title: Role of thrombophilia factors in acute systemic-pulmonary shunt obstruction.
abstract: BACKGROUND
  Systemic-pulmonary shunts are widely used in initial palliation in cyanotic congenital heart disease. The incidence and the relationship between acute shunt obstruction and thrombophilia are not precisely defined. The aim of this study was to determine the frequency of shunt obstruction in the early postoperative period, and to define the frequency and presence of thrombophilia factors in patients treated for acute shunt thrombosis.
  
  
  METHODS
  Between October 2010 and October 2012, 77 patients who had systemic-pulmonary shunt operation were included in this prospective study. Patients who developed shunt obstruction were examined in terms of inherited and acquired thrombophilia factors.
  
  
  RESULTS
  Median patient age was 61 days and median weight was 4.3 kg. Thirty-three patients were neonates. Diameter of the Gore-Tex grafts used for the shunt ranged from 3 mm to 5 mm. Acute shunt occlusion rate was 10% (8/77), and all of these occurred in the first 24 h. Thrombophilia was found in three of eight patients who underwent intervention (surgical and/or transcatheter) due to shunt thrombosis (presence of anti-phospholipid antibodies, n = 1; protein C deficiency, n = 1; and factor V Leiden mutation, n = 1) and only one patient died.
  
  
  CONCLUSIONS
  Acute shunt obstruction developed in 10% of patients who underwent systemic-pulmonary shunt, and emergency surgery or transcatheter intervention can be life saving in this context. Acute shunt obstruction can occur due to mechanical and hemodynamic problems, but clinicians should also consider and evaluate thrombophilia factors.
authors.affiliation: Departments of Pediatric Cardiology, Mehmet Akif Ersoy Cardiovascular Research and Training Hospital, Istanbul, Turkey.
authors.forename: Yakup
authors.initials: Y
authors.lastname: Ergul
authors.affiliation: Departments of Pediatric Cardiology, Mehmet Akif Ersoy Cardiovascular Research and Training Hospital, Istanbul, Turkey.
authors.forename: Neslihan
authors.initials: N
authors.lastname: Kiplapinar
authors.affiliation: Departments of Pediatric Cardiology, Mehmet Akif Ersoy Cardiovascular Research and Training Hospital, Istanbul, Turkey.
authors.forename: Ibrahim Cansaran
authors.initials: IC
authors.lastname: Tanidir
authors.affiliation: Departments of Pediatric Cardiology, Mehmet Akif Ersoy Cardiovascular Research and Training Hospital, Istanbul, Turkey.
authors.forename: Erkut
authors.initials: E
authors.lastname: Ozturk
authors.affiliation: Departments of Pediatric Cardiology, Mehmet Akif Ersoy Cardiovascular Research and Training Hospital, Istanbul, Turkey.
authors.forename: Alper
authors.initials: A
authors.lastname: Guzeltas
authors.affiliation: Department of Pediatric Cardiovascular Surgery, Mehmet Akif Ersoy Cardiovascular Research and Training Hospital, Istanbul, Turkey.
authors.forename: Sertac
authors.initials: S
authors.lastname: Haydin
authors.affiliation: Department of Pediatric Hematology and Oncology, Kanuni Sultan Süleyman Children's Hospital, Istanbul, Turkey.
authors.forename: Arzu
authors.initials: A
authors.lastname: Akcay
authors.affiliation: Department of Pediatric Cardiovascular Surgery, Mehmet Akif Ersoy Cardiovascular Research and Training Hospital, Istanbul, Turkey.
authors.forename: Ersin
authors.initials: E
authors.lastname: Erek
authors.affiliation: Department of Pediatric Cardiovascular Surgery, Mehmet Akif Ersoy Cardiovascular Research and Training Hospital, Istanbul, Turkey.
authors.forename: Mehmet
authors.initials: M
authors.lastname: Yeniterzi
authors.affiliation: Departments of Pediatric Cardiology, Mehmet Akif Ersoy Cardiovascular Research and Training Hospital, Istanbul, Turkey.
authors.forename: Ender
authors.initials: E
authors.lastname: Odemis
authors.affiliation: Department of Pediatric Cardiovascular Surgery, Mehmet Akif Ersoy Cardiovascular Research and Training Hospital, Istanbul, Turkey.
authors.forename: Ihsan
authors.initials: I
authors.lastname: Bakir
mesh_terms: D000714:Anastomosis, Surgical / Q000009:adverse effects; D004632:Emergency Medical Services; D005240:Feasibility Studies; D005260:Female; D005500:Follow-Up Studies; D006083:Graft Occlusion, Vascular / Q000453:epidemiology / Q000209:etiology*; D006801:Humans; D015994:Incidence; D007231:Infant, Newborn; D008297:Male; D011446:Prospective Studies; D011651:Pulmonary Artery / Q000002:abnormalities / Q000601:surgery*; D012189:Retrospective Studies; D013348:Subclavian Artery / Q000601:surgery*; D019851:Thrombophilia / Q000150:complications*; D013927:Thrombosis / Q000453:epidemiology / Q000209:etiology*; D014421:Turkey / Q000453:epidemiology
keywords: cardiac surgery; children; systemic-pulmonary shunt; thrombophilia; thrombosis