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J Thorac Cardiovasc Surg 2005;130:932-933
© 2005 The American Association for Thoracic Surgery

Brief Communication

Mitral valve reconstruction in a compound heterozygote for sickle cell anemia and hemoglobin Lepore

Konstantinos Tziomalos, MD a , * , Vassilia Garipidou, MD a , Eleni Houmpouridou, MD b , Antonios A. Pitsis, MD c , Elias Basayannis, MD a

a Second Propaedeutic Department of Internal Medicine, Aristotle University of Thessaloniki, Hippokration Hospital
b Department of Hematology, St Luke's Hospital, Thessaloniki, Greece
c Thessaloniki Heart Institute, St Luke's Hospital, Thessaloniki, Greece

Received for publication January 23, 2005; accepted for publication February 22, 2005.

* Address for reprints: Konstantinos Tziomalos, MD, 63 Solonos St, Thessaloniki, 54248 Greece

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


Figure 1
Pitsis, Tziomalos, Katsarkas, Basayannis (left to right)


Sickle cell disease presents a multitude of challenges in patients undergoing cardiac operations. In these patients, cardiopulmonary bypass (CPB) may trigger a crisis of profound magnitude. The prevention of sickling of red blood cells and its sequelae of hemolysis and vaso-occlusive crises during CPB is achieved primarily by reducing the level of hemoglobin S before operation. 1,2 Go

Hemoglobin Lepore ({alpha}2[{delta}ß]2) is a hemoglobin variant with a {delta}ß chain that is a result of fusion of a {delta} and a ß globin gene. The hybrid gene is expressed at a roughly intermediate level, between the {delta} and the ß globin genes. The phenotype of the heterozygote thus closely resembles the classical thalassemia trait. To date, only 20 cases of compound heterozygotes with hemoglobin Lepore/hemoglobin S have been reported, and they are characterized by hematologic as well as clinical heterogeneity. The hemoglobin S/hemoglobin . . . [Full Text of this Article]






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