A case of pulmonary hypertension associated with a rare congenital hemolytic anemia

We present a case of a patient with pulmonary hypertension (PHT) associated with a rare congenital hemolytic anemia secondary to a red blood cell fragility disorder. The clinical diagnosis was determined after an extensive negative evaluation for iron overload, cirrhosis, or portal hypertension. Treatment with oral sildenafil in addition to standard therapy, resulted in a significant clinical improvement from New York Heart Association (NYHA) class IV to NYHA class II symptoms. This case provides further evidence of PHT occurring in association with chronic hemolytic disorders, and a potential role for sildenafil therapy.