title: Neonatal aortic arch thrombosis: analysis of thrombophilic risk factors and prognosis.
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.
  
  
  CONCLUSIONS
  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.
authors: 1 Department of Pediatric Cardiology and Intensive Care Medicine, Hannover Medical School, Hannover, Germany.
authors: Ivonne
authors: I
authors: Wieland
authors: 1 Department of Pediatric Cardiology and Intensive Care Medicine, Hannover Medical School, Hannover, Germany.
authors: Thomas
authors: T
authors: Jack
authors: 1 Department of Pediatric Cardiology and Intensive Care Medicine, Hannover Medical School, Hannover, Germany.
authors: Kathrin
authors: K
authors: Seidemann
authors: 1 Department of Pediatric Cardiology and Intensive Care Medicine, Hannover Medical School, Hannover, Germany.
authors: Martin
authors: M
authors: Boehne
authors: 1 Department of Pediatric Cardiology and Intensive Care Medicine, Hannover Medical School, Hannover, Germany.
authors: Florian
authors: F
authors: Schmidt
authors: 1 Department of Pediatric Cardiology and Intensive Care Medicine, Hannover Medical School, Hannover, Germany.
authors: Christoph M
authors: CM
authors: Happel
authors: 1 Department of Pediatric Cardiology and Intensive Care Medicine, Hannover Medical School, Hannover, Germany.
authors: Harald
authors: H
authors: Koeditz
authors: 1 Department of Pediatric Cardiology and Intensive Care Medicine, Hannover Medical School, Hannover, Germany.
authors: Harald
authors: H
authors: Bertram
authors: 1 Department of Pediatric Cardiology and Intensive Care Medicine, Hannover Medical School, Hannover, Germany.
authors: Michael
authors: M
authors: Sasse
mesh_terms: D020016:Activated Protein C Resistance / Q000175:diagnosis / Q000235:genetics*; D000792:Angiography; D001013:Aorta, Thoracic; D001018:Aortic Diseases / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D004452:Echocardiography; D005165:Factor V / Q000235:genetics*; D005260:Female; D006579:Heterozygote; D006801:Humans; D007231:Infant, Newborn; D020244:Infarction, Middle Cerebral Artery / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D008297:Male; D011379:Prognosis; D020151:Protein C Deficiency / Q000175:diagnosis / Q000235:genetics; D012307:Risk Factors; D013927:Thrombosis / Q000175:diagnosis / Q000235:genetics* / Q000628:therapy; D014682:Vena Cava, Inferior; D020246:Venous Thrombosis / Q000175:diagnosis / Q000235:genetics / Q000628:therapy
chemical_list: C095381:factor V Leiden; D005165:Factor V