Abstract
Background : Sildenafil, an oral phosphodiesterase type-5 inhibitor, has vasodilatory effects through a cyclic guanosine 3,5-monophosphate–dependent mechanism, whereas beraprost, an oral prostacyclin analog, induces vasorelaxation through a cAMP-dependent mechanism. This combination has often used but there was little detailed study on it Objectives : To investigate whether the combination of oral sildenafil and beraprost is superior to sildenafil alone in in adult patients with Pulmonary Arterial Hypertention (PAH) related uncorrected secundum Atrial Septal Defect (ASD). Methods : Patients with secundum ASD who developed PAH divided into two group. Group A received oral sildenafil 3x40 mg and oral beraphrost 3x20 mcg. Group B received oral sildenafil only 3x40 mg in a 12-week. Health-related quality of life (HRQoL) was recorded by patients using the Medical Outcomes Study 36-item short form (SF-36) questionnaires at baseline and after 12 of therapy. Therapy adherence was achieved through a series of phone calls and a four-weekly hospital visit. Every normal follow-up appointment included an examina- tion of side effects and a dosage modification based on the clinical situation Results: We didn’t found any significant of proportion different in cofounding factor between groups. Compared with Group B, Group A had better functional capacity, limitation to physical health, energy fatigue, pain, and health change (P=0.00, P=0.03, P=0.044, P=0.026, P=0.008, respectively). Conclusion: Combination between oral sildenafil therapy 40 mg three times per day and beraphrost 20 mcg two times per day significantly increase the HRQoL in PAH patients in uncorrected secundum ASD compared sildena- fil alone
Highlights
Pulmonary arterial hypertension (PAH) is a term used to classify a variety of conditions that have in common an injury to the pulmonary vasculature that produces elevations in pulmonary arterial pressure
Adult patients with Pulmonary Arterial Hypertention (PAH) and uncorrected secundum Atrial Septal Defect (ASD) who had enrolled on the Saiful Anwar-PH registry and signed the informed consent form were included in this study
There were significant increasing for quality of live in group A compared with group B, there were significant different in physical functioning, Limitation to physical health, Energy fatigue, Pain, and health change (P=0.00, P=0.03, P=0.044, P=0.026, P=0.008, respectively)
Summary
Pulmonary arterial hypertension (PAH) is a term used to classify a variety of conditions that have in common an injury to the pulmonary vasculature that produces elevations in pulmonary arterial pressure. PAH is a common (9–35%) consequence of congenital heart disease that primarily affects patients with a left-to-right shunt, including an atrial septal defect (ASD) that has or has not been repaired. It’s impact on physical mobility and mental state that might deteriorate the patients’ health related quality of life (HRQoL).[4] HRQoL is an indicator of personal satisfaction with one's life that is influenced by one's health condition, such as physical stamina, learning function, working relationships, emotional well-being, and spirituality. Sildenafil, an oral phosphodiesterase type-5 inhibitor, has vasodilatory effects through a cyclic guanosine 3,5-monophosphate–dependent mechanism, whereas beraprost, an oral prostacyclin analog, induces vasorelaxation through a cAMP-dependent mechanism This combination has often used but there was little detailed study on it Objectives : To investigate whether the combination of oral sildenafil and beraprost is superior to sildenafil alone in in adult patients with Pulmonary Arterial Hypertention (PAH) related uncorrected secundum Atrial Septal Defect (ASD). Conclusion: Combination between oral sildenafil therapy 40 mg three times per day and beraphrost 20 mcg two times per day significantly increase the HRQoL in PAH patients in uncorrected secundum ASD compared sildenafil alone
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