: Coagulation problems in blue babies 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:
emedicine.medscape.com/article/2035949-workup archsurg.jamanetwork.com/article.aspx?articleid=565545 www.ncbi.nlm.nih.gov/pubmed/12486661 www.jpma.org.pk/full_article_text.php?article_id=4303