Author + information
- Received September 6, 2011
- Accepted September 14, 2011
- Published online June 19, 2012.
A 33-year-old woman was referred to a cardiologist for an early systolic ejection click and grade III/VI mid peaking systolic murmur at the upper left sternal border. An echocardiogram revealed moderate pulmonic stenosis with a mean gradient of 31 mm Hg, peak gradient of 52 mm Hg, and trace pulmonic regurgitation. Right ventricular size, systolic function, and systolic pressure were all normal. Cardiac magnetic resonance (CMR) imaging confirmed a bicuspid pulmonic valve (BPV) with moderately restricted leaflet opening (A, left: end-diastolic; B, right: end-systolic) (Online Video 1). Four-dimensional (4D) CMR demonstrated post-stenotic supravalvular systolic separated flow, with a significant region of counterclockwise recirculation (C, left; Online Video 2). In contrast, 4D CMR in a healthy 37-year-old woman showed primarily attached supravalvular systolic flow, with minimal separated or recirculating regions (D, right; Online Video 3).
Bicuspid pulmonic valve is associated with post-stenotic pulmonary arterial dilation and aneurysm formation (1). 4D CMR in bicuspid aortic valve patients has demonstrated nested helical systolic flow and asymmetrically elevated wall shear stress in the ascending aorta (2). It is hypothesized that this increased hemodynamic burden causes bicuspid aortic valve–associated aortic aneurysm formation. Given the similarities in valve morphology and altered blood flow patterns, separated post-stenotic flow and increased wall shear stress may play a role in bicuspid pulmonic valve-associated pulmonary artery aneurysm formation.
Drs. Fenster and Schroeder received grant support for this work from Siemens Medical Solutions USA, Malvern, Pennsylvania. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- Received September 6, 2011.
- Accepted September 14, 2011.
- American College of Cardiology Foundation