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What coagulation (blood clotting) problems may the patients with congenital cyanotic heart disease (blue babies) have and how are they treated?

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I checked and found following coagulation problems may occur in patients with congenital cyanotic heart diseases (commonest example tetralogy of Fallot):

Thrombotic episodes may occur due to:

  • Hyperviscocity due to markedly elevated haematocrit (red cell mass), which in turn occurs due to chronic hypoxia and cyanosis

  • Iron deficiency leading to spherical shape and reduced deformability of red blood cells

Bleeding episodes can occur due to:

  • Low platelet count (thrombocytopenia)

  • Acquired Von Willebrand syndrome

  • Vitamin K deficiency leading to coagulation factor deficiency

Hence patients with congenital cyanotic heart disease are at risk of both bleeding (from example from lungs- hemoptysis) and thrombosis (e.g. in brain vessels- stroke). Both above problems are more common in patients with severe hypoxia and cyanosis.

Above abnormalities can be treated using following modalities:

  • Platelet transfusion

  • Fresh frozen plasma / coagulation factor concentrates

  • Iron replacement if iron deficiency is documented. Serum ferritin is most sensitive test to document iron stores. Reduced mean corpuscular volume (MCV) indicates microcytosis due to iron deficiency anemia.

  • Vitamin K replacement

  • Temporary improvement occurs with phlebotomy (blood letting) combined with volume replacement using isotonic fluids.

  • Palliative surgical procedures like Blalock-Taussig shunt improves hypoxia by increasing pulmonary (lung) blood flow and reduces most of above abnormalities.

However, unwarranted iron supplementation or vitamin K replacement may lead to thrombotic episodes.

References:

http://emedicine.medscape.com/article/2035949-workup

http://archsurg.jamanetwork.com/article.aspx?articleid=565545

http://www.ncbi.nlm.nih.gov/pubmed/12486661

http://www.jpma.org.pk/full_article_text.php?article_id=4303

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