Isolated ultrafiltration in refractory heart failure on patients with Cooley's anemia

Dilatative cardiomyopathy is a late complication of Cooley's anemia resulting in severe heart failure resistant to conventional medical treatment. Anemia, iron and fluid overload are the main causes of this disease entity. Four male patients (mean age 22.3 +/- 5.4 years) with Cooley's anemia associated heart failure--4th NYHA and fluid overload > 20% body weight--resistant to medical treatment, underwent isolated ultrafiltration (IU). During 90 minutes of treatment an average of 1750 +/- 850 ml of fluid were removed using AN69S plate membrane. Each patient received an average of 7 IU sessions (range 4-13). Cardiac function was assessed before and after each session with echocardiography, venous cardiac catheterization and impedence cardiography. Transient cardiac improvement was observed after each IU session: left ventricular systolic diameter decreased, as did central venous pressure and pulmonary wedge pressure, whereas cardiac index increased. All patients died of unrelated causes. In conclusion IU treatment in dilatative cardiomyopathy resistant to conventional medical treatment in Cooley's anemia patients produces only transient cardiac improvement, but no improvement in overall survival.