ESC 2020 2020 ESC Guidelines for the management of adult congenital heart disease
European Society of Cardiology｜循環器 / 先天性心疾患｜84 ページ｜チャンク 826｜推奨 155 件｜current
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| 頁 | クラス | レベル | 推奨文 | 表 |
|---|---|---|---|---|
| p.19 | I | C | In patients with moderate and severe CHD com- plexity (Table 4) and documented arrhythmias, referral to a centre with a multidisciplinary team and expertise in ACHD patients and ACHD- related arrhythmia is indicated. | |
| p.19 | I | C | In CHD patients with documented arrhythmias or at high risk for post-procedural arrhythmias (e.g. ASD closure at older age) considered for percutaneous or surgical (re)interventions, referral to a centre with a multidisciplinary team with expertise in these interventions and in invasive treatment of arrhythmias is indicated. | |
| p.19 | I | C | In mild CHD, catheter ablation is recommended over long-term medical therapy for sympto- matic, sustained recurrent SVT (AVNRT, AVRT, AT, and IART), or if SVT is potentially related to SCD (Table 7). | |
| p.19 | IIa | C | In moderate and severe CHD, catheter ablation should be considered for symptomatic, sustained recurrent SVT (AVNRT, AVRT, AT, and IART), or if SVT is potentially related to SCD (Table 7), provided that the procedure is performed in experienced centres. | |
| p.20 | I | C | Catheter ablation is indicated as adjunctive ther- apy to ICDs in patients who present with recur- rent monomorphic VT, incessant VT, or electrical storm not manageable by medical ther- apy or ICD reprogramming. | |
| p.20 | IIa | C | Catheter ablation should be considered for symptomatic, monomorphic sustained VT in patients for whom medical therapy is not desired, provided that the procedure is performed in experienced centres. | |
| p.20 | I | C | Implantable cardiac defibrillator ICD implantation is indicated in adults with CHD who are survivors of an aborted cardiac arrest due to VF or haemodynamically untoler- ated VT after evaluation to define the cause of the event and exclusion of reversible causes. | |
| p.20 | I | C | ICD implantation is indicated in adults with CHD and sustained VT after haemodynamic evaluation and repair when indicated. EP evalua- tion is required to identify patients in whom catheter ablation or surgical ablation may be beneficial as adjunctive treatment or in whom it may offer a reasonable alternative. | |
| p.20 | IIa | C | ICD implantation should be considered in adults with CHD with biventricular physiology and a systemic LV presenting with symptomatic heart failure (NYHA II/III) and EF <_35% despite >_ 3 months of optimal medical treatment, provided they are expected to survive substantially longer than one year with good functional status.c | |
| p.20 | IIa | C | ICD implantation should be considered in patients with CHD and unexplained syncope and suspicion for arrhythmia aetiology and either advanced ventricular dysfunction or indu- cible VT/VF at programmed electrical stimulation. | |
| p.20 | IIa | C | ICD implantation should be considered in selected TOF patients with multiple risk factors for SCD, including LV dysfunction, non-sustained, symptomatic VT, QRS duration >_180 ms, extensive RV scarring on CMR, or inducible VT at programmed electrical stimulation. | |
| p.20 | IIb | C | ICD implantation may be considered in patients with advanced single or systemic RV dysfunction (EF systemic RV <35%) in the presence of addi- tional risk factors.d | |
| p.20 | IIa | C | Pacemaker PM implantation should be considered in ACHD patients with bradycardia-tachycardia syndrome to prevent IART, if ablation fails or is not possible. | |
| p.20 | IIa | C | PM implantation should be considered for patients with severe CHD and sinus or | |
| p.20 | IIa | C | junctional bradycardia (daytime heart rate <40 beats per minute or pauses >3 s). PM implantation should be considered for patients with CHD and compromised haemodynamics due to sinus bradycardia or loss of AV synchrony. | |
| p.20 | IIb | C | PM implantation may be considered for patients with moderate CHD and sinus or junctional bra- dycardia (daytime heart rate <40 beats per minute or pauses >3 s). | |
| p.22 | I | C | with PAH-CHD.d | |
| p.22 | I | C | It is recommended that patients with CHD and confirmed pre-capillary PHc are counselled against pregnancy. Risk assessment is recommended in all patients | |
| p.22 | I | A | 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 highrisk patients should be treated with initial com- bination therapy including parenteral prosta- noids.e 6365 | |
| p.22 | IIa | B | In Eisenmenger patients with reduced exercise capacity (6MWT distance <450 m), a treatment strategy with initial endothelin receptor antagonist monotherapy should be considered fol- lowed by combination therapy if patients fail to improve.6769 | |
| p.30 | I | B | In patients with evidence of RV volume over- loadc and no PAH (no non-invasive signs of PAP elevation or invasive confirmation of PVR <3 WU in case of such signs) or LV disease, ASD closure is recommended regardless of symptoms.146,147 | |
| p.30 | I | C | Device closure is recommended as the method of choice for secundum ASD closure when technically suitable. | |
| p.30 | I | C | In elderly patients not suitable for device clo- sure, it is recommended to carefully weigh the surgical risk against the potential benefit of ASD closure. | |
| p.30 | I | C | In patients with non-invasive signs of PAP eleva- tion, invasive measurement of PVR is mandatory. In patients with LV disease, it is recommended | |
| p.30 | I | C | to perform balloon testing and carefully weigh the benefit of eliminating LR shunt against the potential negative impact of ASD closure on outcome due to an increase in filling pressure (taking closure, fenestrated closure, and no closure into consideration). | |
| p.30 | IIa | C | In patients with suspicion of paradoxical embo- lism (exclusion of other causes), ASD closure should be considered regardless of size provid- ing there is absence of PAH and LV disease. | |
| p.30 | IIa | C | In patients with PVR 35 WU, ASD closure should be considered when significant LR shunt is present (Qp:Qs >1.5). | |
| p.30 | IIb | C | In patients with PVR >_5 WU, fenestrated ASD closure may be considered when PVR falls below 5 WU after targeted PAH treatment and significant LR shunt is present (Qp:Qs >1.5). | |
| p.30 | III | C | ASD closure is not recommended in patients with Eisenmenger physiology, patients with PAH and PVR >_5 WU despite targeted PAH treat- ment, or desaturation on exercise.d | |
| p.33 | I | C | In patients with evidence of LV volume over- loadc and no PAH (no non-invasive signs of PAP elevation or invasive confirmation of PVR <3 WU in case of such signs), VSD closure is rec- ommended regardless of symptoms. | |
| p.33 | IIa | C | In patients with no significant LR shunt, but a history of repeated episodes of IE, VSD closure should be considered. | |
| p.33 | IIa | C | In patients with VSD-associated prolapse of an aortic valve cusp causing progressive AR, sur- gery should be considered. | |
| p.33 | IIa | C | In patients who have developed PAH with PVR 35 WU, VSD closure should be considered when there is still significant LR shunt (Qp:Qs >1.5). | |
| p.33 | IIb | C | In patients who have developed PAH with PVR >_5 WU, VSD closure may be considered when there is still significant LR shunt (Qp:Qs >1.5), but careful individual decision in expert centres is required. | |
| p.33 | III | C | VSD closure is not recommended in patients with Eisenmenger physiology and patients with severe PAH (PVR >_5 WU) presenting with desaturation on exercise.d | |
| p.35 | III | C | Complete AVSD Surgical repair is not recommended in patients with Eisenmenger physiology, and patients with PAH (PVR >_5 WU) presenting with desa- turation on exercise.c | |
| p.35 | I | C | Partial AVSD (primum ASD) Surgical closure is recommended in patients with significant RV volume overload and should only be performed by a congenital car- diac surgeon. | |
| p.35 | I | C | AV valve regurgitation Valve surgery, preferably AV valve repair, is recommended in symptomatic patients with moderate to severe AV valve regurgitation and should be performed by a congenital car- diac surgeon. | |
| p.35 | I | C | In asymptomatic patients with severe leftsided AV valve regurgitation, valve surgery is recommended when LVESD >_45 mmd and/or LVEF <_60% provided other causes of LV dysfunction are excluded. | |
| p.35 | IIa | C | In asymptomatic patients with severe left- sided AV valve regurgitation, preserved LV function (LVESD <45 mmd and/or LVEF >60%), high likelihood of successful valve repair, and low surgical risk, intervention should be considered when atrial fibrillation or systolic PAP >50 mmHg is present. Left ventricular outflow tract obstruction | |
| p.36 | I | C | In patients with evidence of LV volume overloadc and no PAH (no non-invasive signs of PAP eleva- tion or invasive confirmation of PVR <3 WU in case of such signs), PDA closure is recommended regardless of symptoms. | |
| p.36 | I | C | Device closure is recommended as the method of choice when technically suitable. | |
| p.36 | IIa | C | In patients who have developed PAH with PVR 35 WU, PDA closure should be considered when there is still significant LR shunt (Qp:Qs >1.5). | |
| p.36 | IIb | C | In patients who have developed PAH with PVR >_5 WU, PDA closure may be considered when there is still significant LR shunt (Qp:Qs >1.5) but careful individual decision in expert centres is required. | |
| p.36 | III | C | PDA closure is not recommended in patients with Eisenmenger physiology and patients with lower limb desaturation on exercise.d | |
| p.38 | I | B | Symptomatic patients with aortic valve stenosis In symptomatic patients with severe high-gradient AS (mean gradient >_40 mmHg), inter- vention is recommended.25,171,174,175 | |
| p.38 | I | C | Intervention is indicated in symptomatic patients with severe low-flow, low-gradient (mean gradient <40 mmHg) AS with reduced EF and evidence of flow (contractile) reserve excluding pseudosevere AS. | |
| p.38 | I | C | Intervention is indicated in asymptomatic patients with severe AS and an abnormal exer- cise test showing symptoms on exercise clearly related to AS. | |
| p.38 | IIa | C | Intervention should be considered in asympto- matic patients with severe AS when they present with a fall in blood pressure below baseline during exercise testing. Intervention should be considered in asymptomatic patients with normal EF and none of the above-mentioned exercise test abnormalities | |
| p.38 | IIa | C | if the surgical risk is low and one of the following findings is present: • Very severe AS defined by a Vmax >5.5 m/s. • Severe valve calcification and a rate of Vmax progression >_0.3 m/s/year. • 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) with- | |
| p.38 | I | C | out other explanation. ascending aorta surgery Surgery is recommended when patients with severe AS undergo surgery of the ascending aorta or of another valve, or CABG. | |
| p.38 | IIa | C | Patients with moderate AS undergoing CABG surgery or surgery of the ascending aorta or another valve should be considered for addi- tional valve replacement. | |
| p.40 | I | C | In patients with symptoms (spontaneous or on exercise test) and mean Doppler gradient >_40 mmHg, surgery is recommended. In patients with mean Doppler gradient <40 | |
| p.40 | I | C | mmHg, surgery is recommended when one or more of the following findings are present: Symptoms attributable to obstruction (exer- tional dyspnoea, angina, syncope). LV systolic dysfunction (EF <50% without other explanation). Surgery required for significant CAD or valvular disease. | |
| p.40 | IIb | C | Patients with mean Doppler gradient >_40 mmHgc but without symptoms, LV systolic dysfunction, LVH, or abnormal exercise test may be considered for repair when the surgical risk is low. | |
| p.41 | I | C | In symptomatic patients (spontaneous or on exercise test) with a mean Doppler gradient >_40 mmHgc or severe AR, surgery is recommended. Asymptomatic patients should be considered for surgery when one or more of the following | |
| p.41 | IIa | C | findings are present: • Mean gradient <40 mmHg but LVEF <50%. • AR is severe and LVESD >50 mm (or 25 mm/m2 BSA) and/or EF <50%d. • Mean Doppler gradient is >_40 mmHgc and marked LVH present. • Mean Doppler gradient is >_40 mmHgc and there is a fall in blood pressure below baseline on exercise. | |
| p.41 | IIb | C | Asymptomatic patients may be considered for surgery when one or more of the following findings are present: • Mean Doppler gradient is >_40 mmHg,c LV is normal (EF >50% and no LVH), exercise testing is normal, and surgical risk is low. • Progression of AR is documented and AR becomes more than mild (to prevent further progression). | |
| p.42 | I | C | Repair of coarctation or re-coarctation (surgi- cally or catheter based) is indicated in hypertensive patientsc with an increased non-invasive gradient between upper and lower limbs con- firmed with invasive measurement (peak-topeak >_20 mmHg) with preference for catheter treatment (stenting), when technically feasible. | |
| p.42 | IIa | C | Catheter treatment (stenting) should be considered in hypertensive patientsc with >_50% nar- rowing relative to the aortic diameter at the diaphragm, even if the invasive peak-to-peak gradient is <20 mmHg, when technically feasible. | |
| p.42 | IIa | C | Catheter treatment (stenting) should be consid- ered in normotensive patientsc with an increased non-invasive gradient confirmed with invasive measurement (peak-to-peak >_20 mmHg), when technically feasible. | |
| p.42 | IIb | C | Catheter treatment (stenting) may be considered in normotensive patientsc with >_50% nar- rowing relative to the aortic diameter at the diaphragm, even if the invasive peak-to-peak gradient is <20 mmHg, when technically feasible. 4.6.5 Follow-up recommendations Residua, sequelae, and complications are listed below: | |
| p.46 | I | C | Marfan syndrome and HTAD Aortic valve repair, using the reimplantation or remodelling with aortic annuloplasty tech- nique, is recommended in young patients with Marfan syndrome or related HTAD with aortic root dilation and tricuspid aortic valves, | |
| p.46 | I | C | when performed by experienced surgeons. Surgery is indicated in patients with Marfan syndrome who have aortic root disease with a maximal aortic sinus diameter >_50 mm.c | |
| p.46 | IIa | C | Surgery should be considered in patients with Marfan syndrome who have aortic root dis- ease with maximal aortic sinus diameter >_45 mmc and additional risk factors.d | |
| p.46 | IIa | C | Surgery should be considered in patients with a TGFBR1 or TGFBR2 mutation (including LoeysDietz syndrome) who have aortic root disease with maximal aortic sinus diameter >_45mm.c | |
| p.46 | IIa | C | Bicuspid aortic disease Aortic surgery should be considered if the ascending aorta is: • >_50 mm in the presence of a bicuspid valve with additional risk factorse or coarctation. • >_55 mm for all other patients. Turner syndrome | |
| p.46 | IIa | C | Elective surgery for aneurysms of the aortic root and/or ascending aorta should be consid- ered for women with Turner syndrome who are >16 years of age, have an ascending aortic size index >25 mm/m2, and have associated risk factors for aortic dissection.f | |
| p.46 | IIb | C | Elective surgery for aneurysms of the aortic root and/or ascending aorta may be consid- ered for women with Turner syndrome who are >16 years of age, have an ascending aortic size index >25 mm/m2, and do not have asso- ciated risk factors for aortic dissection.f | |
| p.47 | I | C | In valvular PS, balloon valvuloplasty is the intervention of choice, if anatomically suitable. | |
| p.47 | I | C | Provided that no valve replacement is required, RVOTO intervention at any level is recommended regardless of symptoms when the stenosis is severe (Doppler peak gradient is >64 mmHgc). | |
| p.47 | I | C | If surgical valve replacement is the only option, it is indicated in patients with severe stenosis who are symptomatic.d If surgical valve replacement is the only optiond in | |
| p.47 | I | C | patients with severe stenosis who are asympto- matic, it is indicated in the presence of one or more of the following. • Objective decrease in exercise capacity. • Decreasing RV function and/or progression of TR to at least moderate. • RVSP >80 mmHg. • RL shunting via an ASD or VSD. | |
| p.47 | IIa | C | Intervention in patients with a Doppler peak gradient <64 mmHg should be considered in the presence of one or more of the following. • Symptoms related to PS. • Decreasing RV function and/or progressive TR to at least moderate. • RL shunting via an ASD or VSD. | |
| p.47 | IIa | C | Peripheral PS, regardless of symptoms, should be considered for catheter interventional treatment if >50% diameter narrowing, and RVSP >50 mmHg, and/or related reduced lung perfusion is present. | |
| p.49 | I | C | Indications for surgery Surgical repair is recommended in patients with severe TR and symptoms or objective deterio- ration of exercise capacity. | |
| p.49 | I | C | It is recommended that surgical repair is performed by a congenital surgeon with specific experience in Ebstein surgery. | |
| p.49 | I | C | If there is an indication for TV surgery, ASD/ PFO closure is recommended at the time of valve repair if it is expected to be haemodynami- cally tolerated. | |
| p.49 | IIa | C | Surgical repair should be considered regardless of symptoms in patients with progressive right heart dilation or reduction of RV systolic function. Indications for catheter intervention | |
| p.49 | I | C | In patients with symptomatic arrhythmias, or pre-excitation on the ECG, electrophysiologic testing followed by ablation therapy, if feasible, or surgical treatment of the arrhythmias in the case of planned heart surgery is recommended. | |
| p.49 | IIa | C | In the case of documented systemic embolism, probably caused by paradoxical embolism, iso- lated device closure of ASD/PFO should be con- sidered but requires careful evaluation before intervention to exclude induction of RA pres- sure increase or fall in cardiac output. | |
| p.49 | IIb | C | If cyanosis (oxygen saturation at rest <90%) is the leading problem, isolated device closure of ASD/PFO may be considered but requires care- ful evaluation before intervention to exclude induction of RA pressure increase or fall in cardiac output. | |
| p.52 | I | C | PVRep is recommended in symptomatic patients with severe PRc and/or at least moderate RVOTO.d | |
| p.52 | I | C | In patients with no native outflow tract,e catheter intervention (TPVI) should be preferred if anatomically feasible. PVRep should be considered in asymptomatic patients with severe PR and/or RVOTO when | |
| p.52 | IIa | C | one of the following criteria is present. • Decrease in objective exercise capacity. • Progressive RV dilation to RVESVi >_80 mL/ m2, and/or RVEDVi >_160 mL/m2 f, and/or progression of TR to at least moderate. • Progressive RV systolic dysfunction. • RVOTO with RVSP >80 mmHg. | |
| p.52 | IIa | C | VSD closure should be considered in patients with residual VSD and significant LV volume overload or if the patient is undergoing pulmonary valve surgery. | |
| p.52 | IIa | C | In patients with sustained VT who are under- going surgical PVRep or transcutaneous valve insertion, pre-operative catheter mapping and transsection of VT-related anatomical isthmuses before or during the intervention should be considered. | |
| p.52 | IIa | C | Electrophysiologic evaluation, including pro- grammed electrical stimulation, should be con- sidered for risk stratification for SCD in patients with additional risk factors (LV/RV dysfunction; non-sustained, symptomatic VT; QRS duration >_180 ms, extensive RV scarring on CMR). | |
| p.52 | IIa | C | ICD implantation should be considered in selected TOF patients with multiple risk factors for SCD, including LV dysfunction, non-sustained, symptomatic VT, QRS duration >_180 ms, extensive RV scarring on CMR, or inducible VT at programmed electrical stimulation. | |
| p.52 | IIb | C | 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 procedure is per- formed in highly experienced centres and that established ablation endpoints have been reached (e.g. non-inducibility, conduction block across ablation lines). | |
| p.57 | I | C | Indications for surgical intervention In symptomatic patients with pulmonary venous atrium obstruction, surgical repair (catheter intervention rarely possible) is recommended. | |
| p.57 | I | C | In symptomatic patients with baffle stenosis not amenable to catheter intervention, surgical repair is recommended. | |
| p.57 | I | C | In symptomatic patients with baffle leaks not amenable to catheter-based closure, surgical repair is recommended. | |
| p.57 | IIa | C | In patients with severe systemic (tricuspid) AV valve regurgitation, without significant ventricular systolic dysfunction (EF >40%), valve repair or replacement should be considered, regardless of symptoms. | |
| p.57 | III | C | PA banding in adults, as LV training with subse- quent arterial switch procedure, is not recommended. | |
| p.57 | I | C | Indications for catheter intervention In symptomatic patients with baffle stenosis, stenting is recommended when technically feasible. | |
| p.57 | I | C | In symptomatic patients with baffle leaks and cyanosis at rest or during exercise, or with strong suspicion of paradoxical emboli, stent- ing (covered) or device closure is recommended when technically feasible. | |
| p.57 | I | C | In patients with baffle leaks and symptoms due to LR shunt, stenting (covered) or device closure is recommended when technically feasible. | |
| p.57 | IIa | C | In asymptomatic patients with baffle leaks with substantial ventricular volume overload due to LR shunt, stenting (covered) or device clo- sure should be considered when technically feasible. | |
| p.57 | IIa | C | In patients with a baffle leak who require a PM/ICD, closure of the baffle leak with a cov- ered stent should be considered, when technically feasible, prior to insertion of transvenous leads. | |
| p.57 | IIb | C | In asymptomatic patients with baffle stenosis, stenting may be considered when technically feasible. | |
| p.58 | I | C | Stenting or surgery (depending on substrate) is recommended for coronary artery stenosis causing ischaemia. | |
| p.58 | IIa | C | Neo-aortic root surgery should be considered when the neo-aortic root is >55 mm, providing average adult stature (for neo-aortic valve replace- ment for severe neo-aortic AR see valvular heart disease guidelines with special considerationsc). | |
| p.58 | IIa | C | Stenting should be considered for PA branch stenosis, regardless of symptoms, if >50% diam- eter narrowing and RVSP >50 mmHg and/or related reduced lung perfusion are present. | |
| p.59 | I | C | In symptomatic patients with severe TR and pre- served or mildly impaired systemic RV systolic function (EF >40%), TV replacement is indicated. | |
| p.59 | IIa | C | In asymptomatic patients with severe TR and pro- gressive systemic RV dilatation and/or mildly impaired systemic RV systolic function (EF >40%), TV replacement should be considered. | |
| p.59 | IIa | C | Biventricular pacing should be considered in case of complete AV block or >40% ventricular pacing requirement. | |
| p.59 | IIb | C | In symptomatic patients with severe TR and more than mildly reduced systemic RV systolic function (EF <_40%), TV replacement may be considered. | |
| p.60 | I | C | Symptomatic patients with RVSP >60 mmHg (may be lower in case of reduced flow) and/or severe PRc should undergo intervention with preference for catheter intervention (TPVI) if anatomically feasible. Asymptomatic patients with severe RVOTO and/ | |
| p.60 | IIa | C | 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). • 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. | |
| p.62 | I | C | 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. | |
| p.62 | IIa | C | Only well-selected symptomatic cyanotic patients, after careful evaluation [low pulmonary vascular resistances, adequate function of the AV valve(s), preserved ventricular function], should be considered candidates for a Fontan circulation. | |
| p.62 | IIa | C | Patients with increased pulmonary blood flow unlikely at adult age should be considered for PA banding or tightening of a previously placed band. | |
| p.62 | IIa | C | Patients with severe cyanosis and decreased pul- monary blood flow, but without elevated PVR or PAP, should be considered for a bidirectional Glenn shunt. | |
| p.62 | IIb | C | Patients with severe cyanosis and decreased pul- monary blood flow not suitable for a Glenn shunt may be considered for a systemic-to-PA shunt. | |
| p.62 | IIa | C | Heart transplantation and heart-lung transplan- tation should be considered when there is no conventional surgical option in patients with poor clinical status. | |
| p.65 | I | C | Sustained atrial arrhythmia with rapid AV con- duction is a medical emergency and should be promptly treated with electrical cardioversion. | |
| p.65 | I | C | Anticoagulation is indicated in the presence, or with a history, of atrial thrombus, atrial arrhythmias, or thromboembolic events. | |
| p.65 | I | C | It is recommended that women with a Fontan circulation and any complication are coun- selled against pregnancy. | |
| p.65 | I | C | Cardiac catheterization is recommended at a low threshold in cases of unexplained oedema, exercise deterioration, new-onset arrhythmia, cyanosis, and haemoptysis. | |
| p.65 | IIa | C | In patients with arrhythmias, a proactive approach of electrophysiologic evaluation and ablation (where appropriate) should be considered. | |
| p.65 | IIa | C | Regular liver imaging (ultrasound, computed tomography, magnetic resonance) should be considered. | |
| p.65 | IIb | C | Endothelin receptor antagonists and phospho- diesterase-5 inhibitors may be considered in selected patients with elevated pulmonary pressure/resistance in the absence of elevated ventricular end diastolic pressure. | |
| p.65 | IIb | C | In selected patients with significant cyanosis, device closure of a fenestration may be con- sidered but requires careful evaluation before intervention to exclude induction of systemic venous pressure increase or fall in cardiac output. AV = atrioventricular. aClass of recommendation. bLevel of evidence. | |
| p.66 | I | C | 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. | |
| p.66 | I | C | Surgery is recommended in patients with ALCAPA. | |
| p.66 | I | C | Surgery is recommended in patients with ARCAPA and symptoms attributable to anomalous coronary artery. | |
| p.66 | IIa | C | Surgery should be considered for ARCAPA in asymptomatic patients with ventricular dysfunction, or myocardial ischaemia attrib- utable to coronary anomaly. Anomalous aortic origin of the coronary artery | |
| p.66 | I | C | 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.c | |
| p.66 | IIa | C | Surgery should be considered in asympto- matic patients with AAOCA (right or left) and evidence of myocardial ischaemia. | |
| p.66 | IIa | C | Surgery should be considered in asymptomatic patients with AAOLCA and no evi- dence of myocardial ischaemia but a highrisk anatomy.c | |
| p.66 | IIb | C | Surgery may be considered for symptomatic patients with AAOCA even if there is no evidence of myocardial ischaemia or highrisk anatomy.c | |
| p.66 | IIb | C | Surgery may be considered for asymptomatic patients with AAOLCA without myocardial ischaemia and without high-risk anatomyc when they present at young age (<35 years). | |
| p.66 | III | C | Surgery is not recommended for AAORCA in asymptomatic patients without myocardial ischaemia and without high-risk anatomy.c | |
| p.70 | I | C | It is recommended that patients with CHD and confirmed pre-capillary PH are counselled against pregnancy. | |
| p.70 | I | C | Risk assessment is recommended in all patients with PAH-CHD. | |
| p.70 | I | C | Device closure is recommended as the method of choice for secundum ASD closure when technically suitable. | |
| p.70 | I | C | In patients with non-invasive signs of PAP elevation, invasive measurement of PVR is mandatory. | |
| p.71 | I | C | Device closure is recommended as the method of choice when technically suitable. | |
| p.71 | I | B | Intervention is recommended in all symptomatic patients with severe high-gradient AS (mean gradient >_40 mmHg). | |
| p.71 | I | C | Surgery is indicated in patients with Marfan syndrome who have aortic root disease with a maximal aortic sinus diameter >_50 mm. | |
| p.72 | I | C | In valvular PS, balloon valvuloplasty is the intervention of choice, if anatomically suitable. | |
| p.72 | I | C | If surgical valve replacement is the only option, it is indicated in patients with severe stenosis who are symptomatic. | |
| p.72 | I | C | Surgical repair is recommended in patients with severe TR and symptoms or objective deterioration of exercise capacity. | |
| p.72 | I | C | It is recommended that surgical repair is performed by a congenital surgeon with specific experience in Ebstein surgery. | |
| p.72 | I | C | PVRep is recommended in symptomatic patients with severe PR and/or at least moderate RVOTO. | |
| p.72 | I | C | In patients with no native outflow tract, catheter intervention (TPVI) should be preferred if anatomically feasible. | |
| p.72 | I | C | In symptomatic patients with baffle stenosis not amenable to catheter intervention, surgical repair is recommended. | |
| p.72 | I | C | In symptomatic patients with baffle leaks not amenable to catheter-based closure, surgical repair is recommended. | |
| p.72 | III | C | PA banding in adults, as LV training with subsequent arterial switch procedure, is not recommended. | |
| p.72 | I | C | In symptomatic patients with baffle stenosis, stenting is recommended when technically feasible. | |
| p.72 | I | C | Stenting or surgery (depending on substrate) is recommended for coronary artery stenosis causing ischaemia. | |
| p.72 | I | C | It is recommended that women with a Fontan circulation and any complication are counselled against pregnancy. | |
| p.73 | I | C | Surgery is recommended in patients with ALCAPA. | |
| p.73 | I | C | Surgery is recommended in patients with ARCAPA and symptoms attributable to anomalous coronary artery. |