Elsevier

The Journal of Pediatrics

Volume 76, Issue 2, February 1970, Pages 221-230
The Journal of Pediatrics

Original article
Hemostasis in cyanotic congenital heart disease**

https://doi.org/10.1016/S0022-3476(70)80166-4Get rights and content

Coagulation studies were performed in 28 children with cyanotic congenital heart disease in an effort to demonstrate intravascular coagulation. Thrombocytopenia and prolonged bleeding time were most common; hypofibrinogenemia, accelerated fibrinolysis, and prolongation of the prothrombin time occurred less frequently. The number of hemostatic defects present in any patient was related to the severity of polycythemia. The pattern of hemostatic abnormalities did not indicate that decompensated intravascular coagulation was the responsible underlying mechanism. Partially compensated intravascular coagulation also did not appear to be responsible, as heparin therapy did not correct the hemostatic defects in one patient, and the half-life of I125 fibrinogen was normal in another. The presence of preoperative hemostatic defects correlated with the severity of postoperative hemorrhage. Correction of the defects by replacement therapy was effective in the prevention and treatment of excessive bleeding when surgical complications were not present. Incontrovertible evidence of intravascular coagulation seems necessary before heparin therapy can be recommended for the treatment of hemorrhage in patients with cyanotic congenital heart disease.

References (30)

  • SommervilleJ. et al.

    Postoperative haemorrhage and related abnormalities of blood coagulation in cyanotic congenital heart disease

    Brit. Heart J.

    (1965)
  • GrossS. et al.

    The platelets in cyanotic congenital heart disease

    Pediatrics

    (1968)
  • Favre-GillyJ. et al.

    Un trouble inattendu de la coagulation dans la maladie bleue: L'Hypoprothrombinemie; son importance en clinique et dans le problems de l'hemostase au cours des operations de Blalok et de Potts

    Sang (Par)

    (1951)
  • AlagilleD. et al.

    Les thrombopathies associees aux cardiopathies congenitales; etude de l'hemostase dans cinquante cas

    Rev Franc. Clin. Biol.

    (1958)
  • ThurnherrN.

    Blood coagulation studies in extracorporeal circulation in man

    Thromb. Diath. Haemorrh.

    (1967)
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      Although major causes of postoperative bleeding in pediatric cardiac surgery are thrombocytopenia, platelet dysfunction, and hemodilution, one possible cause is increased fibrinolysis during CPB [20, 21], which occurs in about 16% of patients [22]. Moreover, congenital heart disease itself in pediatric patients has been shown to be associated with fibrinolysis [22, 23]. We conducted a retrospective study to assess the benefit of TXA in pediatric cardiac surgery in a single-center population, with a good number of participants (231 patients) in relation to the literature, in which cyanotic (123) and acyanotic (108) patients were discretely balanced (1:13 ratio).

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    **

    Supported by Grant No. 425 from the Los Angeles and Riverside County Heart Associations, Medical Research Committee of Delta Delta Delta, and United States Public Health Service Grant No. FR-86 from the National Institutes of Health

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