Key result
Ross procedure with personalised external aortic root support manages severe quadricuspid aortic regurgitation.
Why the study?
Quadricuspid aortic valve is a rare congenital anomaly usually associated with regurgitation, with limited data on functional implications of cusp fusion patterns.
Case Report (n=1)
Illustrates the clinical progression and multidisciplinary surgical planning for a rare quadricuspid aortic valve with functional bicuspid behavior.
Warrants close surveillance for progressive regurgitation in quadricuspid valves; leaves open optimal timing and role of Ross procedure.
Quadricuspid aortic valve is a rare congenital anomaly usually associated with regurgitation. A young woman underwent surveillance after cardiovascular magnetic resonance imaging demonstrated four equal cusps in diastole but bicuspid-type systolic opening from fusion of the left and non-coronary cusps. By 2025, eccentric regurgitation was severe on echocardiography and moderate-to-severe on magnetic resonance imaging, with a regurgitant fraction of 31% and mild left ventricular dilatation. Increasing breathlessness and declining peak oxygen uptake prompted multidisciplinary review. Valve replacement with ascending aortic support was recommended, and she was considered suitable for a Ross procedure with personalised external aortic root support.
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Ahmed et al. (2026) conducted a case report in Quadricuspid aortic valve (n=1). Ross procedure with personalised external aortic root support was evaluated. In a young woman with a quadricuspid aortic valve and severe regurgitation, a Ross procedure with personalised external aortic root support was recommended to address functional bicuspid anatomy.
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