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

Daniel N. Beauchamp, Claudio Ramaciotti, Paul Brown, Poonam P. Thankavel

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish (US)
Pages (from-to)1229-1238
Number of pages10
JournalPediatric Cardiology
Volume43
Issue number6
DOIs
StatePublished - Aug 2022

Keywords

  • Bicuspid aortic valve
  • Congenital heart disease
  • Coronary arteries
  • Pediatric cardiology
  • Transthoracic echocardiography

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Cardiology and Cardiovascular Medicine

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