The Effectiveness of Long-term Inhaled lloprost in Addition to Oral Sildenafil Treatment in Severe Pulmonary Hypertension Associated with Sickle Cell Disease

Pulmonary arterial hypertension (PAH) is a common complication of sickle cell disease (SCD). A 21 year-old female patient with SCD was presented with dyspnea and palpitation during slight effort. Pulmonary arterial systolic pressure (PASP) was 115mmHg on echocardiogram. No improvement of symptoms was achieved with the classical treat­ment and oral sildenafil was given. In the second week of therapy, PASP decreased to 75mmHg. She was presented with chest pain, left shoulder pain and dyspnea six months later. PASP was 125mmHg. No improvement was achieved with the treatment and inhaled iloprost was added to sildenafil treatment. PASP decreased to 65mmHg.