ACHD: anatomy segments and general principles

• This page covers the general principles that apply across adult congenital heart disease: the language used to describe the anatomy, the two guideline classification systems, organisation of care, assessment, and the shared complications of heart failure, arrhythmia, pulmonary hypertension and cyanosis.^[2][1]

It also covers endocarditis, thrombosis, pregnancy, genetics, exercise and procedures.^[1][2] Lesion detail is left to the lesion pages; see Atrial septal defect and PFO: closure indications for atrial septal defect and patent foramen ovale.

ACC/AHA 2025 defines congenital heart disease as a range of structural cardiac abnormalities present before birth attributable to abnormal fetal cardiac development.^[2] That definition excludes inherited disorders that may have cardiac manifestations, such as Marfan syndrome or hypertrophic cardiomyopathy.^[2] It also excludes anatomic variants such as patent foramen ovale.^[2]

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Describing the anatomy: segments and connections

ACC/AHA 2025 uses the International Paediatric and Congenital Cardiac Code nomenclature for anatomic lesions and repairs.^[2]

• ESC 2020 lists what echocardiography provides: cardiac anatomy, situs (including orientation and position of the heart), the connection of the atria and ventricles, the heart valves, and the connection of the ventricles with the great arteries.^[1]

• For the univentricular heart, ESC 2020 states that the segmental approach is required in the echocardiographic examination; it adds that these hearts are always complex and can present with a wide range of abnormalities in situs, orientation and connections.^[1]

• Its fundamental transthoracic items include abdominal and atrial situs, the position of the heart in the chest and of the apex, and the veno-atrial, AV and ventriculo-arterial connections.^[1]

ESC 2020 fundamental TTE items for the univentricular heart (the list includes these three)

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How the guidelines define the connections in common complex lesions

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Grading complexity: ESC mild-moderate-severe and the ACC/AHA AP classification

ESC 2020: three complexity groups

ESC 2020 says congenital heart disease can be classified as mild, moderate or severe (its Table 4).^[1]

• Its arrhythmia rows use these groups: for example, in patients with moderate and severe CHD complexity and documented arrhythmias, referral to a centre with a multidisciplinary team and expertise in ACHD patients and ACHD-related arrhythmia is indicated (Class I, Level C).^[1]

ESC 2020 Table 4: classification of congenital heart disease complexity

^[1]

ACC/AHA 2025: anatomy plus physiology

The ACHD anatomic and physiological (AP) classification was introduced in the 2018 AHA/ACC guideline to characterise ACHD anatomy and physiology more uniformly.^[2] ACC/AHA 2025 says it aims to improve classification of disease severity, given the potential for discordance between anatomic complexity and physiological stage.^[2] Patients receive an anatomic complexity from simple to complex (I–III) and a physiological stage reflecting clinical status (A–D).^[2]

ACC/AHA 2025 Table 4: ACHD AP classification (CHD anatomy + physiological stage)

^[2]

• Classify by the "highest" relevant anatomic or physiological feature; with multiple lesions, the most complex anatomic diagnosis sets the anatomic complexity.^[2]
• Worked example from the guideline: repaired coarctation, asymptomatic and normotensive, with normal aortic dimensions and normal exercise capacity, is IIA; with moderate ventricular dysfunction it becomes IIC.^[2]
• Some combinations are impossible: stage A requires "no anatomic sequelae", so Fontan circulation, a systemic right ventricle or truncus arteriosus can never be stage A.^[2]
• ACC/AHA 2025 reports that multiple studies have confirmed that a higher AP classification is associated with greater short- and long-term mortality risk and that the physiological stage adds prognostic value to the anatomic classification; it adds that a higher AP classification has been associated with worse surgical outcomes and with maternal and fetal complications of pregnancy.^[2]
• The 2025 edition incorporates biomarkers such as NT-proBNP, unplanned heart failure hospitalisations and recent endocarditis, because each identifies patients with poorer prognosis and higher resource utilisation; subacute bacterial endocarditis in the past year is stage D.^[2]
• The suggested follow-up intervals in the lesion tables are not class recommendations, and testing intervals should be tailored to the patient and local resources.^[2]
• Patients with substantial acquired comorbidities unrelated to their congenital lesion may be better followed under the guidelines for acquired heart disease.^[2]

ACC/AHA 2025 Table 5: physiological variables used in the AP classification (rows used here)

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Organisation of care, transition and the ACHD centre

ESC 2020: levels of care

• ESC 2020 says that as patients with congenital heart disease approach adulthood they require transfer to ACHD care, preceded by a preparatory transition phase that continues into adulthood according to the needs of the patient.^[1]

It describes care as a lifelong process that also requires advance care planning strategies.^[1]

• The ESC working group position paper it cites stratified care into three levels: care exclusively in a specialist centre, shared care with general adult cardiac services, and management in non-specialist clinics with access to specialised care if required.^[1]
• Complexity alone should not assign the level of care, because even anatomically simple defects may need specialist care in some circumstances, for example an ASD with pulmonary arterial hypertension.^[1]
• ESC 2020 therefore recommends in its text (no class or level given) that all ACHD patients are seen once in a specialist centre, so that ACHD specialists can determine the most appropriate level of care and follow-up intervals for each individual patient.^[1]

Networks of specialist centres with general adult care should be established for each catchment area.^[1]

• A general cardiologist meeting an unstable patient should not delay treatment but ought to liaise with the patient's ACHD centre immediately about management or transfer.^[1]

ESC 2020 singles out patients after Fontan correction who present with arrhythmia, because even supraventricular arrhythmias are not well tolerated.^[1]

• Its text also recommends that specialist centres have teams including specialist nurses, psychologists and social workers, because anxiety, depression or problems coping with the condition are well-known concerns.^[1]

ESC 2020 Table 5: staff requirements for specialist ACHD centres

^[1]

ACC/AHA 2025: access, transition and the ACHD program

• ACC/AHA 2025 notes that a large proportion of patients with congenital heart disease are lost to care before they reach adulthood, only to return when they develop cardiovascular symptoms and complications.^[2]

Its first take-home message is that adults with congenital heart disease benefit from routine care at ACHD centres and in collaboration with ACHD cardiologists.^[2]

Care access, transition and the ACHD program

^[2]

More complex diagnoses should prompt collaboration with, or referral to, multidisciplinary specialised ACHD care.^[2]

• ACC/AHA 2025 lists possible indications for program referral; the list includes assessment of worsening AP class, advanced CT or CMR imaging beyond local expertise, worsening heart failure, transplant evaluation, catheterisation, electrophysiology, ACHD cardiothoracic surgery, pregnancy, and noncardiac surgery in AP classes IC–D, IIA–D and IIIA–D needing ACHD cardiac anaesthesia.^[2]
• Its Table 7 lists the program components: ACHD cardiologists, congenital cardiac surgeons, advanced practice providers, nurses, advanced imagers, a cardiac intensivist, advanced heart failure and transplant, interventional, electrophysiology and anaesthesia specialists, and maternal-fetal medicine, hepatology, pulmonary hypertension, cardiovascular genetics, palliative medicine, social work and psychology.^[2]

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Assessment: history, imaging, exercise testing and biomarkers

ESC 2020 reports exercise intolerance and palpitations as the most frequent symptoms in adults with congenital heart disease.^[1] Self-estimated physical capacity corresponds poorly with objective exercise capacity.^[1] ACC/AHA 2025 makes the same point: these patients often underreport functional limitations and overestimate their physical capabilities.^[2] In symptomatic patients, ESC 2020 asks the clinician to keep alternative causes in mind, such as anaemia, depression, weight gain and deconditioning.^[1] An ECG and pulse oximetry are routinely carried out alongside the clinical examination.^[1]

Imaging

Echocardiography remains the first-line imaging modality.^[1] ESC 2020 finds it superior to CMR for estimating pressure gradients and PA pressure and for detecting small, highly mobile structures such as vegetations.^[1] CMR is ideal for ventricular volumes, EF, valvular regurgitation, pulmonary and systemic flow and myocardial fibrosis.^[1] ESC 2020 calls CMR the gold-standard imaging method for quantification of volumes.^[1] CMR may be an alternative when echocardiography is of insufficient quality, or a second method when echocardiographic measurements are borderline or ambiguous.^[1]

• Gadolinium should be avoided with a glomerular filtration rate below 30 mL/min/1.73 m2 to mitigate the low risk of nephrogenic systemic fibrosis, so ESC 2020 recommends checking creatinine before CMR.^[1]
• Adults with congenital heart disease and conventional pacemakers or defibrillators can undergo CMR within guidelines where local support is available.^[1]
• Cardiac CT has high spatial resolution and rapid acquisition and is particularly relevant for the great vessels, coronary arteries, collateral arteries and parenchymal lung disease; it can assess ventricular size and function, but with inferior temporal resolution compared with CMR and typically with an increased radiation dose, so it is not used serially for that.^[1]
• ESC 2020 says advanced imaging is usually best reserved for visits to the specialist centre rather than repeated.^[1]
• CMR indications in ESC 2020 Table 6 include RV volumes and EF (subpulmonary, systemic and single ventricle), RVOT obstruction and RV–PA conduits, pulmonary regurgitation, the PAs and aorta, systemic and pulmonary veins, collaterals, coronary anomalies, stress perfusion, masses, myocardial mass, fibrosis and scar, Qp:Qs and the distribution of pulmonary flow; CMR detail is in Cardiac MRI: LGE patterns and clinical questions.^[1]

Exercise testing and catheterisation

• ESC 2020 describes CPET as covering peak oxygen consumption, VE/VCO2 slope, chronotropic and blood pressure response, and exercise-induced arrhythmia and desaturation, with endpoints that correlate well with morbidity and mortality.^[1]

It concludes that serial exercise testing should be part of long-term follow-up protocols.^[1]

Exercise testing in ACHD

^[2]

Cardiac catheterisation is mainly reserved for specific anatomical and physiological questions, or for intervention.^[1] In shunt lesions with Doppler signs of pulmonary hypertension, catheterisation including vasoreactivity testing remains essential to the decision on shunt closure.^[1]

• Before surgery, ESC 2020 advises coronary imaging by CT or invasive angiography in men over 40, postmenopausal women and patients with signs of, or risk factors for, coronary artery disease.^[1]

Natriuretic peptides

BNP and NT-proBNP are the best-studied neurohormones in ACHD and carry important prognostic information.^[1] They are less useful for diagnosing heart failure across lesions because cut-offs vary with the underlying defect and repair.^[1] They are most useful with a biventricular circulation and least useful with a Fontan circulation.^[1] They may also rise in cyanotic heart disease simply through hypoxia-induced secretion.^[1]

Radiation

ACC/AHA 2025 calls low-dose ionising radiation a known carcinogen and asks that, whenever clinically appropriate, studies without ionising radiation are chosen.^[2]

Ionising radiation

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Heart failure, transplantation and mechanical support

ESC 2020 puts heart failure in 20–50% of the ACHD population and names it a main cause of death.^[1]

• Any haemodynamic abnormality that could cause heart failure and can be addressed by intervention or surgery, arrhythmia included, must be excluded and, if possible, treated first.^[1]

• ESC 2020 warns that extrapolating results of heart failure studies to ACHD is inappropriate, particularly with a systemic RV, a failing subpulmonary ventricle or single-ventricle physiology, because the pathophysiology often differs from acquired heart disease.^[1]

• In a biventricular circulation, an impaired systemic LV is generally treated with conventional heart failure therapy, which is also applied in symptomatic patients with a failing systemic RV.^[1]

• Treatment of symptomatic patients with a failing single ventricle in a Fontan circulation, or with a persistent right-to-left shunt, should always be started carefully, taking the labile balance of ventricular preload and systemic afterload into account.^[1]

• ACC/AHA 2025 notes that the randomised trials showing benefit from GDMT, CRT and ICDs excluded ACHD, and that the biventricular circulation with a systemic LV is the setting where extrapolation from the heart failure literature is most appropriate.^[2]

• It adds, as an example, that some patients with ACHD may be predisposed to bradyarrhythmia or endocarditis, which could counterbalance the benefits of beta blockers or device therapy respectively; therefore both the strength of recommendation and the level of evidence for GDMT and device therapy in patients with ACHD and heart failure are lower than for patients without ACHD, in whom large randomised trials show benefits.^[2]

Heart failure, transplant and MCS rows

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ACC/AHA 2025 names heart failure admissions, systemic ventricular dysfunction and protein-losing enteropathy as appropriate triggers for evaluation for advanced therapies, transplantation included.^[2] Despite high early post-transplant mortality, patients with ACHD have equal or even superior long-term survival, particularly at high-volume centres with ACHD accreditation.^[2]

• ESC 2020 recommends in its text timely transplant evaluation by ACHD heart failure specialists in a transplant centre with ACHD expertise, and transfer to an expert centre in acute heart failure.^[1]

It adds that advance care planning, eventually including palliative care, should be offered to all patients with advanced heart failure.^[1]

• The ESC 2026 heart failure guideline refers management of these patients to the 2020 ESC ACHD guideline; general referral triggers are in Advanced HF: referral triggers, inotropes, transplant and LVAD.^[16]

Arrhythmias and sudden cardiac death

• ESC 2020 says that, among the atrial tachycardias of congenital heart disease, the most frequently encountered is late intra-atrial re-entrant tachycardia (IART), in particular cavotricuspid isthmus-dependent atrial flutter.^[1]

Atrial rates of 150 to 250 beats per minute may lead to rapid AV conduction, haemodynamic compromise and SCD.^[1] Monomorphic VT depends on the malformation and type of repair, with the critical isthmus typically bordered by surgical scars and patch material.^[1]

• Maintenance of sinus rhythm is the aim in all patients, and catheter ablation is recommended as first-line therapy over long-term drugs for amenable, circumscribed substrates, because antiarrhythmic drugs often have negative inotropic and/or dromotropic effects (ESC 2020 text).^[1]
• Class IC drugs may slow IART without blocking AV conduction, allowing 1:1 conduction with worsening haemodynamics.^[1]
• If IART or AF is tolerated and has lasted ≥48 h, ESC 2020 says thrombus must be ruled out by TOE and/or anticoagulation given for more than 3 weeks, with rate control using a beta blocker or calcium channel blocker (in patients with normal systemic ventricular function and no pre-excitation), before cardioversion.^[1]
• ACC/AHA 2025 states that rhythm control is typically preferred over rate control for atrial arrhythmias in complex patients such as those with a systemic right ventricle or Fontan circulation.^[2]

SCD related to ventricular arrhythmia accounts for 7–26% of all deaths in adults with congenital heart disease.^[1] Its incidence across the whole population is relatively low (below 0.1% per year), but some defects carry higher risk.^[1] ESC 2020 says ICD implantation for secondary prevention, and for primary prevention with biventricular physiology and a systemic LV, follows standard criteria.^[1] The benefit of primary-prevention ICDs for single or systemic right ventricles is less well established.^[1]

• A subcutaneous ICD may be an alternative with limited venous access or an intracardiac shunt, but not all patients are eligible because of the risk of inappropriate sensing and the lack of antitachycardia and antibradycardia pacing.^[1]

ESC rows on arrhythmia and anticoagulation in adults with congenital heart disease

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• The two bodies set different conditions and classes for an ICD in heart failure with a systemic left ventricle. ACC/AHA 2025: in patients with ACHD and heart failure symptoms who have systemic LV dysfunction and biventricular circulation, who otherwise meet accepted criteria, a primary-prevention ICD is reasonable to prevent sudden cardiac death (COR 2a, LOE C-LD). ESC 2022: in adults with CHD with biventricular physiology and a left systemic ventricle presenting with symptomatic heart failure (NYHA II/III) and EF ≤35% despite ≥3 months of OMT, ICD implantation is indicated (Class I, Level C).^[2][3]

ACC/AHA/HRS rows on arrhythmia and anticoagulation in adults with congenital heart disease

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For Fontan anticoagulation, the tables above hold ESC and ACC/AHA rows with different conditions.^[1][5][2]

• ESC 2020, in its recommendations for intervention after Fontan operation, says anticoagulation is indicated in the presence, or with a history, of atrial thrombus, atrial arrhythmias or thromboembolic events (Class I, Level C).^[1]

• The newer ESC 2024 AF row says oral anticoagulation should be considered in all adult congenital heart disease patients with AF/AFL and intracardiac repair, cyanosis, Fontan palliation or systemic right ventricle, to prevent ischaemic stroke and thromboembolism, regardless of other thromboembolic risk factors (Class IIa, Level C).^[5]

• ACC/AHA 2025 recommends anticoagulation in adults with Fontan circulation and a known or suspected thrombus, atriopulmonary Fontan, a history of thromboembolism or a history of sustained atrial flutter or fibrillation, to reduce the likelihood of thromboembolic disease (COR 1, LOE B-NR).^[2]

• So with atrial thrombus or past thromboembolism after Fontan, the ESC 2020 row (Class I) and the ACC/AHA 2025 row (COR 1) agree. With AF or flutter after Fontan, the ESC 2020 row (Class I; any atrial arrhythmia, present or past) and the ESC 2024 row (Class IIa; AF/AFL with Fontan palliation) differ in class, while the ACC/AHA 2025 row recommends anticoagulation with a history of sustained atrial flutter or fibrillation (COR 1).^[1][2][5]

• On anticoagulation, the ESC 2020 text said that, because their validity in congenital heart disease is uncertain, CHA2DS2-VASc and HAS-BLED should only be used in combination with individually assessed risks.^[1]

• ESC 2020 text (no class or level given) said anticoagulation therapy is recommended in paroxysmal and persistent AF/IART with moderate or severe CHD, but an individualised approach remains necessary.^[1]

• The newer ESC 2024 AF row above defines its group differently, overlapping in part: oral anticoagulation should be considered in all adult congenital heart disease patients with AF/AFL and intracardiac repair, cyanosis, Fontan palliation or systemic right ventricle, to prevent ischaemic stroke and thromboembolism, regardless of other thromboembolic risk factors (Class IIa, Level C).^[5]

For mild CHD, ESC 2020 applied the scores as per general recommendations.^[1] It also noted that NOACs seem similarly safe and effective in congenital heart disease, in the absence of mechanical valves or severe mitral stenosis.^[1] Device and ablation detail is in Ventricular tachycardia, Supraventricular tachycardia: AVNRT and AVRT and Bradycardia and pacing indications.

Pulmonary hypertension in congenital heart disease

• ESC 2020 calls pulmonary hypertension an important prognostic factor in congenital heart disease, needing particular attention in pregnancy and before reparative cardiac or other major surgery.^[1]
• Its clinical subtypes of PAH-CHD are PAH with congenital systemic-to-pulmonary shunts, Eisenmenger syndrome, repaired defects, and PAH that appears coincidental with CHD, frequently with small defects.^[1]

In complex CHD, PAH may be restricted to segments of the pulmonary vascular bed (segmental PAH).^[1] In a Fontan circulation, a rise in PA pressure is more often post-capillary, from raised ventricular filling pressure and/or AV valve regurgitation.^[1]

• Definitions have moved: ESC 2020 required a mean PAP above 20 mmHg with PVR ≥3 WU for pre-capillary PH (history, dated); the ESC/ERS 2022 guideline says PAH may be diagnosed with mPAP >20 mmHg and PVR >2 WU.^[1][10]
• ACC/AHA 2025 Table 5 defines pulmonary arterial hypertension as mean PA pressure by right heart catheterisation ≥20 mm Hg, PCWP ≤15 mm Hg and PVR ≥2 Wood units.^[2]

ESC 2020 Table 8: where each type of pulmonary hypertension occurs in ACHD (its haemodynamic thresholds are dated by the 2022 definition)

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ESC/ERS 2022 rows used here from Recommendation Table 19 (pulmonary arterial hypertension associated with adult congenital heart disease) and Recommendation Table 25 (pulmonary hypertension centres)

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First-line PAH-directed therapy in Eisenmenger syndrome as ACC/AHA 2025 Table 43 sets out the two guidelines (all five rows)

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ACC/AHA 2025 rows used here on PAH therapy in Eisenmenger syndrome

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• These newer ACC/AHA 2025 rows are limited to Eisenmenger syndrome with LV ejection fraction >40%. For specific drug classes, Table 43 above sets the ACC/AHA 2025 classes beside the ESC/ERS 2022 ones.^[2]

• For adults with Eisenmenger syndrome and LV ejection fraction >40% who remain symptomatic or have worsening exercise capacity on a single PAH therapy, ACC/AHA 2025 recommends dual combination therapy with an endothelin receptor antagonist and a phosphodiesterase-5 inhibitor to improve symptoms, hemodynamics and overall survival (COR 1, LOE B-NR); the ESC/ERS 2022 row for adult CHD including Eisenmenger syndrome says sequential combination therapy should be considered if patients do not meet treatment goals (Class IIa, Level C).^[2][10]

• Shunt closure by PVR is covered in Atrial septal defect and PFO: closure indications, and the PH groups and drug classes in Pulmonary hypertension: five groups and PAH-specific therapy.

Cyanosis: a multisystem disorder

Cyanosis is caused by right-to-left shunting through an anatomical communication between the systemic and pulmonary circulations at atrial, ventricular or arterial level.^[1] ESC 2020 states that cyanotic patients are complex and must be followed by an ACHD specialist.^[1] Chronic hypoxaemia triggers secondary erythrocytosis, which ACC/AHA 2025 calls a normal physiological response.^[2] The rise in red cell mass depletes bone marrow iron stores, prompting iron-deficiency anaemia.^[2]

• Adaptive mechanisms (ESC 2020): secondary erythrocytosis, a rightward shift of the oxyhaemoglobin dissociation curve, and an increase in cardiac output.^[1]
• Hyperviscosity symptoms include headache, faintness, dizziness, fatigue, tinnitus, blurred vision, paraesthesia of fingers, toes and lips, muscle pain and weakness; they are unlikely in an iron-replete patient with haematocrit below 65%.^[1]
• Coagulation is altered in both directions, raising the risk of bleeding (haemoptysis, epistaxis) and of thrombosis (TIA or stroke, pulmonary embolism).^[2]
• Haemoptysis is the most common major bleeding event; it is an external sign of intrapulmonary haemorrhage that does not reflect the extent of parenchymal bleeding.^[1]
• Fever with a new or different headache raises suspicion of a brain abscess.^[1]
• ACC/AHA 2025 says chronic cyanosis affects multiple organ systems and can manifest with kidney dysfunction, hypertrophic osteoarthropathy, myocardial dysfunction, neurologic dysfunction, and hypertensive emergency due to neuroendocrine tumours such as phaeochromocytomas and paragangliomas.^[2]

Management of cyanosis (ACC/AHA 2025 section 3.5)

^[2]

• ACC/AHA 2025 Figure 1 sets out management of hyperviscosity symptoms in cyanotic ACHD, and its footnote states that all patients should have at least annual screening for iron deficiency.^[2]
• ESC 2020 text limits therapeutic phlebotomy to moderate or severe hyperviscosity symptoms due to secondary erythrocytosis (haematocrit at least above 65%), in the absence of dehydration and iron deficiency.^[1]
• When phlebotomy is performed, ESC 2020 states that isovolumetric fluid replacement (750–1000 mL of isotonic saline while removing 400–500 mL of blood) should be undertaken.^[1]

ESC 2020 also says current data do not support routine anticoagulation or aspirin to prevent thromboembolism in cyanotic patients.^[1]

• Oxygen saturation must be obtained with pulse oximetry at rest for at least 5 min, and exercise capacity should be assessed regularly, preferably with a 6-minute walk test.^[1]
• Laboratory precaution: plasma volume is reduced, so the sodium citrate in coagulation tubes must be adjusted to haematocrit when it is above 55%.^[1]
• All cyanotic patients need lifelong follow-up every 6–12 months in a specialised ACHD centre, in close collaboration with the family physician.^[1]
• ESC 2020 Table 9 (risk reduction) lists exposures to avoid, including pregnancy in Eisenmenger syndrome and in cyanotic patients without PAH but with saturation below 90%, inappropriate phlebotomies, dehydration, transvenous PM/ICD leads, strenuous exercise, acute exposure to heat (sauna, hot tub or hot shower) or extreme cold, and oestrogen-containing contraception, and adds measures such as air filters on intravenous lines and influenza and pneumococcal vaccination.^[1]
• Acute exposure to high altitude (above 2500 m) should be avoided; gradual ascent up to 2500 m may be tolerated.^[1]

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Endocarditis and thromboembolism

ESC 2020 notes that the risk of infective endocarditis in ACHD is higher than in the general population, with marked variation between lesions.^[1] It asks for good oral and cutaneous hygiene and aseptic measures during healthcare and any invasive procedure in all patients.^[1] Piercings and tattoos are discouraged, and otherwise should be performed under optimal hygienic conditions.^[1]

Endocarditis prophylaxis and stroke prevention rows

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Antibiotic prophylaxis for oro-dental procedures in other cardiovascular disease is covered in Infective endocarditis: 2023 ESC diagnosis, antibiotics, surgery and prevention.^[11]

Pregnancy, contraception and genetics

Counselling and risk

ESC 2020 states that pre-pregnancy counselling should be provided to all women with congenital heart disease.^[1]

• Its text based risk estimation on the modified WHO classification; the ESC 2025 pregnancy guideline now uses its modified WHO 2.0 classification of maternal cardiovascular risk.^[1][13]

ACC/AHA 2025 names the modified WHO classification and the CARPREG 2 score as risk scores that may be used to estimate maternal cardiac risk.^[2] Cyanosis poses a significant fetal risk, with live birth unlikely (below 12%) if oxygen saturation is below 85% (ESC 2020).^[1] ACE inhibitors and angiotensin receptor blockers in particular should not be used in pregnancy.^[1]

ACC/AHA 2025 Table 10 (information adapted from the modified WHO classification)

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Reproductive health and pregnancy rows

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• On contraception, ESC 2020 says the combined oral contraceptive is highly effective (99.9%) but best avoided with pre-existing thrombotic risk, such as cyanosis, Fontan physiology, mechanical valves, prior thrombotic events or PAH.^[1]

Progesterone-only methods do not pose such a high thrombosis risk, and newer oral or intrauterine preparations have high efficacy (above 95%).^[1]

• Patients with a fragile physiology, such as a Fontan circulation, PH, cyanotic CHD or Eisenmenger syndrome, should have intrauterine devices inserted or removed in an environment with ACHD expertise; pregnancy management in general is covered in Cardiovascular disease in pregnancy.^[1]

Genetics and recurrence

ACC/AHA 2025 states that genetic and/or environmental causes can be identified in 20% to 30% of affected patients.^[2] ESC 2020 text says genetic counselling should at least be considered for every ACHD patient.^[1] Conotruncal anomalies affect the outflow tract and great arteries and have a high rate of genetic diagnoses, most commonly 22q11.2 deletion and trisomy 21.^[2] About 60% to 80% of people with 22q11.2 deletion syndrome have congenital heart disease, and 70% of that subset have a conotruncal defect.^[2]

Genetic screening rows

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Selected rows of ACC/AHA 2025 Table 9 (genetic syndromes and their associations with congenital heart disease)

^[2]

ESC 2020 puts the recurrence rate of CHD in offspring at 2–50%, higher when the mother is affected than the father.^[1] It recommends fetal echocardiography in affected couples at 19–22 weeks, possible as early as 15–16 weeks.^[1]

Recurrence rates by the sex of the affected parent (selected rows)

^[1]

• ESC 2020 Table 11 footnote: except for Marfan syndrome, the rates apply to patients with isolated cardiac lesions in whom known genetic or syndromic entities have been excluded.^[1]

Exercise, sport and rehabilitation

ACC/AHA 2025 says most patients can safely engage in regular, moderate physical activity and should be encouraged to do recreational exercise or appropriate activity.^[2] A few conditions, such as systemic ventricular systolic dysfunction, systemic ventricular outflow tract obstruction, hemodynamically significant arrhythmias or aortic dilation, warrant more cautious recommendations.^[2]

• ESC 2020 adds that, in general, physicians have been over-conservative in their advice, that symptoms do not exclude physical activity and that dynamic exercise is more suitable than static exercise.^[1]

Physical activity, sport and rehabilitation rows

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• The 2020 ESC sports cardiology guideline's key message: patients with congenital heart disease should be encouraged to exercise and should be given a personalised exercise prescription.^[20]
• The 2025 AHA/ACC scientific statement on competitive sports (ungraded considerations) groups congenital lesions by anatomy and physiology in its congenital table and says all competitive athletes with diagnosed congenital heart abnormalities should have a 12-lead ECG, echocardiography, oxygen saturation, BP, symptom assessment and the testing listed in that table assessed within 1 year of initial competitive sports participation, depending on lesion.^[19]

Surgery and procedures: who should be involved

ACC/AHA 2025 states that patients with ACHD have greater operative risk than those without ACHD for noncardiac surgery.^[2]

• When possible, patients with ACHD, especially those with anatomic class II or III and/or physiological stage C or D, should receive preoperative evaluation and surgery or other nonsurgical interventions at a centre with an ACHD program.^[2]
• ESC 2020 lists cyanosis, congestive heart failure, poor general health, younger age, PH, respiratory and nervous system operations, complex CHD and urgent procedures as factors raising perioperative risk.^[1]

In complex CHD such as Fontan, Eisenmenger syndrome or cyanosis, it says non-cardiac surgical and interventional procedures should be performed in an expert centre.^[1]

Noncardiac and cardiac procedure rows

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• ACC/AHA 2025 Table 8 (preoperative checklist for noncardiac surgery) lists cyanosis, complex congenital heart disease, frailty, heart failure, operations on the respiratory and nervous systems, pulmonary hypertension and urgent or emergency procedures as factors associated with increased perioperative risk.^[2]

• For cardiac surgery, ESC 2020 notes that risk cannot be calculated with conventional surgical risk scores and that outcome is proven to be superior when ACHD patients are operated on by a congenital cardiac surgeon.^[1]

• Its text sets the "strict recommendation" that all ACHD patients are operated on by a congenital cardiac surgeon in a multidisciplinary environment with expertise in ACHD, except for surgery of uncomplicated BAV, HTAD such as Marfan syndrome, and secundum-type ASD without anomalous pulmonary venous connection and/or absence of PVD.^[1]

• ACC/AHA 2025 says patients with anatomic or physiologically moderate or complex ACHD who undergo cardiac or noncardiac procedures should have an ACHD cardiologist involved; general perioperative assessment is covered in Preoperative cardiac assessment for non-cardiac surgery.^[2]

Mental health, ageing and advance care planning

• ACC/AHA 2025 reports that mental health conditions, including depression, anxiety and post-traumatic stress disorder, have been identified in up to 50% of adults with congenital heart disease.^[2]

Frailty and cognitive decline are noted in more than a third of those older than 40 years.^[2] ESC 2020 records that about 90% of patients with mild, 75% with moderate and 40% with complex defects reach the age of 60.^[1]

Mental health and neurocognitive assessment

^[2]

Most patients, whatever the complexity of their defect, wish to discuss life expectancy before facing life-threatening complications (ESC 2020).^[1] Unplanned admissions, ICD insertion or functional decline can trigger advance care planning conversations.^[1] ESC 2020 also notes that life, health and travel insurance and mortgages are often difficult to obtain.^[1]

Evidence, guidelines and regional differences

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The difference to explain at the viva: ESC 2020 says congenital heart disease can be classified as mild, moderate or severe (its Table 4).^[1] Its severe group also includes any CHD (repaired or unrepaired) associated with pulmonary vascular disease and any cyanotic CHD (unoperated or palliated).^[1] ACC/AHA 2025 assigns an anatomic complexity (I–III) and a physiological stage (A–D) and classifies by the highest relevant feature.^[2] It says the system aims to improve classification of disease severity given the potential for discordance between anatomic complexity and physiological stage.^[2]

• ESC 2020 says that in moderate and severe CHD complexity with documented arrhythmias, referral to a centre with a multidisciplinary team and expertise in ACHD patients and ACHD-related arrhythmia is indicated (Class I, Level C).^[1]
• It adds in its text (no class or level given) that in complex CHD (Fontan, Eisenmenger syndrome, cyanotic patients), non-cardiac surgical and interventional procedures should be performed in an expert centre.^[1]

ACC/AHA 2025 says ACHD patients benefit from routine care at ACHD centres and in collaboration with ACHD cardiologists.^[2]

• Its second take-home message (no class or level given) is that patients with anatomic or physiologically moderate or complex ACHD who undergo cardiac or noncardiac procedures are recommended to have an ACHD cardiologist involved in their care.^[2]

In Australia and New Zealand

No Australian or New Zealand guideline on adult congenital heart disease was found in the PubMed census for this topic (census 2026-10-23). The 2020 CSANZ position statement on people with a Fontan circulation (PMID 31735685) is held only as its PubMed abstract. That abstract describes the Fontan circulation as the circulatory state resulting from an operation in congenital heart disease where systemic venous return is directed to the lungs without an intervening active pumping chamber.^[21] The abstract also describes the statement as consensus agreement about management of the person with a Fontan circulation; its full text was not checked for this topic.^[21] In the absence of an Australian or New Zealand ACHD guideline in this census, this page quotes the ESC and ACC/AHA guidelines.

Guidelines checked for this topic

• Rows on this page come from the ESC 2020 adult congenital heart disease guideline and the ACC/AHA/HRS/ISACHD/SCAI 2025 adult congenital heart disease guideline, with ACHD rows from the ESC 2022 ventricular arrhythmia, ESC 2021 pacing, ESC 2024 AF, ESC/ERS 2022 pulmonary hypertension, ESC 2023 endocarditis, ESC 2025 pregnancy, ESC 2022 non-cardiac surgery, ESC 2026 cardiac rehabilitation and ESC 2026 heart failure guidelines.^{[1][2][3][4][5][10][11][13][14][15][16]}
• AHA/ACC rows come from the 2023 AF, 2018 bradycardia, 2017 ventricular arrhythmia, 2017 syncope, 2020 valvular heart disease, 2021 secondary stroke prevention (AHA/ASA) and 2022 aortic disease guidelines.^{[6][7][8][9][12][17][18]}

Narrative statements come from the ESC 2020 sports cardiology guideline and the 2025 AHA/ACC competitive sports scientific statement (JACC PMID 39976316; Circulation PMID 39973614).^[20][19] The 2025 ACC/AHA guideline is a full revision and replacement of the 2018 AHA/ACC ACHD guideline.^[2]

• Rows marked "History, dated", and rows with a note naming newer rows, have those newer rows from the same body beside them; for the other rows, the same-month and newer guidelines of the same body in the census below were checked and give no revising row (census 2026-10-23).

• The documents in this list are not used; each line gives the held status recorded in the census for this topic.

• The 2018 AHA/ACC adult congenital heart disease guideline is replaced by the 2025 ACC/AHA guideline quoted on this page, which calls itself a full revision and replacement.^[2]

• The PACES/HRS Expert Consensus Statement on the Recognition and Management of Arrhythmias in Adult Congenital Heart Disease: developed in partnership between the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS). Endorsed by the governing bodies of PACES, HRS, the American College of Cardiology (ACC), the American Heart Association (AHA), the European Heart Rhythm Association (EHRA), the Canadian Heart Rhythm Society (CHRS), and the International Society for Adult Congenital Heart Disease (ISACHD) (PMID 24814377) is not held as text in the evidence caches, so it was not checked for this topic.

• The PACES/HRS expert consensus statement on the use of catheter ablation in children and patients with congenital heart disease: Developed in partnership with the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS). Endorsed by the governing bodies of PACES, HRS, the American Academy of Pediatrics (AAP), the American Heart Association (AHA), and the Association for European Pediatric and Congenital Cardiology (AEPC) (PMID 26899545) is not held as text in the evidence caches, so it was not checked for this topic.

• The NHFA/CSANZ Australian Clinical Guidelines for the Diagnosis and Management of Atrial Fibrillation 2018 (PMID 30077228) are not held as text in their version of record, so they were not checked for this topic.

• The 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease (PMID 30121239) is not held as text in the evidence caches, so it was not checked for this topic.

• The 2018 ESC/EACTS Guidelines on myocardial revascularization (PMID 30165437) are not held as text in the evidence caches, so they were not checked for this topic.

• The 2019 ESC Guidelines for the diagnosis and management of chronic coronary syndromes (PMID 31504439) are not held as text in the evidence caches, so they were not checked for this topic.

• The Recommendations for participation in competitive sport in adolescent and adult athletes with Congenital Heart Disease (CHD): position statement of the Sports Cardiology & Exercise Section of the European Association of Preventive Cardiology (EAPC), the European Society of Cardiology (ESC) Working Group on Adult Congenital Heart Disease and the Sports Cardiology, Physical Activity and Prevention Working Group of the Association for European Paediatric and Congenital Cardiology (AEPC) (PMID 32845299) is not held as text in the evidence caches, so it was not checked for this topic.

• The 2020 ESC Guidelines for the management of acute coronary syndromes in patients presenting without persistent ST-segment elevation (PMID 32860058) are not held as text in the evidence caches, so they were not checked for this topic.

• The 2020 ESC Guidelines for the diagnosis and management of atrial fibrillation (PMID 32860505) are not held as text in the evidence caches, so they were not checked for this topic.

• The 2021 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure (PMID 34447992) are not held as text in the evidence caches, so they were not checked for this topic.

• The 2021 ESC/EACTS Guidelines for the management of valvular heart disease (PMID 34453165) are not held as text in the evidence caches, so they were not checked for this topic.

• The 2021 ACC/AHA/SCAI Guideline for Coronary Artery Revascularization (PMID 34882435) is not held as text in the evidence caches, so it was not checked for this topic.

• The 2022 ESC Guidelines on cardio-oncology (PMID 36017568) are not held as text in the evidence caches, so they were not checked for this topic.

• The 2023 Focused Update of the 2021 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure (PMID 37622666) are not held as text in the evidence caches, so they were not checked for this topic.

• Only the PubMed abstract of the 2023 Australian guideline for assessing and managing cardiovascular disease risk (PMID 38623719) is held; its full text is not held and was not checked for this topic.

• Only the PubMed abstract of the A summary of the 2023 Society of Obstetric Medicine of Australia and New Zealand (SOMANZ) hypertension in pregnancy guideline (PMID 38763516) is held; its full text is not held and was not checked for this topic.

• Only the PubMed abstract of the 2024 ACC/AHA/AACVPR/APMA/ABC/SCAI/SVM/SVN/SVS/SIR/VESS Guideline for the Management of Lower Extremity Peripheral Artery Disease (PMID 38743805) is held; its full text is not held and was not checked for this topic.

• The 2024 CSANZ Position Statement on Indications, Assessment and Monitoring of Structural and Valvular Heart Disease With Transthoracic Echocardiography in Adults (PMID 38749800) is not held as text in its version of record, so it was not checked for this topic; only its PubMed abstract is quotable.

• Only the PubMed abstract of the Pulmonary hypertension associated with left heart disease (PMID 39209478) is held; its full text is not held and was not checked for this topic.

• The NHFA/CSANZ Comprehensive Australian Clinical Guideline for Diagnosing and Managing Acute Coronary Syndromes 2025 (PMID 40180468) is not held as text in its version of record, so it was not checked for this topic.

• The JCS/JPCPHS 2025 Guideline on Pulmonary Hypertension and Pulmonary Embolism/Deep Vein Thrombosis (PMID 42386545) is not held as text in the evidence caches, so it was not checked for this topic.

• The HRS/ISACHD/PACES scientific statement on provider and institutional best practices for comprehensive adult congenital heart disease electrophysiology programs (PMID 41679448) is not held as text in the evidence caches, so it was not checked for this topic.

• The 2026 ESC Guidelines for the management of cardiovascular disease and chronic kidney disease, in collaboration with the European Renal Association (ERA) (PMID 42661426) are not held as text in their version of record, so they were not checked for this topic.

• Only the PubMed abstract of the European Respiratory Society clinical practice guidelines update for the treatment of pulmonary arterial hypertension (PMID 42705705) is held; its full text is not held and was not checked for this topic.

• Only the PubMed abstract of the 2026 AHA/ACC/ACS/ASNC/HRS/SCA/SCCT/SCMR/SVM Guideline for Perioperative Cardiovascular Management for Noncardiac Surgery (PMID 42804570) is held; its full text is not held and was not checked for this topic.

• The held guidelines below were checked, and no row from them is used here; each line gives the reason.

• ACC/AHA/HRS 2015 SVT (PMID 26399663): its ACHD rows cover acute and ongoing treatment of SVT, atrial tachycardia and flutter in ACHD, which this page leaves to the arrhythmia pages; the ACHD arrhythmia rows given here are from the guidelines listed above.

• NHFA/CSANZ 2018 heart failure summary in Med J Aust (PMID 30067937): no congenital row; the Heart Lung Circ guideline (PMID 30077227) was checked instead.

• NHFA/CSANZ 2018 heart failure (PMID 30077227): congenital defects appear only in its list of causes of heart failure; no congenital row.

• Fourth Universal Definition of MI (2018, PMID 30571511; superseded by the Fifth): no congenital row.

• 2019 ESC/EAS dyslipidaemias (PMID 31504418): no congenital row.

• ESC 2019 SVT (PMID 31504425): its rows on therapy of SVT in adults with congenital heart disease are SVT-management detail left to the SVT page; the ESC 2020 ACHD ablation rows given above are newer.

• ESC 2019 pulmonary embolism (PMID 31504429): shunting through a PFO or ASD appears only as narrative; no congenital row.

• ESC 2021 prevention (PMID 34458905): no congenital row.

• AHA/ACC 2021 chest pain (PMID 34709879): no congenital row.

• AHA/ACC/HFSA 2022 heart failure (PMID 35363499): congenital heart disease appears in narrative and tables (heart failure risk, imaging); no congenital row.

• AHA/ACC 2023 chronic coronary disease (PMID 37471501): no congenital row.

• ESC 2023 acute coronary syndromes (PMID 37622654): no congenital row.

• ESC 2023 cardiomyopathies (PMID 37622657): congenital heart disease appears only in the cardiomyopathy definition, as an exclusion; no row.

• ESC 2023 diabetes (PMID 37622663): no congenital row.

• 2024 Australia-New Zealand cardiac amyloidosis consensus (PMID 38570258): no congenital content.

• AHA/ACC 2024 hypertrophic cardiomyopathy (PMID 38718139): no congenital row.

• Definition, classification and diagnosis of pulmonary hypertension (2024, PMID 39209475): no graded congenital row.

• Treatment algorithm for pulmonary arterial hypertension (2024, PMID 39209476): no graded congenital row.

• ESC 2024 chronic coronary syndromes (PMID 39210710): no congenital row.

• ESC 2024 hypertension (PMID 39210715): bicuspid aortic valve and coarctation appear as narrative only; no row.

• ESC 2024 peripheral arterial and aortic diseases (PMID 39210722): its coarctation rows (Recommendation Table 69) are lesion-specific and outside this general page.

• AHA/ACC 2024 perioperative (PMID 39316661): its adult congenital heart disease recommendation table (section 6.3.3) is not printed in the held text, so no row is quoted; the ESC 2022 and ACC/AHA 2025 procedure rows are given above. A 2026 AHA/ACC perioperative guideline (PMID 42804570) is also in the census; only its PubMed abstract is held (see its line in the list above).

• ACC/AHA 2025 acute coronary syndromes (PMID 40014670): no congenital row.

• AHA/ACC 2025 blood pressure (PMID 40811497): coarctation appears only as a cause of secondary hypertension; no row.

• 2025 ESC/EAS dyslipidaemias focused update (PMID 40878289): no congenital row.

• ESC/EACTS 2025 valvular heart disease (PMID 40878295): states that it does not deal in detail with all scenarios of congenital disease, which are covered in separate guidelines; no congenital row.

• ESC 2025 myocarditis and pericarditis (PMID 40878297): no congenital row.

• NHFA/CSANZ 2025 ACS guideline summary in Med J Aust (PMID 41693087): no congenital row.

• AHA/ACC 2026 pulmonary embolism (PMID 41712677): no congenital row.

• ACC/AHA 2026 dyslipidemia (PMID 41824552): no congenital row.

• AHA/ACC/ADA/ASN 2026 cardiovascular-kidney-metabolic guideline (PMID 42263157): congenital heart disease appears only as a cross-reference to other guidelines; no row.

• Fifth Universal Definition of MI (2026, PMID 42661425): congenital heart disease appears only among mechanisms of chronic myocardial injury; no row.

Exam pearls

• AP classification: classify by the "highest" feature; Fontan circulation, a systemic RV or truncus arteriosus can never be stage A (ACC/AHA 2025).^[2]
• Stage D includes heart failure hospitalisation in the past 12 months, endocarditis in the prior year, Eisenmenger syndrome, NYHA III–IV, recurrent arrhythmias that are hemodynamically significant and/or refractory to treatment, baseline saturation ≤85% and intermediate- or high-risk PAH.^[2]
• Hypoxemia means resting saturation ≤92% (severe ≤85%); a shunt is hemodynamically significant with chamber enlargement distal to it and/or sustained Qp:Qs ≥1.5 (ACC/AHA 2025 Table 5).^[2]
• Cyanosis: screen for and treat iron deficiency every year to improve exercise capacity and functional status; no prophylactic phlebotomy; phlebotomy may be considered with haematocrit above 65% and hyperviscosity symptoms that persist despite adequate rehydration and treatment of iron deficiency (ACC/AHA 2025).^[2]
• Use an air filter in intravenous lines in cyanotic patients to prevent air embolism (ESC 2020 Table 9, risk reduction strategies in cyanotic congenital heart disease).^[1]
• Live birth is unlikely (below 12%) if maternal saturation is below 85% (ESC 2020).^[1]
• Recurrence is higher when the mother is affected, for example AVSD 11.5–14% versus 1–4.5% (ESC 2020 Table 11).^[1]

References21ShowHide

1. [1]Baumgartner H, et al. 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J, 2021.PMID 32860028
2. [2]Gurvitz M, et al. 2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation, 2026.PMID 41411375
3. [3]Zeppenfeld K, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J, 2022.PMID 36017572
4. [4]Glikson M, et al. 2021 ESC Guidelines on cardiac pacing and cardiac resynchronization therapy. Eur Heart J, 2021.PMID 34455430
5. [5]Van Gelder IC, et al. 2024 ESC Guidelines for the management of atrial fibrillation developed in collaboration with the European Association for Cardio-Thoracic Surgery (EACTS). Eur Heart J, 2024.PMID 39210723
6. [6]Joglar JA, et al. 2023 ACC/AHA/ACCP/HRS Guideline for the Diagnosis and Management of Atrial Fibrillation: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation, 2024.PMID 38033089
7. [7]Kusumoto FM, et al. 2018 ACC/AHA/HRS Guideline on the Evaluation and Management of Patients With Bradycardia and Cardiac Conduction Delay: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. J Am Coll Cardiol, 2019.PMID 30412709
8. [8]Al-Khatib SM, et al. 2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Circulation, 2018.PMID 29084731
9. [9]Shen WK, et al. 2017 ACC/AHA/HRS Guideline for the Evaluation and Management of Patients With Syncope: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Circulation, 2017.PMID 28280231
10. [10]Humbert M, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J, 2023.PMID 36028254
11. [11]Delgado V, et al. 2023 ESC Guidelines for the management of endocarditis. Eur Heart J, 2023.PMID 37622656
12. [12]Otto CM, et al. 2020 ACC/AHA Guideline for the Management of Patients With Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol, 2021.PMID 33342586
13. [13]De Backer J, et al. 2025 ESC Guidelines for the management of cardiovascular disease and pregnancy. Eur Heart J, 2025.PMID 40878294
14. [14]Halvorsen S, et al. 2022 ESC Guidelines on cardiovascular assessment and management of patients undergoing non-cardiac surgery. Eur Heart J, 2022.PMID 36017553
15. [15]Bäck M, et al. 2026 ESC Guidelines on cardiac rehabilitation. Eur Heart J, 2026.PMID 42661418
16. [16]Køber L, et al. 2026 ESC Guidelines for the management of heart failure. Eur Heart J, 2026.PMID 42661420
17. [17]Kleindorfer DO, et al. 2021 Guideline for the Prevention of Stroke in Patients With Stroke and Transient Ischemic Attack: A Guideline From the American Heart Association/American Stroke Association. Stroke, 2021.PMID 34024117
18. [18]Isselbacher EM, et al. 2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines. Circulation, 2022.PMID 36322642
19. [19]Kim JH, et al. Clinical Considerations for Competitive Sports Participation for Athletes With Cardiovascular Abnormalities: A Scientific Statement From the American Heart Association and American College of Cardiology. J Am Coll Cardiol, 2025.PMID 39976316
20. [20]Pelliccia A, et al. 2020 ESC Guidelines on sports cardiology and exercise in patients with cardiovascular disease. Eur Heart J, 2021.PMID 32860412
21. [21]Zentner D, et al. Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement. Heart Lung Circ, 2020.PMID 31735685