Giant right ventricular outflow tract thrombus in hereditary spherocytosis: a case report.
BACKGROUND
In hereditary spherocytosis with severe anemia, splenectomy is a recommended treatment. However, the spleen carries an important role both in immune function and coagulation. The increased risk of bacterial infections associated with splenectomy is well known. Recently, hypercoagulation disorders have also been linked to splenectomy through loss of regulation of platelet activity, loss of filtering function and post-splenectomy thrombocytosis.


METHODS
A 28 year-old smoking women who had previously undergone splenectomy due to hereditary spherocytosis with a moderate thrombocytosis (platelet count 553-635*10(9)/L), presented with recurrent episodes of pulmonary embolisms. Further examination by multimodality cardiac imaging demonstrated a giant chronic thrombus in the right ventricular outflow tract, which eventually had to be surgically removed.


CONCLUSIONS
The present case highlights the increased risk of severe thromboembolic complications following therapeutic splenectomy in hereditary spherocytosis, and emphasis the important role of multimodality cardiac imaging in recurrent pulmonary embolism, diagnosing a giant chronic thrombus in the right ventricular outflow tract.
Department of Heart Disease, Haukeland University Hospital, Bergen, Norway.
Cedric
C
Davidsen
Department of Heart Disease, Haukeland University Hospital, Bergen, Norway.
Terje Hjalmar
TH
Larsen
Department of Clinical Science, University of Bergen, Bergen, Norway.
Eva
E
Gerdts
Department of Heart Disease, Haukeland University Hospital, Bergen, Norway ; Department of Clinical Science, University of Bergen, Bergen, Norway.
Mai Tone
MT
Lønnebakken
Contrast echocardiography; Hereditary spherocytosis; Pulmonary embolism; Right ventricle; Thrombus