Cyanotic Congenital Heart Disease (CCHD) with Symptomatic Erythrocytosis

Left lower Limb Gangrene Following Diarrhoeal Disease and Dehydration in a child with Transposition of the Great Arteries

“…It is of note that the role of elevated haematocrit as a sole risk factor for thrombosis in patients with cyanotic CHD or secondary polycythemia has recently been questioned. 9,13,14 Unlike in patients with polycythemia vera, current literature showed no direct correlation between increased hematocrit level and risk for thrombogenesis. 14 Other workers have reported peripheral gangrene resulting due to other causessuch as septicaemia and DIC.…”

“…There are many factors that could potentially lead to development of thrombosis and even gangrene in children with cyanotic CHD. 2,9 These include polycythemia, infective endocarditis, central embolism, sepsis, dehydration and DIC which may either be induced by infection or immunemediated reactions. 2,8 In our patient, dehydration was presumed to be the most likely precipitating factor in view of the temporal relation between the onset of diarrhoea and the occurrence of limb gangrene.…”

“…Polycythemia is a common complication in patients with cyanotic CHD, and occurs as a physiologic response to tissue hypoxia, which induces increased bone marrow erthythropoisis. 1,9 Polycythemia can precipitate thrombosis especially when there is associated hyper viscosity due to dehydration or iron deficiency; or in the presence of other risk factors such as sepsis and DIC. 1,5,8 Though our patient's hematocrit level was only moderately elevated, she still developed peripheral gangrene most likely because of the associated dehydration, which served as an additional precipitating risk factor.…”

Cardioembolic Stroke in Adults With a History of Congenital Heart Disease

“…Although phlebotomy is routinely used to prevent hyperviscosity for other diseases with erythrocytosis, in CHD it can cause iron deficiency with microcytic erythrocytes and secondary spherocytosis, which exacerbate viscosity and stroke risk. [6][7][8][9] Hydroxyurea has been tried as an alternative to phlebotomy, but it is not routinely used. 6 …”

Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: General management for pulmonary hypertension

“…[15] Overuse of phlebotomy can be hazardous,[16] but unfortunately continues to be practiced by many treating physicians.…”