Adults with sickle cell disease can develop pulmonary hypertension from a multitude of etiologies. Classified as WHO Group 5, there are no therapies approved for the treatment of sickle cell disease-pulmonary hypertension. Thromboembolic disease is prevalent in sickle cell disease and can lead to pulmonary hypertension. The only approved medical therapy for chronic thromboembolic pulmonary hypertension is riociguat. We report the experience, safety and tolerability of riociguat use in a series of sickle cell disease patients with chronic thromboembolic pulmonary hypertension.
Patients were surveyed to assess the impact of dopamine transporter imaging on diagnostic confidence, change in treatment plan, effect on medication compliance, and subjective well-being. Surveys were sent to 140 patients who completed dopamine transporter imaging an average of 18 months prior. Sixty-five surveys from patients (46%) were returned. Questions assessed patients’ perceived impact of the imaging on their care. Increased diagnostic confidence following imaging was reported by 69% of patients. Changes to treatment plan from imaging were reported by 24% of patients. Overall satisfaction with the study and its impact was reported by 70% of patients. Dopamine transporter imaging increased diagnostic confidence among patients and overall patient satisfaction with the impact of imaging on clinical care was high.
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