Thromb Haemost 2017; 117(06): 1199-1207
DOI: 10.1160/TH16-07-0529
Stroke, Systemic or Venous thromboembolism
Schattauer GmbH

Pulmonary embolism and in situ pulmonary artery thrombosis in paediatrics

A systematic review
Madhvi Rajpurkar
1  The Carman and Ann Adams Department of Pediatrics, Division of Hematology Oncology, Wayne State University School of Medicine, Children’s Hospital of Michigan, Detroit, Michigan, USA
,
Tina T. Biss
2  Department of Haematology, The Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK
,
Ernest K. Amankwah
3  Department of Oncology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
4  Clinical and Translational Research Organization, Johns Hopkins All Children’s Hospital, St. Petersburg, Florida, USA
,
Denise Martinez
4  Clinical and Translational Research Organization, Johns Hopkins All Children’s Hospital, St. Petersburg, Florida, USA
,
Suzan Williams
5  Division of Haematology/Oncology, Department of Paediatrics, Hospital for Sick Children, Toronto & University of Toronto,Toronto, Ontario, Canada
,
C. Heleen van Ommen
6  Department of Pediatric Hematology, Erasmus Medical Center Sophia Children’s Hospital, Rotterdam, the Netherlands
,
Neil A. Goldenberg
4  Clinical and Translational Research Organization, Johns Hopkins All Children’s Hospital, St. Petersburg, Florida, USA
7  Division of Hematology, Department of Pediatrics, Johns Hopkins University School of Medicine and Johns Hopkins Medicine Pediatric Thrombosis Program, St. Petersburg, Florida and Baltimore, Maryland, USA
› Author Affiliations
Further Information

Publication History

Received: 14 July 2016

Accepted after major revision: 01 March 2017

Publication Date:
28 November 2017 (online)

Summary

Data on paediatric pulmonary embolism (PE) are scarce. We sought to systematically review the current literature on childhood PE and conducted a search on paediatric PE via PubMed (1946–2013) and Embase (1980–2013). There was significant heterogeneity in reported data. Two patterns were noted: classic thromboembolic PE (TE-PE) and in situ pulmonary artery thrombosis (ISPAT). Mean age of presentation for TE-PE was 14.86 years, and 51% of cases were males. The commonest method for diagnosis of TE-PE was contrast CT with angiography (74% of patients). The diagnosis of TE-PE was often delayed. Although 85% of children with TE-PE had an elevated D-dimer at presentation, it was non-discriminatory for the diagnosis. In paediatric TE-PE, the prevalence of central venous catheters was 23%, immobilisation 38%, systemic infection 31% and obesity 13%, elevated Factor VIII or von Willebrand factor levels 27%, Protein C deficiency 17%, Factor V Leiden 14% and Protein S deficiency 7%. In patients with TE-PE, pharmacologic thrombolysis was used in 29%; unfractionated heparin was the most common initial anticoagulant treatment in 64% and low-molecular-weight heparins the most common follow-up treatment in 83%. Duration of anticoagulant therapy was variable and death was reported in 26% of TE-PE patients. In contrast to TE-PE, patients with ISPAT were not investigated systematically for presence of thrombophilia, had more surgical interventions as the initial management and were often treated with anti-platelet medications. This review summarises important data and identifies gaps in the knowledge of paediatric PE, which may help to design future studies.