• BACKGROUND AND AIMS
    • Complications of bicuspid aortic valve commonly include aortic stenosis, aortic regurgitation, and ascending aortic dilation. The progression of these lesions is not well described.
  • MATERIALS AND METHODS
    • We reviewed 249 bicuspid aortic valve patients with at least two echocardiograms from 2006 to 2016. Valve morphology (right-left or right-noncoronary cusp fusion) was confirmed by visual inspection, and aortic stenosis and regurgitation were quantified according to current guidelines; the ascending aorta was measured at end-systole 2-3 cm above the sinotubular junction. Annualized progression of stenosis, regurgitation, and aortic dilation from first to most recent echocardiogram were compared between right-left and right-nonfused valves using multivariable logistic regression to adjust for baseline differences in groups.
  • RESULTS
    • Among 249 bicuspid aortic valve patients (mean age 47.6 ± 13.5 years, 66.3% male), 75.9% had right-left cusp fusion. At baseline, aortic stenosis was absent or mild in 80.3%; aortic regurgitation was absent or mild in 80.7%; and aortic diameters were 35.0 ± 5.7 mm (sinuses of Valsalva) and 37.4 ± 6.2 mm (ascending). Mean annualized decrease in aortic valve area was 0.07 cm2 /year, with 30% of bicuspid aortic valve patients progressing ≥0.1 cm2 /year. Aortic regurgitation progressed ≥1 grade in 37 patients. Mean annualized increase in ascending aorta diameter was 0.36 mm/year in right-left and 0.65 mm/year in right-nonbicuspid valves.
  • CONCLUSIONS
    • In this serial echocardiographic study of bicuspid aortic valve patients, cusp orientation was not associated with progression of valve dysfunction. Right-noncoronary cusp fusion was associated with ascending aortic diameter progression.