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Cited by 169Congenital Heart Disease StudiesCongenital heart defects researchCardiac Valve Diseases and Treatments

Current outcomes and treatment of tetralogy of Fallot

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van der Ven, Jelle P.G., van den Bosch, Eva, Bogers, Ad J.C.C., Helbing, Willem A.·2019·F1000Research
DOI 10.12688/f1000research.17174.1PMC PMC6719677
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Abstract

Tetralogy of Fallot (ToF) is the most common type of cyanotic congenital heart disease. Since the first surgical repair in 1954, treatment has continuously improved. The treatment strategies currently used in the treatment of ToF result in excellent long-term survival (30 year survival ranges from 68.5% to 90.5%). However, residual problems such as right ventricular outflow tract obstruction, pulmonary regurgitation, and (ventricular) arrhythmia are common and often require re-interventions. Right ventricular dysfunction can be seen following longstanding pulmonary regurgitation and/or stenosis. Performing pulmonary valve replacement or relief of pulmonary stenosis before irreversible right ventricular dysfunction occurs is important, but determining the optimal timing of pulmonary valve replacement is challenging for several reasons. The biological mechanisms underlying dysfunction of the right ventricle as seen in longstanding pulmonary regurgitation are poorly understood. Different methods of assessing the right ventricle are used to predict impending dysfunction. The atrioventricular, ventriculo-arterial and interventricular interactions of the right ventricle play an important role in right ventricle performance, but are not fully elucidated. In this review we present a brief overview of the history of ToF, describe the treatment strategies currently used, and outline the long-term survival, residual lesions, and re-interventions following repair. We discuss important remaining challenges and present the current state of the art regarding these challenges.

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  • The surgical paradigm of correction of TOF has gradually shifted toward a more aggressive approach of preserving the pulmonary annulus at the cost of residual RVOT obstruction.

    “To prevent the consequences of chronic pulmonary valve regurgitation, the surgical paradigm of correction of TOF has gradually shifted toward a more aggressive approach of preserving the pulmonary annulus at the cost of residual RVOT obstruction.7”

    From Comparison of Long-term Outcomes of Valve-Sparing and Transannular Patch Procedures for Correction of Tetralogy of Fallot (2021) · cites this paper as [7]

    The Surgical approaches to repair section describes a shift toward preserving pulmonary valve competence to avoid the long-term effects of regurgitation. It says surgeons sought to do so “without causing significant residual RVOTO,” and that a transannular patch remains necessary when needed for adequate obstruction relief. It therefore does not support the claimed trade-off of accepting residual RVOT obstruction. (LLM, conf=0.88)

    Experimental — accuracy not yet measured

  • The insertion of a transannular patch (TAP) effectively relieves RVOT obstruction but leads to severe pulmonary valve regurgitation.

    “The insertion of a transannular patch (TAP) effectively relieves RVOT obstruction but leads to severe pulmonary valve regurgitation.7”

    From Comparison of Long-term Outcomes of Valve-Sparing and Transannular Patch Procedures for Correction of Tetralogy of Fallot (2021) · cites this paper as [7]

    The Surgical approaches to repair section says a TAP may be necessary for adequate RVOT obstruction relief and that pulmonary regurgitation was more common in patients with TAPs. It does not say TAP leads specifically to severe regurgitation; the Pulmonary regurgitation section reports moderate-to-severe regurgitation rates for repaired patients overall, not for TAP recipients specifically. (LLM, conf=0.86)

    Experimental — accuracy not yet measured

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Cited by

Sorted by citations

Authors
van der Ven, J. P. G., van den Bosch, E., Bogers, A. J. C. C., & Helbing, W. A.
Affiliations
Radboud University Nijmegen (NL)
Corresponding author
Helbing, Willem A.
ORCID
Venue
F1000Research
Publisher
F1000 Research Ltd
Published
2019-08-29
Volume
8
Pages
1530
ISSN
2046-1402 (ISSN-L)
DOI
10.12688/f1000research.17174.1
PMC
PMC6719677
OpenAlex
W2971225247
Cited by
169
References
9
Citations by year
2026: 8 · 2025: 33 · 2024: 32 · 2023: 35 · 2022: 29 · 2021: 18 · 2020: 14
FWCI
2.23
Type
journal article
Language
en
Open access
Licence
Locations
  • F1000Research (publishedVersion, cc-by)
  • Europe PMC (PubMed Central) (publishedVersion, cc-by)
  • PubMed Central (publishedVersion, cc-by)
  • PubMed (publishedVersion)
  • EUR Research Repository (Erasmus University Rotterdam) (publishedVersion, other-oa)
  • EUR Research Repository (Erasmus University Rotterdam) (submittedVersion, other-oa)
  • RePub (Erasmus University Rotterdam) (submittedVersion, other-oa)
  • DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
  • Radboud Repository (Radboud University) (submittedVersion)
  • RePub (Erasmus University Rotterdam) (submittedVersion)
  • Radboud Repository (Radboud University) (submittedVersion, other-oa)
Funders
Dutch Heart Foundation (2013T091)
Topics
  • Health Sciences › Medicine › Epidemiology › Congenital Heart Disease Studies
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Congenital heart defects research
  • Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac Valve Diseases and Treatments
Keywords
tetralogy of Fallot, cyanotic congenital heart disease, long-term survival, right ventricular outflow tract obstruction, pulmonary regurgitation, right ventricular dysfunction, pulmonary valve replacement, ventricular arrhythmias, reintervention
MeSH terms
Cardiac Surgical Procedures, Humans, Pulmonary Valve Insufficiency, Tetralogy of Fallot, Survival Rate, Ventricular Dysfunction, Right

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