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Central venous thrombosis and thrombophilia in cystic fibrosis: A prospective study

Anne Munck1, Ahmed Kheniche2, Corinne Alberti3

  • 1Assistance publique-Hôpitaux de Paris, Hôpital Robert Debré, Paediatric Gastroenterology and Respiratory Department, CF Centre, Université Paris 7, France.

Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society
|August 10, 2014
PubMed

Insights

Cystic fibrosis patients rarely develop catheter venous thrombosis despite high rates of thrombophilia. Medical history and ultrasound screening are recommended over routine lab tests before TIVAD insertion.

Area of Science:

  • Vascular Medicine
  • Hematology
  • Cystic Fibrosis Research

Background:

  • Catheter venous thrombosis poses embolic risks.
  • Cystic fibrosis (CF) patients may have a thrombophilic tendency.
  • The clinical significance of thrombophilia in CF regarding catheter complications is unclear.

Purpose of the Study:

  • To determine the frequency of catheter venous thrombosis (CVT) in CF patients using Doppler-US.
  • To evaluate genetic and acquired thrombophilia risk factors and hypercoagulability.
  • To provide recommendations for laboratory screening before totally implantable vascular access device (TIVAD) insertion in CF patients.

Main Methods:

  • A multicentre prospective study involving 100 CF patients undergoing TIVAD insertion.
  • Colour-Doppler-ultrasound (Doppler-US) performed at 1 and 6 months post-insertion.
  • Blood samples collected at insertion and at 1 and 6 months for thrombophilia assessment.

Main Results:

  • 50% of patients exhibited thrombophilia abnormalities or hypercoagulability.
  • The frequency of catheter venous thrombosis was low at 6.6%.
  • 90 out of 100 patients completed the 6-month follow-up.

Conclusions:

  • Routine biological screening before TIVAD insertion in CF patients is not supported by current data.
  • A history of venous thromboembolism and prospective Doppler-US are crucial for identifying asymptomatic CVT.
  • Patients identified with CVT may benefit from further biological screening and anticoagulant therapy.
Abstract

Keywords:
Cystic fibrosisThrombophiliaThrombosis

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