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J Thorac Cardiovasc Surg 2009;137:e36-e37
© 2009 The American Association for Thoracic Surgery

Brief Communication

Mitral valve repair during bypass in a 4-year-old girl with familial Evans syndrome

Aitizaz Uddin Syed, FRCS Ed, FRCS (C)*, Ali E. Jelly, MD, Arto Nemlander, MD, FathelRahman Elawad Ahmed, MD

North West Armed Forces Hospital, Tabuk, Saudi Arabia

Received for publication March 22, 2008; revisions received May 6, 2008; accepted for publication June 15, 2008.

* Address for reprints: Aitizaz Uddin Syed, FRCS Ed, FRCS (C), Consultant Cardiac Surgeon, North West Armed Forces Hospital, PO Box, 100, Tabuk, Saudi Arabia. (Email: Aitizaz@hotmail.com).

The first 20% of the full text of this article appears below.

In patients with Evans syndrome, autoantibodies of the IgG type are produced against red cells, platelets, and granulocytes, leading to phagocytosis of the coated cells by the reticuloendothelial system. This leads to recurrent episodes of intravascular hemolysis and thrombocytopenia in the affected individuals.1Go We report our experience of open mitral valve repair during cardiopulmonary bypass in a 4-year-old girl. This is the first reported case of a cardiac operation in a patient affected with this rare disorder.

Clinical Summary

The patient presented in March 2004 with mild mitral regurgitation. She was known to have antibody-mediated hemolytic anemia and low platelets counts. This child came from a unique family in which 2 other siblings were given diagnoses of "Evans syndrome."

Her mitral valve regurgitation continued to worsen rapidly, leading to shortness of breath on mild exertion. Echocardiographic analysis in February 2005 showed severe mitral regurgitation with anterior mitral leaflet prolapse. Vena contracta was 6 mm, with an effective regurgitant orifice . . . [Full Text of this Article]






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