Unileaflet mitral valve is an extremely rare congenital anomaly that can present asymptomatically in adulthood but may carry a risk of developing worsening mitral regurgitation later in life.
Unileaflet mitral valve warrants echocardiographic recognition in adults; this case leaves open natural history and progression risk.
Unileaflet mitral valve is the rarest of the congenital mitral valve anomalies and is usually life threatening in infancy due to severe mitral regurgitation (MR). In most asymptomatic individuals, it is mostly due to hypoplastic posterior mitral leaflet. We present a 22-year-old male with palpitations, who was found to have an echocardiogram revealing an elongated anterior mitral valve leaflet with severely hypoplastic posterior mitral valve leaflet appearing as a unileaflet mitral valve without MR. Our case is one of the 11 reported cases in the literature so far. We hereby review those cases and conclude that these patients are likely to be at risk of developing worsening MR later in their lives.
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Shah et al. (2016) studied this question.
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