Late last year, the American College of Cardiology (ACC) and the American Heart Association (AHA) released an update to the 2018 guideline on managing adults with congenital heart disease. The 2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the Management of Adults with Congenital Heart Disease published on December 18, 2025. The guideline was published by the ACC and AHA, and endorsed by the Heart Rhythm Society (HRS), the International Society for Adult Congenital Heart Disease (ISACHD), and the Society for Cardiovascular Angiography and Interventions (SCAI).
Today, we are taking a look at how the 2025 guideline differs from the 2018 release by comparing some key revisions that were made based on the evidence review conducted by the appointed writing committee.
Guidelines Referenced:
2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the Management of Adults With Congenital Heart Disease
2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease
- Published: August 2018
- Full Text
New Guideline Sections, Recommendations, and Revisions
The 2025 update featured new sections and new recommendations within existing sections. New sections include Management of Cyanosis, HLHS/Norwood Repair, and Vascular Rings and Pulmonary Artery Slings. Sections that received all new recommendations include Patients with d-TGA and Rastelli Repair, Patent Ductus Arteriosus, Heart Transplantation and MCS, and Heart Failure.
Ten sections received notable revisions. These revised sections include Atrial Septal Defect, Atrioventricular Septal Defect, Cor Triatriatum Sinister, Ebstein Anomaly, Isolated Branch Pulmonary Artery Stenosis, Management of Right Ventricle-to-Pulmonary Artery Conduits, Patients With dextro-Transposition of the Great Arteries and Atrial Switch, Arterial Switch Operation, Fontan Palliation of Single-Ventricle Physiology, and Eisenmenger Syndrome. The Eisenmenger Syndrome section appears as a standalone section, whereas the 2018 version paired the syndrome with severe pulmonary hypertension.
The following table reflects the revised guidance published in the 2025 update. To view the complete guideline, along with all the new recommendations and sections, view the full-text version using the link featured above.
Key Recommendation Revisions (2018-2025)
| Topic | 2025 Guideline | 2018 Guideline |
|---|---|---|
| Atrial Septal Defect | In adults with an isolated unrepaired secundum ASD, transcatheter closure is usually preferred to surgical repair to reduce length of stay and recovery time. | Percutaneous or surgical closure may be considered for adults with ASD when net left-to-right shunt (Qp:Qs) is ≥1.5:1, pulmonary artery (PA) systolic pressure is ≥50% of systemic arterial systolic pressure, and/or pulmonary vascular resistance (PVR) is greater than one-third of the systemic resistance. |
| Atrioventricular Septal Defect | Adults with an unrepaired AVSD or those with residual shunts after prior repair should be assessed for the presence of PAH to guide medical therapy and assess suitability for repair. In adults with repaired AVSD and LVOT obstruction with symptoms attributable to the obstruction, or LV systolic dysfunction (LV ejection fraction <50%), surgical repair is recommended to improve functional status and preserve ventricular function. In adults with an unrepaired AVSD or those with residual shunts after previous repair, significant left-to-right shunt (Qp:Qs ≥1.5), and/or significant or progressive atrial/ventricular dilatation with no PAH (PVR ≤2 Wood units), closure of the AVSD or residual shunt is recommended to improve functional class and clinical outcomes. | Cardiac catheterization can be useful in adults with atrioventricular septal defect (AVSD) when pulmonary hypertension is suspected. Operation for discrete left ventricular outflow tract (LVOT) obstruction in adults with ASVD is reasonable with a maximum gradient of ≥50 mm Hg, a lesser gradient if heart failure (HF) symptoms are present, or if concomitant moderate-to-severe mitral or atrial regurgitation are present. Surgery for primary repair of ASVD or closure of residual shunts in adults with repaired ASVD is recommended when there is a net left-to-right shunt (Qp:Qs ≥1.5:1), PA systolic pressure <50% systemic, and PVR less than one-third systemic. |
| Cor Triatriatum Sinister | In asymptomatic adults with unrepaired cor triatriatum sinister and severe membrane obstruction, surgical resection can be useful to prevent the sequelae of left atrial hypertension. | Surgical repair is indicated for adults with cor triatriatum sinister for symptoms attributable to the obstruction or substantial gradient across the membrane. |
| Ebstein Anomaly | In adults with Ebstein anomaly, an electrophysiological study is recommended for patients with symptomatic arrhythmia or asymptomatic ventricular preexcitation to assess for substrates for ablation, including multiple or high-risk accessory pathways. | Electrophysiological study with or without catheter ablation can be useful in the diagnostic evaluation of adults with Ebstein anomaly and ventricular preexcitation but without supraventricular tachycardia. |
| Isolated Branch Pulmonary Artery Stenosis | In adults with symptoms related to peripheral or branch PA stenosis, pulmonary branch balloon angioplasty and/or stent implantation is recommended to improve hemodynamics and symptoms. | In adults with peripheral or branch PA stenosis, PA dilation and stenting can be useful. |
| Management of Right Ventricle-to-Pulmonary Artery Conduits | For adults with an RV-to-PA conduit, severe stenosis and/or severe regurgitation, and symptoms or worsening functional capacity or a sustained arrhythmia, conduit intervention should be performed to improve cardiovascular status. | RV-to-PA conduit intervention is reasonable for adults with an RV-to-PA conduit and moderate or greater PR or moderate or greater stenosis with reduced functional capacity or arrhythmia. |
| Patients With dextro-Transposition of the Great Arteries and Atrial Switch | Adults with d-TGA and atrial switch with progressive exercise intolerance, HF, pulmonary hypertension, worsening atrial or ventricular tachyarrhythmia, pathway obstruction, or baffle leak should undergo invasive hemodynamic assessment to identify targets for medical, percutaneous, or surgical intervention. In adults with d-TGA and atrial switch with new or progressive symptoms of HF, it is reasonable to obtain a resting electrocardiogram and outpatient ambulatory rhythm monitoring, to ensure timely diagnosis and treatment of tachyarrhythmia or bradyarrhythmia. In adults with d-TGA and atrial switch who have sustained intra-atrial reentrant arrhythmias or atrial fibrillation, oral anticoagulation can be useful to prevent embolic events. | Assessment for a communication through the interatrial baffle or venous stenosis is reasonable for adults with d-TGA with atrial switch, particularly if transvenous pacemaker/ICD implantation is considered or leads are already present. Ambulatory monitoring for bradycardia or sinus node dysfunction is recommended for adults with d-TGA with atrial switch, especially if treated with beta blockers or other rate-slowing agents. GDMT with appropriate attention to the need for anticoagulation is recommended to promptly restore sinus rhythm for adults with d-TGA with atrial switch repair presenting with atrial arrhythmia. |
| Arterial Switch Operation | In adults with an arterial switch operation and symptoms concerning for myocardial ischemia, coronary evaluation with coronary angiography, cross-sectional imaging, and/or functional coronary assessment for an anatomic etiology should be performed. | Physiological tests of myocardial perfusion for adults with d-TGA after arterial switch can be beneficial for assessing symptoms suggestive of myocardial ischemia. |
| Fontan Palliation of Single-Ventricle Physiology | In adults with Fontan circulation, annual laboratory evaluation is recommended to assess for evidence of organ-system dysfunction or hematologic abnormality. In adults with Fontan circulation and the absence of high-risk features (history of thromboembolism, sustained atrial flutter/fibrillation, or atriopulmonary Fontan) or bleeding contraindications, treatment with either aspirin or anticoagulation is recommended to reduce the probability of thromboembolic disease. | In adults after Fontan palliation, it is reasonable to perform biochemical and hematological testing on an annual basis, especially for liver and renal function. Antiplatelet therapy or anticoagulation with a vitamin K antagonist may be considered in adults after Fontan palliation without known or suspected thrombus, thromboembolic events, or prior arrhythmia. |
| Eisenmenger Syndrome | In adults with Eisenmenger syndrome and an LV ejection fraction >40% who are symptomatic or have reduced exercise capacity, initial monotherapy with PAH-directed therapy is recommended to improve symptoms, hemodynamics and overall survival. In adults with Eisenmenger syndrome and an LV ejection fraction >40% who are symptomatic or have reduced exercise capacity, initial monotherapy with PAH-directed therapy is recommended to improve symptoms, hemodynamics, and overall survival. In adults with Eisenmenger syndrome and an LV ejection fraction >40% who remain symptomatic or have worsening exercise capacity on a single PAH therapy, dual combination therapy with an endothelin receptor antagonist and a PDE-5 inhibitor is recommended to improve symptoms, hemodynamics, and overall survival. | Bosentan is a reasonable therapy to treat symptomatic adults with Eisenmenger syndrome with 1 of the following: shunts other than ASD/VSD (eg, PDA, aortopulmonary window), or complex congenital heart lesions or Down syndrome. It is reasonable to use phosphodiesterase type 5 (PDE-5) inhibitors (eg, sildenafil, tadalafil) to treat symptomatic adults with Eisenmenger syndrome with ASD, VSD, or great artery shunt. In symptomatic adults with Eisenmenger syndrome, bosentan and PDE-5 inhibitors are reasonable in combination if symptomatic improvement does not occur with either medication alone. |
While not a revision itself, it's worth noting that the 2025 guideline also mentions that, going forward, emerging research will be reviewed on an ongoing basis, and "full revisions" and "focused updates" to the guideline will be phased out in favor of more dynamic updates focused on targeted sections.
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