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Cited by 34Congenital Heart Disease StudiesCardiac Valve Diseases and TreatmentsPulmonary Hypertension Research and Treatments

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End‐Diastolic Forward Flow and Restrictive Physiology in Repaired Tetralogy of Fallot: A Systematic Review and Meta‐Analysis

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Van den Eynde, Jef, Derdeyn, Emilie, Schuermans, Art, Shivaram, Pushpa, Budts, Werner, Danford, David A., Kutty, Shelby·2022·Journal of the American Heart Association
DOI 10.1161/jaha.121.024036PMC PMC9075485
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Abstract

Background Pulmonary arterial end-diastolic forward flow (EDFF) following repaired tetralogy of Fallot has been thought to represent right ventricular (RV) restrictive physiology, but is not fully understood. This systematic review and meta-analysis sought to clarify its physiological and clinical correlates, and to define a framework for understanding EDFF and RV restrictive physiology. Methods and Results PubMed/MEDLINE, Embase, Scopus, and reference lists of relevant articles were searched for observational studies published before March 2021. Random-effects meta-analysis was performed to identify factors associated with EDFF. Forty-two individual studies published between 1995 and 2021, including a total of 2651 participants (1132 with EDFF; 1519 with no EDFF), met eligibility criteria. The pooled estimated prevalence of EDFF among patients with repaired tetralogy of Fallot was 46.5% (95% CI, 41.6%-51.3%). Among patients with EDFF, the use of a transannular patch was significantly more common, and their stay in the intensive care unit was longer. EDFF was associated with greater RV indexed volumes and mass, as well as smaller E-wave velocity at the tricuspid valve. Finally, pulmonary regurgitation fraction was greater in patients with EDFF, and moderate to severe pulmonary regurgitation was more common in this population. Conclusions EDFF is associated with dilated, hypertrophied RVs and longstanding pulmonary regurgitation. Although several studies have defined RV restrictive physiology as the presence of EDFF, our study found no clear indicators of poor RV compliance in patients with EDFF, suggesting that EDFF may have multiple causes and might not be the precise equivalent of RV restrictive physiology.

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  • Patients with repaired TOF tend to require multiple pulmonary valve replacements.

    “Patients with repaired TOF tend to require multiple PV replacements (PVR), and CMR plays a pivotal role in selecting the correct replacement timing [44,48,49].”

    From Multimodality imaging assessment of tetralogy of Fallot: From diagnosis to long-term follow-up (2023) · cites this paper as [49]

    The Discussion (Perspectives for Future Research and Clinical Practice; page unavailable) describes pulmonary valve replacement as a common procedure in repaired tetralogy of Fallot. It does not say patients tend to need multiple replacements. Its discussion of CMR concerns identifying end-diastolic forward flow, not selecting replacement timing. (LLM, conf=0.76)

    Experimental — accuracy not yet measured

  • RV restrictive physiology should be suspected when, despite long-standing pulmonary regurgitation, the RV is not dilated.

    “The RVRP needs to be suspected when, despite the presence of a long-standing PR, the RV is not dilated.”

    From Multimodality imaging assessment of tetralogy of Fallot: From diagnosis to long-term follow-up (2023) · cites this paper as [49]

    In the Discussion (Table 4; page unavailable), the authors describe a small-RV phenotype with abnormal filling that corresponds most closely to RVRP, supporting suspicion of restriction when the RV has not enlarged. They do not establish an undilated RV despite longstanding PR as a diagnostic rule; the Discussion also notes that residual outflow obstruction can prevent enlargement even with substantial PR. (LLM, conf=0.78)

    Experimental — accuracy not yet measured

  • RV restrictive physiology refers to abnormalities in RV diastolic function observed after initial repair and during late follow-up, initially linked to end-diastolic forward flow into the pulmonary arteries notable at CMR and echocardiography.

    “RV restrictive physiology (RVRP) refers to abnormalities in RV diastolic function observed after initial repair and during late follow-up. Initially linked to end-diastolic forward flow (EDFF) into the Pas, that was notable both at CMR and echocardiography.”

    From Multimodality imaging assessment of tetralogy of Fallot: From diagnosis to long-term follow-up (2023) · cites this paper as [49]

  • CMR plays a pivotal role in selecting the correct timing of pulmonary valve replacement in repaired TOF.

    “Patients with repaired TOF tend to require multiple PV replacements (PVR), and CMR plays a pivotal role in selecting the correct replacement timing [44,48,49].”

    From Multimodality imaging assessment of tetralogy of Fallot: From diagnosis to long-term follow-up (2023) · cites this paper as [49]

    The Results (Study Selection and Characteristics; page unavailable) identify CMR as a tool used to detect end-diastolic forward flow, not to select pulmonary valve replacement timing. The Discussion (Perspectives for Future Research and Clinical Practice; page unavailable) says the relevance of that finding to risk stratification for replacement still needs investigation. Searches for CMR-guided replacement timing found no passage addressing the claim. (LLM, conf=0.73)

    Experimental — accuracy not yet measured

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Research works printed but not linked: 66

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Metadata

Authors
Van den Eynde, J., Derdeyn, E., Schuermans, A., Shivaram, P., Budts, W., Danford, D. A., & Kutty, S.
Affiliations
  • Helen B. Taussig Heart Center The Johns Hopkins Hospital and School of Medicine Baltimore MD
  • University of Antwerp (BE)
  • Universitair Ziekenhuis Leuven (BE)
  • Augusta University (US)
  • Johns Hopkins University (US)
Corresponding author
Kutty, Shelby
ORCID
Venue
Journal of the American Heart Association
Publisher
Ovid Technologies (Wolters Kluwer Health)
Published
2022-04-05
Volume
11
Issue
7
Pages
e024036-e024036
ISSN
2047-9980 (ISSN-L)
DOI
10.1161/jaha.121.024036
PMC
PMC9075485
OpenAlex
W4220754218
Cited by
34
References
69
Citations by year
2026: 6 · 2025: 13 · 2024: 7 · 2023: 7 · 2022: 1
FWCI
1.11
Type
journal article
Language
en
Open access
Licence
cc-by-nc-nd
Locations
  • Journal of the American Heart Association (publishedVersion, cc-by-nc-nd)
  • PubMed Central (publishedVersion, cc-by-nc-nd)
  • PubMed (publishedVersion)
  • Lirias (submittedVersion, cc-by-nc-nd)
  • DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion)
Topics
  • Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Congenital Heart Disease Studies
  • Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac Valve Diseases and Treatments
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Pulmonary Hypertension Research and Treatments
Keywords
repaired tetralogy of Fallot, right ventricular hypertrophy, right ventricular dilatation, pulmonary regurgitation, transannular patch, right ventricular compliance, right ventricular mass
MeSH terms
Diastole, Humans, Pulmonary Valve Insufficiency, Tetralogy of Fallot, Tricuspid Valve, Ventricular Function, Right, Ventricular Dysfunction, Right

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