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Cyanotic congenital heart disease (CCHD) with symptomatic erythrocytosis

Shelonitda S Rose1, Ashish A Shah, Donald R Hoover

  • 1Division of Hematology, Department of Medicine, UMDNJ/Robert Wood Johnson Medical School, New Brunswick, NJ, USA. rosesh@umdnj.edu

Journal of General Internal Medicine
|October 6, 2007
PubMed

Insights

Secondary erythrocytosis in cyanotic congenital heart disease (CCHD) differs from polycythemia vera (PV). Hydration and iron therapy, not phlebotomy, effectively treat CCHD-related erythrocytosis symptoms.

Area of Science:

  • Cardiology
  • Hematology
  • Internal Medicine

Background:

  • Cyanotic congenital heart disease (CCHD) can cause secondary erythrocytosis.
  • Current practice often involves phlebotomy for CCHD patients with elevated hematocrit, aiming for levels below 65%.

Observation:

  • A 21-year-old female with CCHD presented with symptomatic erythrocytosis (hemoglobin 25.2 g/dl, hematocrit 75.8%), experiencing numbness and tingling.
  • Her symptoms resolved with intravenous hydration, suggesting hyperviscosity symptoms were related to dehydration and possibly iron deficiency.

Findings:

  • Unlike polycythemia vera (PV), a direct association between elevated hematocrit and thrombosis in CCHD is not established.
  • The patient's symptoms improved with volume replacement and low-dose iron therapy, indicating these as appropriate treatments.

Implications:

  • Phlebotomy in CCHD patients may lead to iron deficiency, microcytosis, and potentially increased blood viscosity, exacerbating cerebrovascular accident risk.
  • Treatment for symptomatic erythrocytosis in CCHD should focus on hydration and iron management rather than phlebotomy.

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