Role of Cardiovascular Imaging in the Follow-Up of Patients with Fontan Circulation
Open accessAbstract
Since its first description in 1971, the Fontan procedure and its modifications have led to a substantial improvement in the survival rates of patients with a variety of types of complex Congenital Heart Disease (CHD) characterised by the presence of a single, dominant ventricle. However, despite the significant improvement of the prognosis over the years, Fontan patients are still exposed to several cardiovascular and systemic complications. It is, therefore, important to fully understand the pitfalls hidden behind a Fontan anatomy and the potential predictors of ventricular failure. Cardiovascular imaging plays a key role in this context, allowing for the early identification of complications with important prognostic implications. Echocardiography remains the first-line imaging modality for serial evaluation of Fontan patients. However, there is a growing role of cardiovascular magnetic resonance and cardiac computed tomography from pre-operative assessment to longitudinal follow-up. The aim of this paper will be to provide a comprehensive overview of the role, strengths, and weaknesses of each imaging modality in the assessment of congenital cardiac conditions palliated with the Fontan procedure.
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Phase-contrast flow CMR sequence measures blood flow velocities and can assess the severity of any obstructions or abnormalities in blood flow.
“This sequence measures blood flow velocities and can assess the severity of any obstructions or abnormalities in blood flow.”
From Multimodality imaging assessment of tetralogy of Fallot: From diagnosis to long-term follow-up (2023) · cites this paper as [33]
The CMR Protocol (§3.1; page not provided) says phase-contrast imaging quantifies blood-flow velocities and can examine flow across sites for stenosis. The discussion of Fontan complications (§3.2; page not provided) adds that flow sequences can identify accelerations and estimate peak velocity. These passages support evaluating particular obstructions and flow abnormalities, but do not establish that the sequence assesses the severity of any such abnormality. (LLM, conf=0.82)
Experimental — accuracy not yet measuredCine CMR images allow for evaluation of wall thickness and regional and global systolic function, as well as calculation of LV and RV end-diastolic and end-systolic volumes and ejection fraction.
“These images allow for the evaluation of wall thickness and regional and global systolic function, as well as the calculation of the LV and RV end-diastolic and end-systolic volumes and the ejection fraction.”
From Multimodality imaging assessment of tetralogy of Fallot: From diagnosis to long-term follow-up (2023) · cites this paper as [33]
Section 3.2 (page unavailable) says bSSFP cine images assess regional wall-motion abnormalities and systolic impairment and calculate end-diastolic and end-systolic volumes. Section 3.1 says cine slices are used to calculate ventricular volumes and ejection fractions. The paper does not specifically attribute wall-thickness evaluation to cine images, and its findings concern Fontan patients’ dominant and sometimes rudimentary ventricles rather than routine measurement of both the LV and RV. (LLM, conf=0.87)
Experimental — accuracy not yet measuredRV myocardial work indices have shown an important correlation with invasive indices of RV systolic function and prognostic value, especially in patients with pulmonary hypertension.
“Although RV MW indices have shown an important correlation with invasive indices of RV systolic function and prognostic value, especially in patients with pulmonary hypertension [28,33], their role in TOF still needs to be investigated.”
From Multimodality imaging assessment of tetralogy of Fallot: From diagnosis to long-term follow-up (2023) · cites this paper as [33]
The Introduction and §2.2, Single Ventricle Function, discuss ventricular performance and prognostic measures in Fontan patients, but not right-ventricular myocardial work indices. Searches for myocardial work and for correlations with invasive RV systolic measures in pulmonary hypertension found no passage addressing the attributed claim. (LLM, conf=0.74)
Experimental — accuracy not yet measured
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Moscatelli, S., Pergola, V., Motta, R., Fortuni, F., Borrelli, N., Sabatino, J., Leo, I., Avesani, M., Montanaro, C., Surkova, E., et al. (2023) · Children · doi:10.3390/children10111747 · Cited by 17 · see citing works
Self-citationThe Role of Multimodality Imaging in Pediatric Cardiomyopathies
Moscatelli, S., Leo, I., Bianco, F., Borrelli, N., Beltrami, M., Garofalo, M., Milano, E., Bisaccia, G., Iellamo, F., Bassareo, P., et al. (2023) · Journal of Clinical Medicine · doi:10.3390/jcm12144866 · Cited by 12 · see citing works
Self-citationMoscatelli, S., Leo, I., Bianco, F., Surkova, E., Pezel, T., Donald, N. A., Triumbari, E. K. A., Bassareo, P. P., Pradhan, A. K., Cimini, A., et al. (2023) · Diagnostics · doi:10.3390/diagnostics13243638 · Cited by 11 · see citing works
Self-citationMoscatelli, S., Pozza, A., Leo, I., Ielapi, J., Scatteia, A., Piana, S., Cavaliere, A., Reffo, E., & Salvo, G. D. (2024) · Children · doi:10.3390/children11070878 · Cited by 11 · see citing works
Self-citationMoscatelli, S., Leo, I., Lisignoli, V., Boyle, S., Bucciarelli-Ducci, C., Secinaro, A., & Montanaro, C. (2023) · Children · doi:10.3390/children10050763 · Cited by 6 · see citing works
Self-citationAvesani, M., Sabatino, J., Borrelli, N., Cattapan, I., Leo, I., Pelaia, G., Moscatelli, S., Bianco, F., Bassareo, P. P., Martino, F., et al. (2024) · Frontiers in Cardiovascular Medicine · doi:10.3389/fcvm.2024.1301116 · Cited by 5 · see citing works
Self-citationBorrelli, N., Sabatino, J., Gimelli, A., Avesani, M., Pergola, V., Leo, I., Moscatelli, S., Abbate, M., Motta, R., Sarro, R. D., et al. (2025) · Journal of Clinical Medicine · doi:10.3390/jcm14061862 · Cited by 5 · see citing works
Self-citationKowalczyk, M., Kordybach-Prokopiuk, M., Marczak, M., Hoffmań, P., & Kowalski, M. (2024) · International Journal of Cardiology · doi:10.1016/j.ijcard.2024.132286 · Cited by 3 · see citing works
Duarte, V. E., & Rajpal, S. (2024) · Cardiac Electrophysiology Clinics · doi:10.1016/j.ccep.2026.01.002 · Cited by 3 · see citing works
Borderline Ventricles: From Evaluation to Treatment
Mazza, G. A., Oreto, L., Tuo, G., Sirico, D., Moscatelli, S., Meliota, G., Micari, A., Guccione, P., Rinelli, G., & Favilli, S. (2024) · Diagnostics · doi:10.3390/diagnostics14080823 · Cited by 1 · see citing works
Self-citationPatient-specific input data for predictive modelling of the Fontan procedure
Dobroserdova, T. K., Анатольевна, Ю. Л., Vassilevski, Y. V., & Свободов, А. А. (2024) · Mathematical Modelling of Natural Phenomena · doi:10.1051/mmnp/2024013 · Cited by 1 · see citing works
Intracardiac fluid dynamic analysis: available techniques and novel clinical applications
Leo, I., Cersosimo, A., Ielapi, J., Sabatino, J., Sicilia, F., Strangio, A., Figliozzi, S., Torella, D., & Rosa, S. D. (2024) · BMC Cardiovascular Disorders · doi:10.1186/s12872-024-04371-3 · Cited by 1 · see citing works
Self-citationŞuteu, C. C., Cerghit-Paler, A., Gozar, L., Făgărășan, A., Șuteu, N., & Iancu, M. (2025) · Journal of Clinical Medicine · doi:10.3390/jcm14228134 · Cited by 1 · see citing works
Sanders, E. N., Chen, J. Z., Nissen, T. E., Daily, J. A., Zakaria, D., Dalby, S. T., & Bolin, E. H. (2024) · Pediatric Cardiology · doi:10.1007/s00246-024-03651-1 · Cited by 1 · see citing works
Editorial: An International Journey into the Research Progress of Pediatric Cardiology
Mapelli, M., & Zagni, P. (2023) · Children · doi:10.3390/children10020321 · Cited by 1 · see citing works
Extracardiac Conduit Subocclusion in an Adult Patient With a Fontan Circulation
D’Angelo, E. C., Palmieri, R., Lisignoli, V., Già, G. D., Porta, M. D., Iuzzolino, R., Curione, D., Albanese, S. B., Butera, G., & Montanaro, C. (2025) · JACC Case Reports · doi:10.1016/j.jaccas.2025.103399
Self-citationMoscatelli, S., Sabatino, J., Leo, I., Borrelli, N., Avesani, M., Salvo, G. D., Montanaro, C., Pergola, V., Motta, R., Ielapi, J., et al. (2025) · Journal of Cardiovascular Development and Disease · doi:10.3390/jcdd12090349
Self-citationÇevlik, B., Oğuz, A. S., Kangel, D., Yakut, K., Toprak, M. H. H., Tanıdır, İ. C., Baş, S., Hatemi, A. C., & Öztürk, E. (2026) · Journal of Clinical Medicine · doi:10.3390/jcm15135286
Platnick, C., Frank, B. S., Romanowicz, J., de Alba, C. G., Chase, M. A., Umer, A., Chaudhuri, N. R., Jonathan, A., Chauhan, D., Mascio, C. E., et al. (2026) · Frontiers in Cardiovascular Medicine · doi:10.3389/fcvm.2026.1937933
- Authors
- Moscatelli, S., Borrelli, N., Sabatino, J., Leo, I., Avesani, M., Montanaro, C., & Di Salvo, G.
- Affiliations
- Royal Brompton & Harefield NHS Foundation Trust (GB)
- Ospedale Monaldi (IT)
- Azienda Ospedale - Università Padova (IT)
- Division of Paediatric Cardiology, Department of Women and Children’s Health, University Hospital Padua, 35128 Padua, Italy
- ORCID
- Venue
- Children
- Publisher
- MDPI AG
- Published
- 2022-11-30
- Volume
- 9
- Issue
- 12
- Pages
- 1875
- ISSN
- 2227-9067 (ISSN-L)
- PMC
- PMC9777137
- OpenAlex
- W4311013877
- Cited by
- 21
- References
- 77
- Citations by year
- 2026: 2 · 2025: 4 · 2024: 8 · 2023: 7
- FWCI
- 0.75
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Licence
- Locations
- Children (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
- Archivio Istituzionale della Ricerca - Università degli Studi della Campania "Luigi Vanvitelli" (submittedVersion)
- Padua Research Archive (University of Padova) (submittedVersion, cc-by)
- Topics
- Health Sciences › Medicine › Epidemiology › Congenital Heart Disease Studies
- Health Sciences › Medicine › Radiology, Nuclear Medicine and Imaging › Cardiac Imaging and Diagnostics
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac Arrhythmias and Treatments
- Keywords
- Fontan circulation, Fontan procedure, congenital heart disease, cardiovascular imaging, echocardiography, cardiovascular magnetic resonance, cardiac CT, ventricular failure, single ventricle
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