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The Lone Cusp: A Patient With a Regurgitant Unicuspid Valve With Aortic Aneurysm.

• UAV is a rare clinical entity often misdiagnosed as BAV. • UAV can manifest with isolated AR. • UAV has a bimodal distribution: acommissural (infants) or unicommissural (adults). • TEE or cardiac magnetic resonance should be considered in younger patients with BAV. • Aortopathy can occur without severe UAV stenosis.

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