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Es una afección cardíaca congénita que ocurre durante el desarrollo del corazón en el útero de la madre. Durante el desarrollo de este órgano, partes de su lado izquierdo (válvula mitral, válvula aórtica del ventrículo izquierdo y la aorta) no se desarrollan por completo. En pacientes con esta afección, el lado izquierdo del corazón es incapaz de enviar suficiente sangre al cuerpo.
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