2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the Management of Adults With Congenital Heart Disease

Authors, Journal, Affiliations, Type, DOI

• Chair: Michelle Gurvitz, MD, MS; Co–Vice Chairs: Eric V. Krieger, MD; Stephanie Fuller, MD, MS
• Writing committee: 26 members including ACHD cardiologists, congenital cardiac surgeons, ACHD interventional cardiologists, electrophysiologists, HF cardiologists, advanced practice nurses, and a patient advocate
• Collaborating societies: HRS, ISACHD, SCAI
• Journal: Circulation, 2026;153:e115–e251
• Type: Full-revision Clinical Practice Guideline (replaces 2018 AHA/ACC ACHD Guideline)
• DOI: 10.1161/CIR.0000000000001402
• Literature search: 2017–July 2025 (MEDLINE, EMBASE, Cochrane Library, CINAHL)

Overview

This guideline is a comprehensive full revision of the 2018 AHA/ACC guideline for adults with congenital heart disease (ACHD). It covers all aspects of ACHD management, including a refined anatomic–physiologic (AP) classification system, general principles for care delivery, and specific recommendations across all major congenital lesion groups. Key practice-changing updates include new pulmonary valve replacement criteria in repaired tetralogy of Fallot (shifting from RVEDVI to RV end-systolic volume index), upgrading Eisenmenger PAH-directed therapy to COR 1, mandatory annual liver surveillance in Fontan circulation, and transcatheter closure as the preferred approach for secundum ASD. New stand-alone sections cover cyanosis management, ACHD heart failure/transplantation, Fontan liver disease, and vascular rings.

Keywords

Adult congenital heart disease, aortic coarctation, aortic valve stenosis, cardiac catheterization, cardiac surgical procedures, congenital heart disease, cor triatriatum, coronary vessel anomalies, Ebstein anomaly, tetralogy of Fallot, transposition of great vessels, ventricular outflow obstruction, pulmonary valve stenosis

Key Takeaways

ACHD Anatomic–Physiologic (AP) Classification System

• The AP classification assigns anatomic complexity (I: Simple, II: Moderate, III: Great Complexity) and physiological stage (A–D), together defining ACHD AP class
• Stage D now includes: HF hospitalization in past 12 months, endocarditis in prior year, Eisenmenger syndrome, NYHA III/IV, severe hypoxemia (SpO₂ ≤85%), and intermediate/high-risk PAH
• Stage C now explicitly includes BNP/NT-proBNP ≥2× ULN as a criterion
• Patients with Fontan, systemic RV, or truncus arteriosus cannot be in physiological stage A
• Higher AP classification correlates with greater short- and long-term mortality; the physiological stage adds prognostic value beyond anatomy alone
• concepts/ACHD-AP-Classification

Care Delivery and General Principles

• ACHD AP IB–D, IIA–D, IIIA–D: Must be evaluated and managed by or in collaboration with an ACHD cardiologist (COR 1, B-NR)
• ACHD AP IA: At least 1 evaluation by an ACHD cardiologist to develop a plan of care (COR 1, B-NR) — new recommendation
• Noncardiac surgery: ACHD IC–D, IIB–D, IIIA–D require ACHD cardiologist involvement pre- and post-procedure (COR 1); congenital-trained anesthesiologist required for ACHD IC–D, IIC–D, IIIA–D (COR 1, C-LD)
• Transition of care: Structured patient-centered transition education (COR 1, A); transfer policies pediatric→ACHD (COR 1, B-NR)
• Genetic screening: Referral for genetic evaluation in adults with ACHD and family history of CHD or features of genetic syndrome (COR 2a)
• Pregnancy: Routine cesarean delivery has no benefit and may cause harm in most ACHD patients without obstetric indication or high-risk cardiac condition (COR 3: No Benefit) — new recommendation
• Sports: Competitive sports participation is reasonable after comprehensive ACHD specialist evaluation (COR 2a) — new recommendation

Management of Cyanosis (New Section)

• Annual iron deficiency screening and treatment (COR 1, B-NR)
• New or worsening neurologic deficits → urgent brain imaging to exclude cerebral abscess or stroke (COR 1, B-NR)
• Hyperviscosity symptoms → oral/IV rehydration first; phlebotomy only if hematocrit >65% and symptoms persist despite rehydration and iron correction
• Prophylactic phlebotomy: COR 3: No Benefit — exacerbates iron deficiency and increases stroke risk
• Pheochromocytoma/paraganglioma screening if recurrent unexplained hypertension or tachycardia (COR 2a)

Heart Failure in ACHD (New Section)

• GDMT for HF (ACEi/ARB/ARNI + beta-blocker + MRA + SGLT2i) is reasonable in ACHD with systemic LV dysfunction and biventricular circulation (COR 2a, C-LD)
• CRT reasonable for those meeting standard CRT criteria with systemic LV and biventricular circulation (COR 2a, B-NR); benefit noted in 65–77% of ACHD with systemic LV morphology
• Primary-prevention ICD reasonable for ACHD with systemic LV dysfunction meeting conventional criteria (COR 2a, C-LD)
• Advanced HF evaluation by ACHD + HF specialists recommended for advanced therapies (COR 1, B-NR)
• Durable MCS reasonable as bridge to transplantation in refractory ACHD HF (COR 2a, B-NR)

Shunt Lesions

Atrial Septal Defect (ASD):

• Secundum ASD: Transcatheter closure preferred over surgical repair to reduce length of stay and recovery (COR 1) — upgraded from COR 2b
• ASD + PAH → risk assessment and PAH specialist consultation (COR 1)
• ASD + paradoxical embolism → closure to prevent recurrence (COR 1)
• ASD + significant shunt (Qp:Qs ≥1.5) + PAH (PVR 5–8 WU): closure can be beneficial if PVR <5 WU achievable with targeted PAH therapy (COR 2a)

Ventricular Septal Defect (VSD):

• Assess for PAH in all unrepaired VSD (COR 1)
• VSD + PAH → managed by ACHD + PH specialists (COR 1)
• VSD with Qp:Qs <1.5 and no other indication: closure should not be performed (COR 3: No Benefit)

Atrioventricular Septal Defect (AVSD):

• Assess for PAH in all unrepaired AVSD (COR 1)
• AVSD + LVOT obstruction with symptoms or LVEF <50% → surgical repair (COR 1)

Left-Sided Lesions

Bicuspid Aortic Valve (BAV):

• Aortic diameter >4 cm at sinuses or ascending aorta: lifelong surveillance for thoracic aortic disease (COR 1) — new
• Valve replacement (surgical or transcatheter) should be reviewed by heart valve + ACHD expert team using shared decision-making (COR 1) — new
• First-degree relatives: 1-time echocardiographic screening (7% BAV prevalence, 30% aortic disease if relatives also have BAV)

Coarctation of the Aorta (CoA):

• Unrepaired CoA → cross-sectional imaging for severity and anatomy (COR 1)
• Repaired CoA → periodic cross-sectional imaging for re-CoA, aneurysm, pseudoaneurysm (COR 1)
• Exertional symptoms → evaluate for coronary artery disease (COR 2a) — new
• CMR preferred; CT angiography if prior CoA stent

Right-Sided Lesions

Ebstein Anomaly:

• Symptomatic arrhythmia or asymptomatic ventricular preexcitation → EP study (COR 1; upgraded from COR 2a)

Valvular Pulmonary Stenosis:

• Asymptomatic moderate/severe PS + ≥moderate TR or any RV dysfunction → pulmonary valve intervention (COR 1) — new
• Post-treatment pulmonary regurgitation: CMR recommended to quantify (COR 1) — new

Tetralogy of Fallot (repaired):

• Echocardiography for routine hemodynamic assessment (COR 1)
• CMR gold standard for quantifying PR, ventricular function, PA anatomy, and fibrosis (COR 1)
• PVR indications (asymptomatic): RV end-systolic volume index >80 mL/m² OR RV end-diastolic volume ≥2× LVEDV OR RVEF ≤46% OR LVEF ≤50% OR progressive decline in exercise capacity — at least 2 of these 5 criteria (COR 2a); shift away from prior emphasis on RVEDVI
• Symptomatic + moderate/greater PR → PVR (surgical or transcatheter) recommended (COR 1)
• ICD for high-risk SCD patients (PREVENTION-ACHD, Brompton, PACES risk scores) (COR 2a)
• Catheter ablation adjunctive to ICD for recurrent monomorphic VT (COR 2a) — new
• concepts/Tetralogy-of-Fallot

Complex Lesions

d-TGA with Atrial Switch:

• Progressive exercise intolerance or HF → CPET (COR 1)
• Invasive hemodynamic assessment for progressive symptoms/HF/PAH/arrhythmia/pathway obstruction/baffle leak (COR 1)
• Baffle leak causing symptoms → closure (COR 1)
• Pathway stenosis causing symptoms → relieve stenosis (COR 1)
• Worsening/refractory HF → refer to HF/transplant program (COR 1)
• Atrial arrhythmias → oral anticoagulation (COR 2a)

d-TGA with Arterial Switch:

• Symptoms of myocardial ischemia → coronary evaluation with angiography ± cross-sectional imaging ± functional assessment (COR 1; upgraded from COR 2a)
• Evidence of ischemia → coronary revascularization (COR 1)

CCTGA:

• Periodic echocardiography for chamber function, systemic TV function, associated lesions (COR 1) — new
• Ambulatory rhythm monitoring to screen for high-grade AV block (COR 2a) — new
• Symptomatic high-grade AV block → physiological pacing (CRT or conduction system pacing) (COR 2a) — new

Fontan Circulation:

• Annual liver imaging + AFP (alpha-fetoprotein) + lab evaluation for FALD/hepatocellular carcinoma (COR 1) — new
• Annual laboratory evaluation for organ dysfunction and hematologic abnormality (COR 1; upgraded from COR 2a)
• Hepatologist referral (COR 2a) — new; liver biopsy before transplantation consideration (COR 2a)
• New/progressive symptoms or organ dysfunction → cardiac catheterization (COR 1)
• New/progressive severe hypoxemia or hypotension → advanced cardiac imaging to exclude thrombus (COR 1)
• Antithrombotic: aspirin or anticoagulation in all Fontan without high-risk features or contraindications (COR 1; upgraded from COR 2b) — high risk = prior thromboembolism, sustained AFL/AF, or atriopulmonary Fontan
• Atrial flutter/AF → timely cardioversion (COR 1)
• Sinus node dysfunction with pacemaker → atrial-based pacing, minimize ventricular pacing (COR 1)
• AV block with high ventricular pacing burden (>40%) → apical pacing preferred (COR 2a)
• Cardiac rehabilitation/exercise programs beneficial (COR 2a)
• Pulmonary vasodilators may be considered in select patients (COR 2b)
• Fontan circulatory failure → formal HF/transplant evaluation by program experienced in adult Fontan transplantation (COR 1)
• Multidisciplinary committee review before transplantation (COR 1)
• concepts/Fontan-Circulation

Eisenmenger Syndrome:

• Invasive hemodynamic assessment to confirm diagnosis and exclude contributors (COR 1)
• Joint ACHD + PAH subspecialist management (COR 1)
• Pregnancy: COR 3: Harm — advise against; maternal mortality 30–50%
• Initial monotherapy with PAH-directed therapy (ERA or PDE5i) for symptomatic patients or reduced exercise capacity with LVEF >40% (COR 1; upgraded from COR 2a)
• Dual combination ERA + PDE5i if persistent symptoms on single agent (COR 1; upgraded from COR 2a)
• Atrial arrhythmia → prompt rhythm restoration (COR 1) — new
• Exercise program + PAH therapy (COR 2a) — new
• Routine oral anticoagulation: COR 3: No Benefit — high bleeding risk, no survival benefit; anticoagulation only if high-risk features (atrial arrhythmia, PA thrombosis, prior thromboembolism) and low bleeding risk (COR 2b)
• Shunt closure: COR 3: Harm — perioperative and long-term risks
• Endocardial leads with intracardiac shunts: COR 3: Harm — systemic thromboembolism risk
• concepts/Eisenmenger-Syndrome

Coronary Artery Anomalies

• Anomalous left coronary artery from right sinus: surgery reasonable in high-risk anatomy even if asymptomatic (COR 2a)
• Anomalous coronary artery from pulmonary artery (ALCAPA) → evidence of ischemia → revascularization (COR 1)

Limitations of the Document

• Most evidence in ACHD is non-randomized (B-NR or C-LD); RCTs are rare due to small and heterogeneous population
• Many recommendations extrapolated from acquired heart disease data (especially HF GDMT, CRT, ICD)
• Fontan management recommendations largely based on observational studies; limited long-term outcome data
• Guideline does not cover patients <18 years, acquired valvular heart disease, or inherited conditions with cardiac manifestations (e.g., Marfan syndrome, HCM)
• SCD risk scores (PREVENTION-ACHD, Brompton, PACES) for TOF have relatively small event numbers and have not been externally validated prospectively

Key Concepts Mentioned

• concepts/ACHD-AP-Classification — refined classification system with new Stage C/D criteria
• concepts/Tetralogy-of-Fallot — new PVR criteria (RVESVi), SCD risk stratification, ablation role
• concepts/Fontan-Circulation — annual liver surveillance, antithrombotic upgrade, exercise, transplantation
• concepts/Eisenmenger-Syndrome — PAH monotherapy COR 1, dual ERA+PDE5i COR 1, shunt closure COR 3:Harm
• entities/Pulmonary-Hypertension — PAH-directed therapy in Eisenmenger; PVR thresholds for shunt closure
• entities/Heart-Failure — GDMT, CRT, ICD, MCS in ACHD with systemic LV dysfunction
• concepts/Conduction-System-Pacing — physiological pacing in CCTGA; Fontan pacing strategy

Key Entities Mentioned

• entities/Atrial-Fibrillation — anticoagulation in Fontan, rhythm control in Eisenmenger and complex ACHD
• entities/Pulmonary-Hypertension — Eisenmenger PAH therapy, ASD/VSD PH management
• entities/Heart-Failure — ACHD HF section (new); GDMT, MCS, transplantation

Wiki Pages Updated

• Created: wiki/sources/ACHD-AHA-2025.md
• Created: wiki/concepts/ACHD-AP-Classification.md
• Created: wiki/concepts/Tetralogy-of-Fallot.md
• Created: wiki/concepts/Fontan-Circulation.md
• Created: wiki/concepts/Eisenmenger-Syndrome.md
• Updated: wiki/sourceindex.md
• Updated: wiki/wikiindex.md
• Updated: log.md