Adult Congenital Heart Disease Clinical Practice Guidelines (ESC, 2020)

European Society of Cardiology

This is a quick summary of the guidelines without analysis or commentary. For more information, go directly to the guidelines by clicking the link in the reference.

November 17, 2020

The European Society of Cardiology (ESC) updated their 2010 guidelines on the management of adult congenital heart disease (ACHD) in 2020.[1,2] Class I and III recommendations are outlined.

Arrhythmias in ACHD

Refer patients with moderate and severe CHD complexity and documented arrhythmias to a center with a multidisciplinary team and expertise in ACHD patients and ACHD-related arrhythmia.

Refer CHD patients with documented arrhythmias or at high risk for postprocedural arrhythmias (eg, atrial septal defect [ASD] closure at older age) considered for percutaneous or surgical (re)interventions to a center with a multidisciplinary team with expertise in these interventions and in invasive treatment of arrhythmias.

In mild CHD, the ESC recommends catheter ablation over long-term medical therapy for symptomatic, sustained recurrent supraventricular tachycardia (SVT) (atrioventricular node reentrant tachycardia [AVNRT], atrioventricular reentrant tachycardia [AVRT], atrial tachycardia [AT], and intraatrial reentrant tachycardia [IART]), or if SVT is potentially related to sudden cardiac death (SCD).

Catheter ablation is indicated as adjunctive therapy to implantable cardioverter defibrillators (ICDs) in those who have recurrent monomorphic ventricular tachycardia (VT), incessant VT, or electrical storm refractory to medical therapy or ICD reprogramming.

ICD implantation is indicated in adults with CHD who have:

  • Survived an aborted cardiac arrest due to ventricular fibrillation (VF) or hemodynamically untolerated VT after evaluation to define the event etiology and exclude reversible causes

  • Sustained VT after hemodynamic evaluation and repair when indicated

Electrophysiologic (EP) evaluation is required to identify those in whom catheter or surgical ablation may be a beneficial adjunctive therapy or may offer a reasonable alternative.

CHD-Associated Pulmonary Arterial Hypertension (PAH)

Counsel patients with CHD and confirmed precapillary pulmonary hypertension (PH) against pregnancy.

All patients with PAH-CHD should undergo risk assessment.

In low- and intermediate-risk patients with repaired simple lesions and precapillary PH, initial oral combination therapy or sequential combination therapy is recommended; treat high-risk patients with initial combination therapy including parenteral prostanoids.

ASD (native and residual)

ASD closure is recommended regardless of symptoms in those with evidence of right ventricular (RV) volume overload without PAH (no noninvasive signs of pulmonary arterial pressure [PAP] elevation or invasive proof of pulmonary vascular resistance [PVR] <3 Wood units [WU] in case of such signs) or left ventricular (LV) disease.

Device closure is recommended as the method of choice for secundum ASD closure when technically suitable. In seniors not candidates for device closure, carefully weigh the surgical risk against the potential benefit of ASD closure.

In patients with noninvasive signs of PAP elevation, invasive PVR measurement is required. In patients with LV disease, perform balloon testing, and carefully weigh the benefit of eliminating left-to-right (LR) shunt against the potential negative impact of ASD closure on outcome due to an increase in filling pressure (when considering closure, fenestrated closure, and no closure).

ASD closure is not recommended in those with Eisenmenger physiology, those with PAH and PVR ≥5 WU despite targeted PAH treatment, or exercise desaturation.

Ventricular Septal Defect (VSD) (native and residual)

VSD closure is recommended regardless of symptoms in patients with evidence of LV volume overload without PAH (no noninvasive signs of PAP elevation or invasive proof of PVR <3 WU in case of such signs).

VSD closure is not recommended in those with Eisenmenger physiology and those with severe PAH (PVR ≥5 WU) who present with exercise desaturation.

Atrioventricular Septal Defect (AVSD)

Surgical repair is not recommended in those with Eisenmenger physiology and patients with PAH (PVR ≥5 WU) who present with exercise desaturation.

Surgical closure performed by a congenital cardiac surgeon is recommended in patients with significant RV volume overload. Valve surgery, preferably AV valve repair, performed by a congenital cardiac surgeon is recommended in symptomatic patients with moderate to severe AV valve regurgitation.

In asymptomatic patients with severe left-sided AV valve regurgitation, valve surgery is recommended when LV end systolic diameter (ESD) is ≥45 mm and/or LV ejection fraction (EF) is ≤60% after ruling out other causes of LV dysfunction.

Patent Ductus Arteriosus (PDA)

In patients with evidence of LV volume overload and no PAH (no noninvasive signs of PAP elevation or invasive confirmation of PVR <3 WU in case of such signs), PDA closure is recommended regardless of symptoms.

Device closure is recommended as the method of choice when technically suitable.

PDA closure is not recommended in patients with Eisenmenger physiology and patients with lower limb desaturation on exercise.

Valvular Aortic Stenosis (AS)

Intervention is recommended in:

  • All symptomatic patients with severe high-gradient AS (mean gradient ≥40 mmHg)

  • Those with severe low-flow, low-gradient (mean gradient <40 mmHg) AS with reduced EF and evidence of flow (contractile) reserve excluding pseudosevere AS

  • Asymptomatic patients with severe AS and an abnormal exercise test showing clear AS-related exertional symptoms

  • Asymptomatic patients with severe AS and systolic LV dysfunction (LVEF <50%) not due to another cause

Surgery is recommended when those with severe AS undergo surgery of the ascending aorta or of another valve, or coronary artery bypass graft (CABG).

Supravalvular AS

Surgery is recommended in patients with:

  • Symptoms (spontaneous or on exercise test) and mean Doppler gradient ≥40 mmHg

  • Doppler gradient <40 mmHg, in the presence of ≥1 of the following findings: Symptoms attributable to obstruction (exertional dyspnea, angina, syncope); LV systolic dysfunction (EF <50% otherwise unexplained); surgery required for significant coronary artery disease (CAD) or valvular disease

Subaortic Stenosis

Surgery is recommended in symptomatic patients (spontaneous or on exercise test) with a mean Doppler gradient ≥40 mmHg or severe aortic regurgitation (AR).

Coarctation and Recoarctation of the Aorta

Repair of coarctation or recoarctation (surgically or catheter based) is indicated in hypertensive patients with an increased noninvasive gradient between the upper and lower limbs confirmed with invasive measurement (peak-to-peak ≥20 mmHg); catheter treatment (stenting) preferred when technically feasible.

Aortic Surgery in Aortopathies

Aortic valve repair (using the reimplantation or remodeling with aortic annuloplasty technique) performed by experienced surgeons is recommended in young patients with Marfan syndrome or related heritable thoracic aortic disease (HTAD) with aortic root dilation and tricuspid aortic valves.

Surgery is indicated in patients with Marfan syndrome who have aortic root disease with a maximal aortic sinus diameter ≥50 mm.

RV Outflow Tract Obstruction (OTO)

In valvular pulmonary stenosis (PS), balloon valvuloplasty is the intervention of choice, if anatomically suitable.

As long as no valve replacement is required, RVOTO intervention at any level is recommended regardless of symptoms when the stenosis is severe (Doppler peak gradient >64 mmHg).

If surgical valve replacement is the only option, it is indicated in (1) symptomatic patients with severe stenosis; or (2) asymptomatic patients with severe stenosis in the presence of ≥1 of the following:

  • Objective decrease in exercise capacity

  • Falling RV function and/or progression of tricuspid regurgitation (TR) to at least moderate

  • RV systolic pressure (SP) >80 mmHg

  • Right-to-left (RL) shunting via an ASD or VSD

Ebstein Anomaly

Surgical repair is recommended in patients with severe TR and symptoms or objective deterioration of exercise capacity. Surgical repair should be performed by a congenital cardiac surgeon with expertise in Ebstein surgery.

If tricuspid valve (TV) surgery is indicated, ASD/PFO (patent foramen ovale) closure is recommended at the time of valve repair if it is expected to be hemodynamically tolerated.

In patients with symptomatic arrhythmias or electrocardiographic (ECG) preexcitation, EP testing followed by ablation therapy, if feasible, or surgical treatment of the arrhythmias in the case of planned heart surgery is recommended.

After Repair of Tetralogy of Fallot

Pulmonary valve replacement (PVRep) is recommended in symptomatic patients with severe pulmonary regurgitation (PR) and/or at least moderate RVOTO.

In those without a native outflow tract, catheter intervention (transcatheter pulmonary valve implantation [TPVI]) is preferred if anatomically feasible.

Transposition of the Great Arteries (TGA)

In symptomatic TGA patients after atrial switch operation with:

  • Pulmonary venous atrium obstruction, surgical repair is recommended (catheter intervention is rarely possible)

  • Baffle stenosis not amenable to catheter intervention, surgical repair is recommended

  • Baffle leaks not amenable to catheter-based closure, surgical repair is recommended

  • Baffle stenosis, stenting is recommended when technically feasible

  • Baffle leaks and cyanosis at rest or during exercise, or with strong suspicion of paradoxical emboli, stenting (covered) or device closure is recommended when technically feasible

PA banding, as LV training with subsequent arterial switch procedure, is not recommended in TGA adults after atrial switch operation.

In TGA patients after atrial switch operation who have baffle leaks and symptoms due to LR shunt, stenting (covered) or device closure is recommended when technically feasible.

After arterial switch operation, for TGA patients with ischemia due to coronary artery stenosis, stenting or surgery (depending on substrate) is recommended.

For symptomatic patients with congenitally corrected TGA who have severe TR and preserved or mildly impaired systemic RV systolic function (EF >40%), TV replacement is indicated.

Right Ventricular to Pulmonary Artery Conduits

Symptomatic patients with RVSP >60 mmHg (may be lower in case of reduced flow) and/or severe PR should undergo intervention, with catheter intervention (TPVI) preferred if anatomically feasible.

Univentricular Heart (UVH)

Adults with unoperated or palliated UVHs should undergo careful evaluation in specialized centers, including multimodality imaging as well as invasive work-up, to determine whether surgical or interventional procedures may provide benefit.

After Fontan Operation

Sustained atrial arrhythmia with rapid AV conduction is a medical emergency; treat promptly with electrical cardioversion.

Anticoagulation is indicated in the presence, or with a history, of atrial thrombus, atrial arrhythmias, or thromboembolic events.

Counsel women with a Fontan circulation and any complication against pregnancy.

Cardiac catheterization is recommended at a low threshold in cases of unexplained edema, exercise deterioration, new-onset arrhythmia, cyanosis, and hemoptysis.

Anomalous Coronary Arteries

To confirm/exclude myocardial ischemia in patients with coronary anomalies nonpharmacologic functional imaging is recommended (eg, nuclear study, echocardiography, or cardiovascular magnetic resonance [CMR] with physical stress).

Anomalous Coronary Arteries from the Pulmonary Artery (CAPA)

Surgery is recommended in patients with:

  • Anomalous left CAPA (ALCAPA)

  • Anomalous right CAPA (ARCAPA) and symptoms attributable to anomalous coronary artery

Anomalous Aortic Origin of the Coronary Artery (AAOCA)

Surgery is recommended for AAOCA in patients with typical angina symptoms who show evidence of stress-induced myocardial ischemia in a matching territory or high-risk anatomy.

Surgery is not recommended for anomalous aortic origin of the right coronary artery (AAORCA) in asymptomatic patients without myocardial ischemia and without high-risk anatomy.

For more information, please go to Atrial Septal Defect, Patent Ductus Arteriosus (PDA), Aortic Stenosis, Tetralogy of Fallot (TOF) in Adults, Transposition of the Great Arteries, Congenitally Corrected Transposition, Aortic Coarctation, Ebstein Anomaly, and Anomalous Left Coronary Artery From the Pulmonary Artery.

For more Clinical Practice Guidelines, please go to Guidelines.

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