Erythrocytosis and iron status in Eisenmenger syndrome: an illustrative case study

Abstract

Abstract Background Patients with Eisenmenger syndrome are chronically hypoxaemic and should therefore mount a secondary erythrocytosis. This response can be attenuated by iron deficiency. Historically, patients with Eisenmenger-associated erythrocytosis often underwent venesection but recent data have challenged this practice. Case presentation An illustrative case of a 30-year-old female with Eisenmenger syndrome secondary to a ventricular septal defect is discussed. Her resting saturations on room air were 84%. She was receiving pulmonary arterial hypertension targeted therapy with sildenafil 25 mg three times a day and bosentan 125 mg twice daily. Her local haematologist was planning on therapeutic venesection as her haematocrit was elevated at 0.57. Her haemoglobin was 16.7 g/dl, ferritin levels were 15 μg/L and transferrin saturations were 10.5%. What are the indications for venesection? Should she receive iron supplementation instead? Data to help guide decision-making are reviewed and a clinical approach is suggested. Conclusions Iron status should be regularly checked in Eisenmenger syndrome patients and replaced appropriately. There is no role for routine venesection in patients with Eisenmenger syndrome; this should be reserved for the small proportion of patients with symptoms of hyperviscosity in the absence of dehydration.