Severe Bicuspid Aortic Valve Disease in an Adult Cystic Fibrosis Patient

Abstract

Bicuspid aortic valve is the most common congenital heart disease. Bicuspid aortic valves are prone to accelerated degenerative changes and aortopathies. These changes often manifest in adulthood as severe aortic stenosis or mixed aortic valve disease. Cystic fibrosis patients are at high risk of adverse surgical outcomes. As survival in cystic fibrosis continues to increase, managing comorbidities including severe aortic stenosis requires consideration. The relatively non-invasive transcatheter aortic valve replacement has been posed as an intervention for high-risk patients with severe symptomatic aortic stenosis. However, traditional randomized trials have excluded patients with bicuspid aortic valves. Herein we present an extremely rare association of severe bicuspid aortic valve stenosis in an adult cystic fibrosis patient. Furthermore, we discuss the clinical course and a multi-disciplinary approach for the management of this rare scenario.

Keywords: aortic valve stenosis; bicuspid aortic valve; cystic fibrosis; surgical aortic valve replacement; tavr (transcatheter aortic valve replacement).

Figure 1. Cardiac MRI

Cardiac MRI showing A) three-chamber view in long axis during systole showing severe bicuspid aortic stenosis with a large anterior leaflet (red arrow), smaller posterior leaflet (yellow arrow), and eccentric jet of blood (blue star) across the valve B) during diastole showing mild aortic regurgitation jet (green arrow) C) short-axis view showing bicuspid aortic valve with large anterior (green star) and small posterior leaflet (white star).