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Title of Journal: J GEN INTERN MED

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Abbravation: Journal of General Internal Medicine

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Springer-Verlag

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DOI

10.1002/maco.201470073

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1525-1497

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Cyanotic Congenital Heart Disease CCHD with Symp

Authors: Shelonitda S Rose Ashish A Shah Donald R Hoover Parvin Saidi
Publish Date: 2007/10/05
Volume: 22, Issue: 12, Pages: 1775-1777
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Abstract

Secondary erythrocytosis of cyanotic congenital heart disease CCHD is pathologically different from primary erythrocytosis of polycythemia vera PV An association between elevated hematocrit and thrombosis has been established in PV patients and treatment guidelines recommend maintaining hematocrit 45 Although an association between elevated hematocrit and thrombosis has not been established in CCHD and secondary erythrocytosis the current clinical practice is to phlebotomize these patients to hematocrit 65 We report a 21yearold woman with CCHD who presented with symptomatic erythrocytosis with numbness and tingling with hemoglobin 252 g/dl and hematocrit 758 Her symptoms resolved with IV hydration Other factors including dehydration and iron deficiency may precipitate hyperviscosity symptoms The treatment is volume replacement and lowdose iron therapy not phlebotomy Repeated phlebotomy causes iron deficiency with microcytic erythrocytes which increases the whole blood viscosity and therefore can potentially accentuate rather than decrease the risk for a cerebrovascular accident

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