A small but significant proportion (5-10%) of patients with severe mitral stenosis present with atypical features due to extreme pulmonary vascular resistance, which can obscure classical diagnostic signs.
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May prompt consideration of extreme PVR when mitral stenosis presents atypically; leaves open need for validation in modern cohorts.
A rise in pulmonary arterial pressure is almost invariable in severe mitral stenosis. In many patients it is slight or moderate but in a small proportion the pulmonary circulation reacts with extreme elevation of pulmonary vascular resistance and pulmonary arterial pressure. Some of these patients show the usual features of mitral stenosis but others present a difficult diagnostic problem, for many of the classical signs and symptoms of mitral stenosis may be absent or modified owing to the hindrance to blood flow proximal to the pulmonary capillary bed. From our ex- perience we believe that between five and ten per cent of patients found to require mitral valvotomy show these atypical features and this agrees with the incidence (7.6%) found by Wood (1954).
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Mackinnon et al. (1956) studied this question.
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