Interpretation of the 2025 ACC/AHA guidelines for the management of adults with congenital heart disease_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

LING Jing ^1,2 , WU Jiaxiong ^1,2 , LIANG Runzhang ^1,2 , PENG Zirui ^1,2 , YUAN Haiyun ^1,2 ,  WEN Shusheng ^1,2

1. Department of Cardiovascular Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial People’s Hospital (Guangdong Academy of Medical Sciences), Southern Medical University, Guangzhou, 510080, P. R. China;
2. Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangzhou, 510080, P. R. China;

Corresponding?author：

WEN Shusheng, Email: wenshusheng@gdph.org.cn

Keywords：

Adult congenital heart disease; lifelong management; pulmonary arterial hypertension; multidisciplinary team; pregnancy and reproduction; arrhythmia; guideline interpretation

DOI：

10.7507/1007-4848.202601104

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With the rapid advancements in surgical and interventional therapies, over 90% of children with congenital heart disease can now survive into adulthood with appropriate treatment. As a rapidly expanding population, adult congenital heart disease (ACHD) patients often present with complex anatomical abnormalities and post-procedural complications, posing significant long-term management challenges for clinicians. The American College of Cardiology and American Heart Association jointly released the 2025 guidelines for the management of adult congenital heart disease, which represents a comprehensive and fundamental update following the inaugural 2008 edition. This new guideline not only integrates the latest evidence-based data but also marks a paradigm shift in ACHD management, from a disease-centered approach to a patient-centered lifelong continuum of care. This article aims to provide an interpretation of the guidelines from the perspective of a cardiac surgeon to provide references for the management of various disease subtypes.

Citation： LING Jing, WU Jiaxiong, LIANG Runzhang, PENG Zirui, YUAN Haiyun, WEN Shusheng. Interpretation of the 2025 ACC/AHA guidelines for the management of adults with congenital heart disease. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2026, 33(6): 874-883. doi: 10.7507/1007-4848.202601104 Copy

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2.	Johnson AJ, McGrath LB, Khan AM, et al. Hospital care for adult patients with congenital heart diseases. Heart Surg Forum, 2023, 26(6): E842-E854.
3.	Lachtrupp CL, Valente AM, Gurvitz M, et al. Associations between clinical outcomes and a recently proposed adult congenital heart disease anatomic and physiological classification system. J Am Heart Assoc, 2021, 10(18): e021345.
4.	Tay EL, Peset A, Papaphylactou M, et al. Replacement therapy for iron deficiency improves exercise capacity and quality of life in patients with cyanotic congenital heart disease and/or the Eisenmenger syndrome. Int J Cardiol, 2011, 151(3): 307-312.
5.	Bozkurt B, Coats AJS, Tsutsui H, et al. Universal definition and classification of heart failure: a report of the Heart Failure Society of America, Heart Failure Association of the European Society of Cardiology, Japanese Heart Failure Society and Writing Committee of the Universal Definition of Heart Failure: endorsed by the Canadian Heart Failure Society, Heart Failure Association of India, Cardiac Society of Australia and New Zealand, and Chinese Heart Failure Association. Eur J Heart Fail, 2021, 23(3): 352-380.
6.	Chubb H, Mah DY, Shah M, et al. Multicenter study of survival benefit of cardiac resynchronization therapy in pediatric and congenital heart disease. JACC Clin Electrophysiol, 2024, 10(3): 539-550.
7.	Brida M, Chessa M, Celermajer D, et al. Atrial septal defect in adulthood: a new paradigm for congenital heart disease. Eur Heart J, 2022, 43(28): 2660-2671.
8.	Redfield MM. Building better defects: novel atrial septal defect strategies for cardiovascular therapy. JACC Heart Fail, 2023, 11(8 Pt 2): 1131-1134.
9.	Gupta SS, Sinha A, Sarasam R, et al. Ventricular septal defect. QJM, 2016, 109(10): 691-692.
10.	Bernard AC, Marchetta S, Dulgheru R, et al. Ventricular septal defect and infective endocarditis. Acta Cardiol, 2021, 76(1): 97-98.
11.	Backer CL, Eltayeb O, Mongé MC, et al. Shunt lesions partⅠ: patent ductus arteriosus, atrial septal defect, ventricular septal defect, and atrioventricular septal defect. Pediatr Crit Care Med, 2016, 17(8 Suppl 1): S302-S309.
12.	Martin BJ, Karamlou TB, Tabbutt S. Shunt lesions partⅡ: anomalous pulmonary venous connections and truncus arteriosus. Pediatr Crit Care Med, 2016, 17(8 Suppl 1): S310-S314.
13.	Kuntz MT, Staffa SJ, Graham D, et al. Trend and outcomes for surgical versus transcatheter patent ductus arteriosus closure in neonates and infants at US children's hospitals. J Am Heart Assoc, 2022, 11(1): e022776.
14.	Kilkenny K, Frishman W. Cor triatriatum: a review. Cardiol Rev, 2025, 33(4): 330-333.
15.	Arockiam AD, Rosenzveig A, Sorathia S, et al. Contemporary review of subaortic stenosis characteristics, multi-modality imaging and management. Curr Cardiol Rep, 2025, 27(1): 164.
16.	van der Linde D, Roos-Hesselink JW, Rizopoulos D, et al. Surgical outcome of discrete subaortic stenosis in adults: a multicenter study. Circulation, 2013, 127: 1184-1191.
17.	Sz?cs K, Toprak B, Sch?n G, et al. Concomitant cardiovascular malformations in isolated bicuspid aortic valve disease: a retrospective cross-sectional study and meta-analysis. Cardiovasc Diagn Ther, 2022, 12(4): 400-414.
18.	Kozel BA, Barak B, Kim CA, et al. Williams syndrome. Nat Rev Dis Primers, 2021, 7(1): 42.
19.	Salciccioli KB, Zachariah JP. Coarctation of the aorta: modern paradigms across the lifespan. Hypertension, 2023, 80(10): 1970-1979.
20.	Egbe AC, Miranda WR, Jain CC, et al. Low incidence of new intracranial aneurysms in adults with coarctation of aorta on serial brain imaging. Am J Cardiol, 2024, 216: 46-47.
21.	Mazzolai L, Teixido-Tura G, Lanzi S, et al. 2024 ESC guidelines for the management of peripheral arterial and aortic diseases. Eur Heart J, 2024, 45(36): 3538-3700.
22.	Egbe AC, Miranda WR, Connolly HM. Predictors of left ventricular reverse remodelling after coarctation of aorta intervention. Eur Heart J Cardiovasc Imaging, 2021, 22: 1168-1173.
23.	Connolly HM, Dearani JA, Miranda WR. Ebstein anomaly: we should do better. J Am Coll Cardiol, 2023, 81(25): 2431-2433.
24.	Karl TR, Stocker C. Tetralogy of Fallot and its variants. Pediatr Crit Care Med, 2016, 17(8 Suppl 1): S330-S336.
25.	Hammond BH, Etheridge SP, Saarel EV. Tetralogy of Fallot: a long journey but getting closer to perfection. Circ Arrhythm Electrophysiol, 2023, 16(6): e012042.
26.	Wijesekera VA. Tetralogy of Fallot in adults. Aust J Gen Pract, 2024, 53(7): 449-452.
27.	Broberg CS, van Dissel A, Minnier J, et al. Long-term outcomes after atrial switch operation for transposition of the great arteries. J Am Coll Cardiol, 2022, 80(10): 951-963.
28.	Krause U, Teubener ST, Müller MJ, et al. Fate after the Mustard procedure for d-transposition of the great arteries: impact of age, complexity, and atrial tachyarrhythmias: a single center experience. Pediatr Cardiol, 2023, 44(8): 1746-1753.
29.	Mateos Gaitán R, Boix-Palop L, Mu?oz García P, et al. Infective endocarditis in patients with cardiac implantable electronic devices: a nationwide study. Europace, 2020, 22(7): 1062-1070.
30.	Fricke TA, Buratto E, Weintraub RG, et al. Long-term outcomes of the arterial switch operation. J Thorac Cardiovasc Surg, 2022, 163(1): 212-219.
31.	Habib G, Erba PA, Iung B, et al. Clinical presentation, aetiology and outcome of infective endocarditis. Results of the ESC-EORP EURO-ENDO (European Infective Endocarditis) registry: a prospective cohort study. Eur Heart J, 2019, 40(39): 3222-3232.
32.	Jacob KA, H?rer J, Hraska V, et al. Anatomic and physiologic repair of congenitally corrected transposition of the great arteries. J Am Coll Cardiol, 2024, 84(25): 2471-2486.
33.	Habib G, Lancellotti P, Antunes MJ, et al. 2015 ESC guidelines for the management of infective endocarditis: the Task Force for the Management of Infective Endocarditis of the European Society of Cardiology (ESC). Endorsed by: European Association for Cardio-Thoracic Surgery (EACTS), the European Association of Nuclear Medicine (EANM). Eur Heart J, 2015, 36(44): 3075-3128.
34.	Lewis MJ, Reardon LC, Aboulhosn J, et al. Morbidity and mortality in adult Fontan patients after heart or combined heart-liver transplantation. J Am Coll Cardiol, 2023, 81(22): 2161-2171.
35.	Berg CJ, Bauer BS, Hageman A, et al. Mortality risk stratification in Fontan patients who underwent heart transplantation. Am J Cardiol, 2017, 119: 1675-1679.
36.	King G, Ayer J, Celermajer D, et al. Atrioventricular valve failure in Fontan palliation. J Am Coll Cardiol, 2019, 73(7): 810-822.
37.	Hilscher MB, Johnson JN. Fontan-associated liver disease. Semin Liver Dis, 2025, 45(1): 114-128.
38.	Arvanitaki A, Gatzoulis MA, Opotowsky AR, et al. Eisenmenger syndrome: JACC state-of-the-art review. J Am Coll Cardiol, 2022, 79(12): 1183-1198.
39.	Manuel L, Freeman L, Nashef SA. Surgery for Eisenmenger syndrome: time for a rethink? J R Soc Med, 2019, 112(12): 512-513.
40.	Jegatheeswaran A, Alsoufi B. Anomalous aortic origin of a coronary artery: 2020 year in review. J Thorac Cardiovasc Surg, 2021, 162(2): 353-359.
41.	Gentile F, Castiglione V, De Caterina R. Coronary artery anomalies. Circulation, 2021, 144(12): 983-996.

1. Gurvitz M, Krieger EV, Fuller S, 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. J Am Coll Cardiol, 2026, 87(7): 822-976.
2. Johnson AJ, McGrath LB, Khan AM, et al. Hospital care for adult patients with congenital heart diseases. Heart Surg Forum, 2023, 26(6): E842-E854.
3. Lachtrupp CL, Valente AM, Gurvitz M, et al. Associations between clinical outcomes and a recently proposed adult congenital heart disease anatomic and physiological classification system. J Am Heart Assoc, 2021, 10(18): e021345.
4. Tay EL, Peset A, Papaphylactou M, et al. Replacement therapy for iron deficiency improves exercise capacity and quality of life in patients with cyanotic congenital heart disease and/or the Eisenmenger syndrome. Int J Cardiol, 2011, 151(3): 307-312.
5. Bozkurt B, Coats AJS, Tsutsui H, et al. Universal definition and classification of heart failure: a report of the Heart Failure Society of America, Heart Failure Association of the European Society of Cardiology, Japanese Heart Failure Society and Writing Committee of the Universal Definition of Heart Failure: endorsed by the Canadian Heart Failure Society, Heart Failure Association of India, Cardiac Society of Australia and New Zealand, and Chinese Heart Failure Association. Eur J Heart Fail, 2021, 23(3): 352-380.
6. Chubb H, Mah DY, Shah M, et al. Multicenter study of survival benefit of cardiac resynchronization therapy in pediatric and congenital heart disease. JACC Clin Electrophysiol, 2024, 10(3): 539-550.
7. Brida M, Chessa M, Celermajer D, et al. Atrial septal defect in adulthood: a new paradigm for congenital heart disease. Eur Heart J, 2022, 43(28): 2660-2671.
8. Redfield MM. Building better defects: novel atrial septal defect strategies for cardiovascular therapy. JACC Heart Fail, 2023, 11(8 Pt 2): 1131-1134.
9. Gupta SS, Sinha A, Sarasam R, et al. Ventricular septal defect. QJM, 2016, 109(10): 691-692.
10. Bernard AC, Marchetta S, Dulgheru R, et al. Ventricular septal defect and infective endocarditis. Acta Cardiol, 2021, 76(1): 97-98.
11. Backer CL, Eltayeb O, Mongé MC, et al. Shunt lesions partⅠ: patent ductus arteriosus, atrial septal defect, ventricular septal defect, and atrioventricular septal defect. Pediatr Crit Care Med, 2016, 17(8 Suppl 1): S302-S309.
12. Martin BJ, Karamlou TB, Tabbutt S. Shunt lesions partⅡ: anomalous pulmonary venous connections and truncus arteriosus. Pediatr Crit Care Med, 2016, 17(8 Suppl 1): S310-S314.
13. Kuntz MT, Staffa SJ, Graham D, et al. Trend and outcomes for surgical versus transcatheter patent ductus arteriosus closure in neonates and infants at US children's hospitals. J Am Heart Assoc, 2022, 11(1): e022776.
14. Kilkenny K, Frishman W. Cor triatriatum: a review. Cardiol Rev, 2025, 33(4): 330-333.
15. Arockiam AD, Rosenzveig A, Sorathia S, et al. Contemporary review of subaortic stenosis characteristics, multi-modality imaging and management. Curr Cardiol Rep, 2025, 27(1): 164.
16. van der Linde D, Roos-Hesselink JW, Rizopoulos D, et al. Surgical outcome of discrete subaortic stenosis in adults: a multicenter study. Circulation, 2013, 127: 1184-1191.
17. Sz?cs K, Toprak B, Sch?n G, et al. Concomitant cardiovascular malformations in isolated bicuspid aortic valve disease: a retrospective cross-sectional study and meta-analysis. Cardiovasc Diagn Ther, 2022, 12(4): 400-414.
18. Kozel BA, Barak B, Kim CA, et al. Williams syndrome. Nat Rev Dis Primers, 2021, 7(1): 42.
19. Salciccioli KB, Zachariah JP. Coarctation of the aorta: modern paradigms across the lifespan. Hypertension, 2023, 80(10): 1970-1979.
20. Egbe AC, Miranda WR, Jain CC, et al. Low incidence of new intracranial aneurysms in adults with coarctation of aorta on serial brain imaging. Am J Cardiol, 2024, 216: 46-47.
21. Mazzolai L, Teixido-Tura G, Lanzi S, et al. 2024 ESC guidelines for the management of peripheral arterial and aortic diseases. Eur Heart J, 2024, 45(36): 3538-3700.
22. Egbe AC, Miranda WR, Connolly HM. Predictors of left ventricular reverse remodelling after coarctation of aorta intervention. Eur Heart J Cardiovasc Imaging, 2021, 22: 1168-1173.
23. Connolly HM, Dearani JA, Miranda WR. Ebstein anomaly: we should do better. J Am Coll Cardiol, 2023, 81(25): 2431-2433.
24. Karl TR, Stocker C. Tetralogy of Fallot and its variants. Pediatr Crit Care Med, 2016, 17(8 Suppl 1): S330-S336.
25. Hammond BH, Etheridge SP, Saarel EV. Tetralogy of Fallot: a long journey but getting closer to perfection. Circ Arrhythm Electrophysiol, 2023, 16(6): e012042.
26. Wijesekera VA. Tetralogy of Fallot in adults. Aust J Gen Pract, 2024, 53(7): 449-452.
27. Broberg CS, van Dissel A, Minnier J, et al. Long-term outcomes after atrial switch operation for transposition of the great arteries. J Am Coll Cardiol, 2022, 80(10): 951-963.
28. Krause U, Teubener ST, Müller MJ, et al. Fate after the Mustard procedure for d-transposition of the great arteries: impact of age, complexity, and atrial tachyarrhythmias: a single center experience. Pediatr Cardiol, 2023, 44(8): 1746-1753.
29. Mateos Gaitán R, Boix-Palop L, Mu?oz García P, et al. Infective endocarditis in patients with cardiac implantable electronic devices: a nationwide study. Europace, 2020, 22(7): 1062-1070.
30. Fricke TA, Buratto E, Weintraub RG, et al. Long-term outcomes of the arterial switch operation. J Thorac Cardiovasc Surg, 2022, 163(1): 212-219.
31. Habib G, Erba PA, Iung B, et al. Clinical presentation, aetiology and outcome of infective endocarditis. Results of the ESC-EORP EURO-ENDO (European Infective Endocarditis) registry: a prospective cohort study. Eur Heart J, 2019, 40(39): 3222-3232.
32. Jacob KA, H?rer J, Hraska V, et al. Anatomic and physiologic repair of congenitally corrected transposition of the great arteries. J Am Coll Cardiol, 2024, 84(25): 2471-2486.
33. Habib G, Lancellotti P, Antunes MJ, et al. 2015 ESC guidelines for the management of infective endocarditis: the Task Force for the Management of Infective Endocarditis of the European Society of Cardiology (ESC). Endorsed by: European Association for Cardio-Thoracic Surgery (EACTS), the European Association of Nuclear Medicine (EANM). Eur Heart J, 2015, 36(44): 3075-3128.
34. Lewis MJ, Reardon LC, Aboulhosn J, et al. Morbidity and mortality in adult Fontan patients after heart or combined heart-liver transplantation. J Am Coll Cardiol, 2023, 81(22): 2161-2171.
35. Berg CJ, Bauer BS, Hageman A, et al. Mortality risk stratification in Fontan patients who underwent heart transplantation. Am J Cardiol, 2017, 119: 1675-1679.
36. King G, Ayer J, Celermajer D, et al. Atrioventricular valve failure in Fontan palliation. J Am Coll Cardiol, 2019, 73(7): 810-822.
37. Hilscher MB, Johnson JN. Fontan-associated liver disease. Semin Liver Dis, 2025, 45(1): 114-128.
38. Arvanitaki A, Gatzoulis MA, Opotowsky AR, et al. Eisenmenger syndrome: JACC state-of-the-art review. J Am Coll Cardiol, 2022, 79(12): 1183-1198.
39. Manuel L, Freeman L, Nashef SA. Surgery for Eisenmenger syndrome: time for a rethink? J R Soc Med, 2019, 112(12): 512-513.
40. Jegatheeswaran A, Alsoufi B. Anomalous aortic origin of a coronary artery: 2020 year in review. J Thorac Cardiovasc Surg, 2021, 162(2): 353-359.
41. Gentile F, Castiglione V, De Caterina R. Coronary artery anomalies. Circulation, 2021, 144(12): 983-996.

Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Interpretation of the 2025 ACC/AHA guidelines for the management of adults with congenital heart disease

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