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Large Right Ventricle Thrombus in Uhl’s Anomaly: A Rare Presentation of Extremely Rare Disease

Uhl’s anomaly is an extremely rare congenital cardiac malformation and is characterized by the partial or complete absence of right ventricular myocardium. The absence of myocardium may be the result of primary non-development of myocytes or a form of selective apoptosis. It is mainly sporadic altho...

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Detalles Bibliográficos
Autores principales: Singh, Gurkirat, Khemani, Hemant, Shaikh, Shakil, Bade, Arun, Singla, Rahul, Bansal, Narender Omprakash
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elmer Press 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6469915/
https://www.ncbi.nlm.nih.gov/pubmed/31019643
http://dx.doi.org/10.14740/cr758w
Descripción
Sumario:Uhl’s anomaly is an extremely rare congenital cardiac malformation and is characterized by the partial or complete absence of right ventricular myocardium. The absence of myocardium may be the result of primary non-development of myocytes or a form of selective apoptosis. It is mainly sporadic although some familial occurrences have been reported. Congestive cardiac failure is the most common mode of presentation. Associated congenital cardiac malformations are also reported. We report a case of a 17-year-old male who presented with symptoms and signs of right heart failure, during evaluation found to have large right ventricle free wall thrombus.