The Initial Diagnostic Approach to Adult Congenital Heart Disease: A Practical Imaging Pathway for General Cardiologists and Cardiac Imagers
References
- Erikssen G, Liestøl K, Seem E, et al. Achievements in congenital heart defect surgery: a prospective, 40-year study of 7038 patients. Circulation. 2014 Dec 23;131(4):337-346; discussion 46. doi: 10.1161/circulationaha.114.012033
- Gilboa SM, Devine OJ, Kucik JE, et al. Congenital Heart Defects in the United States: Estimating the Magnitude of the Affected Population in 2010. Circulation 2016 Jul 12;134(2):101-109. doi: 10.1161/circulationaha.115.019307
- 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 Feb 24;87(7):822-976. doi: 10.1016/j.jacc.2025.09.006
- Baumgartner H, De Backer J, Babu-Narayan SV, et al. 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J. 2021 Feb 11;42(6):563-645. doi: 10.1093/eurheartj/ehaa554
- Sachdeva R, Valente AM, Armstrong AK, et al. ACC/AHA/ASE/HRS/ISACHD/SCAI/SCCT/SCMR/SOPE 2020 Appropriate Use Criteria for Multimodality Imaging During the Follow-Up Care of Patients With Congenital Heart Disease: A Report of the American College of Cardiology Solution Set Oversight Committee and Appropriate Use Criteria Task Force, American Heart Association, American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and Society of Pediatric Echocardiography. J Am Coll Cardiol. 2020 Feb 18;75(6):657-703. doi: 10.1016/j.jacc.2019.10.002
- Fratz S, Chung T, Greil GF, et al. Guidelines and protocols for cardiovascular magnetic resonance in children and adults with congenital heart disease: SCMR expert consensus group on congenital heart disease. J Cardiovasc Magn Reson. 2013 Jun 13;15(1):
51 . doi: 10.1186/1532-429x-15-51 - Di Salvo G, Miller O, Babu Narayan S, et al. Imaging the adult with congenital heart disease: a multimodality imaging approach-position paper from the EACVI. Eur Heart J Cardiovasc Imaging. 2018 Oct 1;19(10):1077-1098. doi: 10.1093/ehjci/jey102
- Li W, West C, McGhie J, et al. Consensus recommendations for echocardiography in adults with congenital heart defects from the International Society of Adult Congenital Heart Disease (ISACHD). Int J Cardiol. 2018 Dec 1;272:77-83. doi: 10.1016/j.ijcard.2018.07.058
- Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol. 2019 Apr 2;73(12):e81-e192. doi: 10.1016/j.jacc.2018.08.1029
- Mitchell C, Rahko PS, Blauwet LA, et al. Guidelines for Performing a Comprehensive Transthoracic Echocardiographic Examination in Adults: Recommendations from the American Society of Echocardiography. J Am Soc Echocardiogr. 2019 Jan;32(1):1-64. doi: 10.1016/j.echo.2018.06.004
- Lopez L, Saurers DL, Barker PCA, et al. Guidelines for Performing a Comprehensive Pediatric Transthoracic Echocardiogram: Recommendations From the American Society of Echocardiography. J Am Soc Echocardiogr. 2024 Feb;37(2):119-170. doi: 10.1016/j.echo.2023.11.015
- Corbett L, Forster J, Gamlin W, et al. A practical guideline for performing a comprehensive transthoracic echocardiogram in the congenital heart disease patient: consensus recommendations from the British Society of Echocardiography. Echo Res Pract. 2022 Oct 18;9(1):
10 . doi: 10.1186/s44156-022-00006-5 - Silvestry FE, Cohen MS, Armsby LB, et al. Guidelines for the Echocardiographic Assessment of Atrial Septal Defect and Patent Foramen Ovale: From the American Society of Echocardiography and Society for Cardiac Angiography and Interventions. J Am Soc Echocardiogr. 2015 Aug;28(8):910-958. doi: 10.1016/j.echo.2015.05.015
- Frommelt P, Lopez L, Dimas VV, et al. Recommendations for Multimodality Assessment of Congenital Coronary Anomalies: A Guide from the American Society of Echocardiography: Developed in Collaboration with the Society for Cardiovascular Angiography and Interventions, Japanese Society of Echocardiography, and Society for Cardiovascular Magnetic Resonance. J Am Soc Echocardiogr. 2020 Mar;33(3):259-294. doi: 10.1016/j.echo.2019.10.011
- Simpson J, Lopez L, Acar P, et al. Three-dimensional Echocardiography in Congenital Heart Disease: An Expert Consensus Document from the European Association of Cardiovascular Imaging and the American Society of Echocardiography. J Am Soc Echocardiogr. 2017 Jan;30(1):1-27. doi: 10.1016/j.echo.2016.08.022
- Cohen MS, Eidem BW, Cetta F, et al. Multimodality Imaging Guidelines of Patients with Transposition of the Great Arteries: A Report from the American Society of Echocardiography Developed in Collaboration with the Society for Cardiovascular Magnetic Resonance and the Society of Cardiovascular Computed Tomography. J Am Soc Echocardiogr. 2016 Jul;29(7):571-621. doi: 10.1016/j.echo.2016.04.002
- Babu-Narayan SV, Giannakoulas G, Valente AM, et al. Imaging of congenital heart disease in adults. Eur Heart J. 2016 Apr 14;37(15):1182-1195. doi: 10.1093/eurheartj/ehv519
- Rangosch A, Duarte VE, Quiñones MA. Interatrial Septum Assessment by Transthoracic Echocardiography Part 1: Secundum ASD. Methodist Debakey Cardiovasc J. 2024 Aug 27;20(1):77-79. doi: 10.14797/mdcvj.1456
- Rangosch A, Duarte VE, Quiñones MA. Interatrial Septum Assessment Part 2: Sinus Venosus Defects. Methodist Debakey Cardiovasc J. 2026 Jan 13;22(1):1-3. doi: 10.14797/mdcvj.1778
- Duarte VE, Rajpal S. The Role of Multimodality Imaging in the Evaluation of Heart Failure and Surgical Transplant Planning of Patients with Adult Congenital Heart Disease. Heart Fail Clin. 2024 Apr;20(2):189-198. doi: 10.1016/j.hfc.2024.01.002
- Muthurangu V, Lurz P, Critchely JD, et al. Real-time assessment of right and left ventricular volumes and function in patients with congenital heart disease by using high spatiotemporal resolution radial k-t SENSE. Radiology. 2008 Sep;248(3):782-791. doi: 10.1148/radiol.2482071717
- Zhang X, Huang Y, Wang L, et al. Transcatheter Closure of Atrial Septal Defects with Cardiac Computed Tomography Sizing: Eight-Year Single-Center Practice. Cardiology. 2020;145(10):654-662. doi: 10.1159/000508650
- Modi VA, Dhore-Patil A, Valderrabano M, et al. Iatrogenic Ventricular Septal Defect Following Simple PVC Ablation: A Case of Delayed Percutaneous Closure. JACC Case Rep. 2025 Jul 30;30(21):
104435 . doi: 10.1016/j.jaccas.2025.104435 - Lotz J, Meier C, Leppert A, et al. Cardiovascular flow measurement with phase-contrast MR imaging: basic facts and implementation. Radiographics. 2002 May-Jun;22(3):651-671. doi: 10.1148/radiographics.22.3.g02ma11651
- Isaak A, Mesropyan N, Hart C, et al. Non-contrast free-breathing 3D cardiovascular magnetic resonance angiography using REACT (relaxation-enhanced angiography without contrast) compared to contrast-enhanced steady-state magnetic resonance angiography in complex pediatric congenital heart disease at 3T. J Cardiovasc Magn Reson. 2022 Nov 17;24(1):
55 . doi: 10.1186/s12968-022-00895-9 - Fotaki A, Pushparajah K, Hajhosseiny R, et al. Free-breathing, Contrast Agent-free Whole-Heart MTC-BOOST Imaging: Single-Center Validation Study in Adult Congenital Heart Disease. Radiol Cardiothorac Imaging. 2023 Feb 16;5(1):
e220146 . doi: 10.1148/ryct.220146 - Pennig L, Wagner A, Weiss K, et al. Imaging of the pulmonary vasculature in congenital heart disease without gadolinium contrast: Intraindividual comparison of a novel Compressed SENSE accelerated 3D modified REACT with 4D contrast-enhanced magnetic resonance angiography. J Cardiovasc Magn Reson. 2020 Jan 23;22(1):
8 . doi: 10.1186/s12968-019-0591-y - Steeden JA, Pandya B, Tann O, et al. Free breathing contrast-enhanced time-resolved magnetic resonance angiography in pediatric and adult congenital heart disease. J Cardiovasc Magn Reson. 2015 May 22;17(1):
38 . doi: 10.1186/s12968-015-0138-9 - Hope MD, Hope TA, Zhu C, et al. Vascular Imaging With Ferumoxytol as a Contrast Agent. AJR Am J Roentgenol. 2015 Sep;205(3):W366-W373. doi: 10.2214/ajr.15.14534
- Bashir MR, Bhatti L, Marin D, et al. Emerging applications for ferumoxytol as a contrast agent in MRI. J Magn Reson Imaging. 2015 Apr;41(4):884-898. doi: 10.1002/jmri.24691
- Cavalcante JL, Lalude OO, Schoenhagen P, et al. Cardiovascular Magnetic Resonance Imaging for Structural and Valvular Heart Disease Interventions. JACC Cardiovasc Interv. 2016 Mar 14;9(5):399-425. doi: 10.1016/j.jcin.2015.11.031
- Bissell MM, Raimondi F, Ait Ali L, et al. 4D Flow cardiovascular magnetic resonance consensus statement: 2023 update. J Cardiovasc Magn Reson. 2023 Jul 20;25(1):
40 . doi: 10.1186/s12968-023-00942-z - Rizk J. 4D flow MRI applications in congenital heart disease. Eur Radiol. 2021 Feb;31(2):1160-1174. doi: 10.1007/s00330-020-07210-z
- Gorecka M, Bissell MM, Higgins DM, et al. Rationale and clinical applications of 4D flow cardiovascular magnetic resonance in assessment of valvular heart disease: a comprehensive review. J Cardiovasc Magn Reson. 2022 Aug 22;24(1):
49 . doi: 10.1186/s12968-022-00882-0 - Oechtering TH, Nowak A, Sieren MM, et al. Repeatability and reproducibility of various 4D Flow MRI postprocessing software programs in a multi-software and multi-vendor cross-over comparison study. J Cardiovasc Magn Reson. 2023 Mar 28;25(1):
22 . doi: 10.1186/s12968-023-00921-4 - Greenhill MJ, Rangan P, Su W, et al. MRI in Patients with Cardiovascular Implantable Electronic Devices and Fractured or Abandoned Leads. Radiol Cardiothorac Imaging. 2024 Jun;6(3):
e230303 . doi: 10.1148/ryct.230303 - Schaller RD, Brunker T, Riley MP, et al. Magnetic Resonance Imaging in Patients With Cardiac Implantable Electronic Devices With Abandoned Leads. JAMA Cardiol. 2021 May 1;6(5):549-556. doi: 10.1001/jamacardio.2020.7572
- Kim D, Collins JD, White JA, et al. SCMR expert consensus statement for cardiovascular magnetic resonance of patients with a cardiac implantable electronic device. J Cardiovasc Magn Reson. 2024 Summer;26(1):
100995 . doi: 10.1016/j.jocmr.2024.100995 - Pedrosa I, Altman DA, Dillman JR, et al. American College of Radiology Manual on MR Safety: 2024 Update and Revisions. Radiology. 2025 Apr;315(1):
e241405 . doi: 10.1148/radiol.241405 - Hilbert S, Jahnke C, Loebe S, et al. Cardiovascular magnetic resonance imaging in patients with cardiac implantable electronic devices: a device-dependent imaging strategy for improved image quality. Eur Heart J Cardiovasc Imaging. 2018 Sep 1;19(9):1051-1061. doi: 10.1093/ehjci/jex243
- Weinreb JC, Rodby RA, Yee J, et al. Use of Intravenous Gadolinium-based Contrast Media in Patients with Kidney Disease: Consensus Statements from the American College of Radiology and the National Kidney Foundation. Radiology. 2021 Jan;298(1):28-35. doi: 10.1148/radiol.2020202903
- Woolen SA, Shankar PR, Gagnier JJ, et al. Risk of Nephrogenic Systemic Fibrosis in Patients With Stage 4 or 5 Chronic Kidney Disease Receiving a Group II Gadolinium-Based Contrast Agent: A Systematic Review and Meta-analysis. JAMA Intern Med. 2020 Feb 1;180(2):223-230. doi: 10.1001/jamainternmed.2019.5284
- Han BK, Rigsby CK, Hlavacek A, et al. Computed Tomography Imaging in Patients with Congenital Heart Disease Part I: Rationale and Utility. An Expert Consensus Document of the Society of Cardiovascular Computed Tomography (SCCT): Endorsed by the Society of Pediatric Radiology (SPR) and the North American Society of Cardiac Imaging (NASCI). J Cardiovasc Comput Tomogr. 2015 Nov-Dec;9(6):475-492. doi: 10.1016/j.jcct.2015.07.004
- Duarte VE, Urey MA, Adler ED, et al. Multi-Organ Transplantation in Adult Congenital Heart Disease: Navigating the Unique Challenges of a Distinct Patient Population. Methodist Debakey Cardiovasc J. 2025 May 15;21(3):72-82. doi: 10.14797/mdcvj.1576
- Verheijen DBH, Engele LJ, Egorova AD, et al. Late follow-up of neo-aortic dimensions and coronary arteries in adult patients after the arterial switch operation. Int J Cardiol Congenit Heart Dis. 2023 Oct 14;14:
100481 . doi: 10.1016/j.ijcchd.2023.100481 - Kumar P, Bhatia M. Role of Computed Tomography in Postoperative Follow-up of Arterial Switch Operation. J Cardiovasc Imaging. 2021 Jan;29(1):1-19. doi: 10.4250/jcvi.2020.0106
- Han BK, Garcia S, Aboulhosn J, et al. Technical recommendations for computed tomography guidance of intervention in the right ventricular outflow tract: Native RVOT, conduits and bioprosthetic valves:: A white paper of the Society of Cardiovascular Computed Tomography (SCCT), Congenital Heart Surgeons’ Society (CHSS), and Society for Cardiovascular Angiography & Interventions (SCAI). J Cardiovasc Comput Tomogr. 2024 Jan-Feb;18(1):75-99. doi: 10.1016/j.jcct.2023.06.005
- Han BK, Rigsby CK, Leipsic J, et al. Computed Tomography Imaging in Patients with Congenital Heart Disease, Part 2: Technical Recommendations. An Expert Consensus Document of the Society of Cardiovascular Computed Tomography (SCCT): Endorsed by the Society of Pediatric Radiology (SPR) and the North American Society of Cardiac Imaging (NASCI). J Cardiovasc Comput Tomogr. 2015 Nov-Dec;9(6):493-513. doi: 10.1016/j.jcct.2015.07.007
- Kumar P, Bhatia M. Computed Tomography in the Evaluation of Fontan Circulation. J Cardiovasc Imaging. 2021 Apr;29(2):108-122. doi: 10.4250/jcvi.2020.0119
- Lesser AM, Newell MC, Samara MA, et al. Radiation dose and image quality of 70 kVp functional cardiovascular computed tomography imaging in congenital heart disease. J Cardiovasc Comput Tomogr. 2016 Mar-Apr;10(2):173-178. doi: 10.1016/j.jcct.2015.12.009
DOI: https://doi.org/10.14797/mdcvj.1868 | Journal eISSN: 1947-6108
Language: English
Page range: 5 - 27
Submitted on: May 10, 2026
Accepted on: Jun 12, 2026
Published on: Jun 30, 2026
Published by: Houston Methodist DeBakey Heart & Vascular Center
In partnership with: Paradigm Publishing Services
Keywords:
© 2026 Alicia Rangosch, Aneesh Dhore-Patil, Valeria E. Duarte, published by Houston Methodist DeBakey Heart & Vascular Center
This work is licensed under the Creative Commons Attribution-NonCommercial 4.0 License.