Coagulation problems in blue babies

Question

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

Answer 1

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