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This patient has cyanotic congenital heart disease with Eisenmenger syndrome. Patients with Eisenmenger syndrome usually have hemoglobin levels of 18-20 g/dL and a hematocrit level > 65%. In this patient, the level is markedly reduced since she has cyanotic congenital heart disease. Her decrease in hemoglobin/hematocrit is most likely associated with menorrhagia, and her resulted in fatigue and exertional dyspnea due to reduced oxygen-carrying capacity being that she is cyanotic. The treatment of choice, unless volume-depleted, would be low-dose iron therapy (325mg/day) for 7-10 days and a re-evaluation of her Hb/Hct afterwards. If they have increased, iron therapy should be discontinued.

Blood transfusion is required if the hemoglobin level is less than 10 g/dL, has severe symptoms related to the anemia, in the presence of excessive bleeding, or at the time of surgery and expected blood loss.

Treatment options for patients with this syndrome include lung transplantation with intracardiac repair and pulmonary vasodilator therapy. Pulmonary vasodilators can improve symptoms, but would not be the treatment of choice in this patient until the hemoglobin is corrected. If symptoms persist after correcting the anemia, then pulmonary vasodilators may benefit this patient. Another option would be heart-lung transplantation.

Observation and follow-up is not appropriate.

Key Point

In a patient with Eisenmenger Syndrome and anemia, the treatment of choice is low dose iron therapy and reassessment of the Hb/Hct after 7-10 days.

Reference:  ACC/AHA 2008 Guidelines for the Management of Adults with Congenital Heart Disease.

A 52 year old lady with unrepaired congenital heart disease and chronic cyanosis presents with new onset fatigue and exertional dyspnea. She has a ventricular septal defect with Eisenmenger syndrome. The remainder of her history is significant for menorrhagia.

On examination, her BP is 121/68, HR 72 bpm and regular, JVP has prominent a wave, nondisplaced PMI, with a +2 parasternal pulse. She has an accentuated pulmonic S2; no gallops/canters are appreciated. There is a soft murmur at the LSB and an ejection click a the 2nd LIS. There is no diastolic murmur. Her carotid exam is normal. Cyanosis and digital clubbing is present.

Her lab results are significant for a hematocrit of 48%, hemoglobin 13.5 g/dL, sCr 1.3 mg/dL, and a normal fasting plasma glucose.

Her EKG demonstrates right axis deviation and RVH. The CXR reveals prominent central pulmonary arteries and decreased vascularity in the lung fields.

Which treatment is most appropriate at this time?