Mitral Valve Replacement and Tricuspid Valve Repair in a Patient with Sickle Cell Disease

Introduction

Sickle cell disease (SCD) was first described by a Chicago physician, Herrik, in 1910. The sickle cell gene (S gene) has a worldwide distribution but has its greatest concentration in West Central Africa, the northeast corner of Saudi Arabia and East Central India. Homozygous sickle cell disease produces a chronic incurable illness with unpredictable life-threatening events. However, the sickle cell trait (SCT), the carrier state, is almost completely benign.

A reduction of the hemoglobin S (HbS) fraction to less than 30 % is required for patients undergoing cardiopulmonary bypass (CPB) [[1]]. This can be achieved by simple transfusion, partial exchange transfusion, complete exchange transfusion with the cardiopulmonary bypass circuit, or a combination of these techniques [[2], [3]].

We report the management of a Greek woman, with homozygous SCD and rheumatoid arthritis, who underwent partial exchange transfusion preoperatively and subsequent mitral valve replacement and tricuspid valve repair at normothermia.

References

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