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Coronary Artery Origins Pattern in Pediatric Patients with Right-Left Fusion Bicuspid Aortic Valve

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Abstract

Bicuspid aortic valve (BAV) is a common congenital heart defect associated with coronary artery (CA) variants, including higher incidence of left CA dominance and shorter left main CA length. We observed by transthoracic echocardiography that left and right CA origins appear closer together in pediatric patients with right-left fusion (R/L) BAV compared to patients with trileaflet aortic valves. We sought to objectively confirm this observation. A retrospective review of pediatric echocardiograms with R/L BAV at a single institution (12/2010–11/2018) was performed. The ‘coronary angle’ was defined as the angle between the left and right coronary artery origins in the parasternal short axis view relative to the center of the aortic valve orifice. Values were compared to age-matched controls. Patients with inadequate images, anomalous coronary origins, or predefined significant congenital heart defects were excluded. We compared 191 R/L BAV patients (64% male) to 136 controls (57% male). Coronary angle was significantly more acute in R/L BAV than in controls (117.9° ± 16.7° vs 139.0° ± 10.1°, p < 0.0001). This was independent of age and gender. The difference persisted when BAV patients with abnormal aortic annulus/root diameters were removed from analysis (119.5° ± 15.1° vs 139.0° ± 10.1°, p < 0.0001). CA origins are closer together in R/L BAV independent of age, gender, or annulus/root size. This new anatomical description may aid in the diagnosis of subtle (‘forme fruste’) R/L BAV, assist in interventional planning, and improve understanding of the relationship between BAV and CA development.

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Data Availability

Measurements and statistical analysis results are available from the authors if needed.

Code Availability

Not applicable

Abbreviations

AI:

Aortic insufficiency

ANOVA:

Analysis of variance

AS:

Aortic stenosis

ASD:

Atrial septal defect

BAV:

Bicuspid aortic valve

BSA:

Body surface area

CA:

Coronary artery/arteries

CoA:

Coarctation of the aorta

HSD:

Honestly significant difference

ICC:

Intraclass correlation coefficient

LCA:

Left coronary artery

LCC:

Left coronary cusp

LMCA:

Left main coronary artery

MS:

Mitral stenosis

NCC:

Noncoronary cusp

PFO:

Patent foramen ovale

PLAX:

Parasternal long axis

PSAX:

Parasternal short axis

R/L BAV:

Right-left fusion bicuspid aortic valve

RCA:

Right coronary artery

RCC:

Right coronary cusp

SD:

Standard deviation

SupraAS:

Supravalvar aortic stenosis

TAV:

Trileaflet aortic valve

TTE:

Transthoracic echocardiography

VSD:

Ventricular septal defect

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Acknowledgements

Joan Reisch, PhD (UT Southwestern Department of Population and Data Sciences) performed statistical analyses for this study.

Funding

No funds, grants, or other support was received from any external source. Statistical analysis was paid for by our echocardiography laboratory’s research fund.

Author information

Author notes

  1. Daniel N. Beauchamp

    Present address: Pediatric Residency Program, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Ave, Cincinnati, OH, 45229, USA

  2. Paul Brown & Poonam P. Thankavel

    Present address: Medical City Children’s Cardiology Specialists, 7777 Forest Ln, Ste A337, Dallas, TX, 75230, USA

Authors and Affiliations

  1. University of Texas Southwestern Medical School, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX, 75390, USA

    Daniel N. Beauchamp

  2. Division of Pediatric Cardiology, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX, 75390, USA

    Claudio Ramaciotti & Poonam P. Thankavel

  3. Children’s Health, 1935 Medical District Dr, Dallas, TX, 75235, USA

    Claudio Ramaciotti, Paul Brown & Poonam P. Thankavel

Authors
  1. Daniel N. Beauchamp
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  2. Claudio Ramaciotti
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Contributions

DNB: Lead author, project design, data collection (including coronary angle measurements), data management, manuscript review. CR: Project design, data collection (including coronary angle measurements), manuscript review. PB: Project design, data collection, manuscript review. PPT: Principal Investigator, project design, data collection (including coronary angle and aortic diameter measurements), manuscript review.

Corresponding author

Correspondence to Daniel N. Beauchamp.

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Conflict of Interest

The authors have no relevant financial or non-financial interests to disclose.

Ethical Approval

This retrospective chart review study involving human participants was in accordance with the ethical standards of the institutional and national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. The Institutional Review Boards at UT Southwestern Medical Center and Children’s Health approved this study.

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Informed consent was not required for this retrospective study that utilized non-identifiable data collected for routine clinical purposes.

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Consent for publication was not required for this retrospective study that utilized non-identifiable data collected for routine clinical purposes.

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Beauchamp, D.N., Ramaciotti, C., Brown, P. et al. Coronary Artery Origins Pattern in Pediatric Patients with Right-Left Fusion Bicuspid Aortic Valve. Pediatr Cardiol 43, 1229–1238 (2022). https://doi.org/10.1007/s00246-022-02843-x

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  • DOI: https://doi.org/10.1007/s00246-022-02843-x

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