Uhl anomaly
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Uhl anomaly is a rare cardiac malformation that was first identified by Dr. Henry Uhl in 1952. It is characterized by the absence of the right ventricle (RV) myocardium, either entirely or partially, and the replacement of the RV myocardium by nonfunctional fibroelastic tissue that resembles parchment.[1] As of 2010 less than 100 cases have been reported in literature.[2]
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Uhl anomaly | |
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Uhl anomaly is inherited via autosomal dominant manner | |
Specialty | Medical genetics |
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Patients will typically present as infants with right-sided heart failure.[3] Atrial right-to-left shunting is frequently observed as the cause of cyanosis.[4]
Typically, magnetic resonance imaging, computed tomography, and echocardiography are used to make the diagnosis.[5]