Serial echocardiography for valve dysfunction and aortic dilation in bicuspid aortic valves

Complications of bicuspid aortic valve commonly include aortic stenosis, aortic regurgitation, and ascending aortic dilation. The progression of these lesions is not well described.

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.

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.

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.