention, surgical repair is recommended.
In symptomatic patients with baffle leaks not amenable to catheter-based
I C
closure, surgical repair is recommended.
In patients with severe systemic (tricuspid) AV valve regurgitation, without
significant ventricular systolic dysfunction (EF >40%), valve repair or IIa C
replacement should be considered, regardless of symptoms.
PA banding in adults, as LV training with subsequent arterial switch
III C
procedure, is not recommended.
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for intervention in transposition of the great
arteries after atrial switch operation (2)
Recommendations Class Level
Indications for catheter intervention
In symptomatic patients with baffle stenosis, stenting is recommended when
I C
technically feasible.
In symptomatic patients with baffle leaks and cyanosis at rest or during
exercise, or with strong suspicion of paradoxical emboli, stenting (covered) or I C
device closure is recommended when technically feasible.
In patients with baffle leaks and symptoms due to L–R shunt, stenting
I C
(covered) or device closure is recommended when technically feasible.
In asymptomatic patients with baffle leaks with substantial ventricular
volume overload due to L–R shunt, stenting (covered) or device closure IIa C
©ESC
should be considered when technically feasible.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for intervention in transposition of the great
arteries after atrial switch operation (3)
Recommendations Class Level
Indications for catheter intervention (continued)
In patients with a baffle leak who require a PM/ICD, closure of the baffle leak
with a covered stent should be considered, when technically feasible, prior to IIa C
insertion of transvenous leads.
In asymptomatic patients with baffle stenosis, stenting may be considered
IIb C
©ESC
when technically feasible.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for interventions in transposition of the great
arteries after arterial switch operation
Recommendations Class Level
Stenting or surgery (depending on substrate) is recommended for coronary
I C
artery stenosis causing ischaemia.
Neo-aortic root surgery should be considered when the neo-aortic root is
>55 mm, providing average adult stature (for neo-aortic valve replacement
IIa C
for severe neo-aortic AR see valvular heart disease guidelines with special
considerations*).
Stenting should be considered for PA branch stenosis, regardless of
symptoms, if >50% diameter narrowing and RVSP >50 mmHg and/or related IIa C
©ESC
reduced lung perfusion are present.
*When applying the ESC valvular heart disease guidelines for the decision to operate, it has to be taken into account that thi s is a reoperation and technically more difficult.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for interventions in congenitally corrected
transposition of the great arteries
Recommendations Class Level
In symptomatic patients with severe TR and preserved or mildly impaired
I C
systemic RV systolic function (EF >40%), TV replacement is indicated.
In asymptomatic patients with severe TR and progressive systemic RV
dilatation and/or mildly impaired systemic RV systolic function (EF >40%), TV IIa C
replacement should be considered.
Biventricular pacing should be considered in case of complete AV block or
IIa C
>40% ventricular pacing requirement.
In symptomatic patients with severe TR and more than mildly reduced
IIb C
©ESC
systemic RV systolic function (EF ≤40%), TV replacement may be considered.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for intervention in right ventricular to
pulmonary artery conduits
Recommendations Class Level
Symptomatic patients with RVSP >60 mmHg (may be lower in case of
reduced flow) and/or severe PR* should undergo intervention with I C
preference for catheter intervention (TPVI) if anatomically feasible.
Asymptomatic patients with severe RVOTO and/or severe PR should be
considered for intervention, preferably catheter intervention (TPVI) if
anatomically feasible, when at least one of the following criteria is present:
• Decrease in objective exercise capacity (CPET)
IIa C
• Progressive RV dilation to RVESVi ≥80 mL/m2 and/or
RVEDVi ≥160 mL/m2 and/or progression of TR to at least moderate
• Progressive RV systolic dysfunction
• RVSP >80 mmHg.
©ESC
*Regurgitant fraction by CMR >30–40%.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Special considerations and recommendations for intervention in
univentricular heart (1)
Recommendations Class Level
It is recommended that adults with unoperated or palliated UVHs undergo
careful evaluation in specialized centres, including multimodality imaging as
I C
well as invasive work-up to decide whether they may benefit from surgical or
interventional procedures.
Only well-selected symptomatic cyanotic patients, after careful evaluation
[low pulmonary vascular resistances, adequate function of the AV valve(s),
IIa C
preserved ventricular function], should be considered candidates for a
Fontan circulation.
Patients with increased pulmonary blood flow – unlikely at adult age – should
IIa C
be considered for PA banding or tightening of a previously placed band.
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Special considerations and recommendations for intervention in
univentricular heart (2)
Recommendations Class Level
Patients with severe cyanosis and decreased pulmonary blood flow, but
without elevated PVR or PAP, should be considered for a bidirectional Glenn IIa C
shunt.
Patients with severe cyanosis and decreased pulmonary blood flow not
IIb C
suitable for a Glenn shunt may be considered for a systemic-to-PA shunt.
Heart transplantation and heart-lung transplantation should be considered
when there is no conventional surgical option in patients with poor clinical IIa C
©ESC
status.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Special considerations and recommendations for intervention
after Fontan operation (1)
Recommendations Class Level
Sustained atrial arrhythmia with rapid AV conduction is a medical emergency
I C
and should be promptly treated with electrical cardioversion.
Anticoagulation is indicated in the presence, or with a history, of atrial
I C
thrombus, atrial arrhythmias, or thromboembolic events.
It is recommended that women with a Fontan circulation and any
I C
complication are counselled against pregnancy.
Cardiac catheterization is recommended at a low threshold in cases of
unexplained oedema, exercise deterioration, new-onset arrhythmia, I C
©ESC
cyanosis, and haemoptysis.
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Special considerations and recommendations for intervention
after Fontan operation (2)
Recommendations Class Level
In patients with arrhythmias, a proactive approach of electrophysiologic
IIa C
evaluation and ablation (where appropriate) should be considered.
Regular liver imaging (ultrasound, computed tomography, magnetic
IIa C
resonance) should be considered.
Endothelin receptor antagonists and phosphodiesterase-5 inhibitors may be
considered in selected patients with elevated pulmonary pressure/resistance IIb C
in the absence of elevated ventricular end diastolic pressure.
In selected patients with significant cyanosis, device closure of a fenestration
may be considered but requires careful evaluation before intervention to
IIb C
exclude induction of systemic venous pressure increase or fall in cardiac
output.
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for the management of patients with
anomalous coronary arteries (1)
Recommendations Class Level
Non-pharmacological functional imaging (e.g. nuclear study,
echocardiography, or CMR with physical stress) is recommended in patients I C
with coronary anomalies to confirm/exclude myocardial ischaemia.
A) Anomalous coronary arteries from the pulmonary artery (ACAPA)
Surgery is recommended in patients with ALCAPA. I C
Surgery is recommended in patients with ARCAPA and symptoms attributable
I C
to anomalous coronary artery.
Surgery should be considered for ARCAPA in asymptomatic patients with
ventricular dysfunction, or myocardial ischaemia attributable to coronary IIa C
anomaly.
©ESC
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for the management of patients with
anomalous coronary arteries (2)
Recommendations Class Level
B) Anomalous aortic origin of the coronary artery (AAOCA)
Surgery is recommended for AAOCA in patients with typical angina symptoms
who present with evidence of stress-induced myocardial ischaemia in a I C
matching territory or high-risk anatomy.*
Surgery should be considered in asymptomatic patients with AAOCA (right or
IIa C
left) and evidence of myocardial ischaemia.
Surgery should be considered in asymptomatic patients with AAOLCA and no
IIa C
evidence of myocardial ischaemia but a high-risk anatomy.*
©ESC
*High-risk anatomy includes features such as an intramural course and orifice anomalies (slit-like orifice, acute-angle take-off, orifice >1 cm above the sinotubular
junction).
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
Recommendations for the management of patients with
anomalous coronary arteries (3)
Recommendations Class Level
B) Anomalous aortic origin of the coronary artery (AAOCA) (continued)
Surgery may be considered for symptomatic patients with AAOCA even if
IIb C
they have neither evidence of myocardial ischaemia nor high-risk anatomy.*
Surgery may be considered for asymptomatic patients with AAOLCA without
myocardial ischaemia and without high-risk anatomy* when they present at IIb C
young age (<35 years).
Surgery is not recommended for AAORCA in asymptomatic patients without
III C
myocardial ischaemia and without high-risk anatomy.*
©ESC
*High-risk anatomy includes features such as an intramural course and orifice anomalies (slit-like orifice, acute-angle take-off, orifice >1 cm above the sinotubular
junction).
2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
www.escardio.org/guidelines
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2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)
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2020 ESC Guidelines for the management of adult congenital heart disease (ACHD)
www.escardio.org/guidelines (European Heart Journal 2020 - doi/10.1093/eurheartj/ehaa554)