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01.12.2016 | Case report | Ausgabe 1/2016 Open Access

Thrombosis Journal 1/2016

Giant right ventricular outflow tract thrombus in hereditary spherocytosis: a case report

Thrombosis Journal > Ausgabe 1/2016
Cedric Davidsen, Terje Hjalmar Larsen, Eva Gerdts, Mai Tone Lønnebakken
Wichtige Hinweise

Electronic supplementary material

The online version of this article (doi:10.​1186/​s12959-016-0083-3) contains supplementary material, which is available to authorized users.

Competing interests

The authors declare that they have no competing interests.

Authors’ contributions

CD wrote the initial manuscript, did the literature review and prepared figures for publishing. THL contributed by image interpretation and acquisition. EG had the idea for the manuscript and contributed with critical revision of the manuscript. MTL supervised the drafting of the manuscript, helped with image acquisition and interpretation, and revised the final manuscript. All authors read and approved the final manuscript.



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.

Case presentation

A 28 year-old smoking women who had previously undergone splenectomy due to hereditary spherocytosis with a moderate thrombocytosis (platelet count 553–635*109/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.


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.
Additional file 1:
Transthoracic echocardiography showing the giant right ventricular thrombus in a parasternal short axis view. (WMV 5132 kb)
Additional file 2:
Contrast echocardiography in the parasternal short axis view, demonstrating the characteristic lack of contrast enhancement in the right ventricular thrombus. (WMV 5126 kb)
Additional file 3:
Cardiac magnetic resonance imaging in a sagittal plane demonstrating the piston-like shape of the thrombus, and how it protrudes through the pulmonary valve in systole. (WMV 1165 kb)
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