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Neonatal aortic arch thrombosis: analysis of thrombophilic risk factors and prognosis

Published online by Cambridge University Press:  21 January 2013

Ivonne Wieland ,
Thomas Jack ,
Kathrin Seidemann ,
Martin Boehne ,
Florian Schmidt ,
Christoph M. Happel ,
Harald Koeditz ,
Harald Bertram  and
Michael Sasse
Ivonne Wieland
Affiliation:
Department of Pediatric Cardiology and Intensive Care Medicine, Hannover Medical School, Hannover, Germany Department of Haematology and Oncology, Hannover Medical School, Hannover, Germany
Thomas Jack*
Affiliation:
Department of Pediatric Cardiology and Intensive Care Medicine, Hannover Medical School, Hannover, Germany
Kathrin Seidemann
Affiliation:
Department of Pediatric Cardiology and Intensive Care Medicine, Hannover Medical School, Hannover, Germany
Martin Boehne
Affiliation:
Department of Pediatric Cardiology and Intensive Care Medicine, Hannover Medical School, Hannover, Germany
Florian Schmidt
Affiliation:
Department of Pediatric Cardiology and Intensive Care Medicine, Hannover Medical School, Hannover, Germany
Christoph M. Happel
Affiliation:
Department of Pediatric Cardiology and Intensive Care Medicine, Hannover Medical School, Hannover, Germany
Harald Koeditz
Affiliation:
Department of Pediatric Cardiology and Intensive Care Medicine, Hannover Medical School, Hannover, Germany
Harald Bertram
Affiliation:
Department of Pediatric Cardiology and Intensive Care Medicine, Hannover Medical School, Hannover, Germany
Michael Sasse
Affiliation:
Department of Pediatric Cardiology and Intensive Care Medicine, Hannover Medical School, Hannover, Germany
*
Correspondence to: Dr T. Jack, MD, Department of Pediatric Cardiology and Intensive Care Medicine, Hannover Medical School, Carl-Neuberg-Str. 1, 30635 Hannover, Germany. Tel: +49 511 532 9041; Fax: +49 511 532 8390; E-mail: Jack.Thomas@mh-hannover.de
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Abstract

Arterial thrombosis in neonates and children is a rare event and is often associated with external risk factors such as asphyxia or sepsis. We report our experiences with two neonates with spontaneous aortic arch thrombosis mimicking aortic coarctation. Despite single case reports until now, no data exist for the underlying thrombophilic risk factors and prognosis of this rare event. Both patients were carriers of a heterozygous factor V Leiden mutation, which has been reported once before as a risk factor for aortic arch thrombosis. One of our patients was operated upon successfully and is alive. The second patient suffered a large infarction of the right medial cerebral artery and had a thrombotic occlusion of the inferior caval vein. The patient obtained palliative care and died at the age of 6 days. In the literature, we identified 19 patients with neonatal aortic arch thrombosis. Of the 19 patients, 11 (58%) died. Including the two reported patients, the mortality rate of patients with multiple thromboses was 80% (8/10) compared with 18% (2/11) for patients with isolated aortic arch thrombosis; this difference reached statistical significance (p = 0.009). The analysis of thrombophilic disorders revealed that factor V Leiden mutation and protein C deficiency seem to be the most common risk factors for aortic arch thrombosis. Conclusion: Neonatal aortic arch thrombosis is a very rare but life-threatening event, with a high rate of mortality, especially if additional thrombotic complications are present. Factor V Leiden mutation seems to be one important risk factor in the pathogenesis of this fatal disease.

Keywords

Aortic arch thrombosisthrombophilic risk factorscoarctation of the aorta
Type
Original Articles
Information
Cardiology in the Young , Volume 24 , Issue 1 , February 2014 , pp. 33 - 39
Copyright
Copyright © Cambridge University Press 2013 

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Footnotes

†

These authors contributed equally.

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