Journal of Rare Cardiovascular Diseases

Health-related QoL (HRQoL) is defined as ‘‘the functional effect of an illness and its consequent therapy upon a patient, as perceived by the patient’’ and is a reflection of one’s health on physical, psychological, and social aspects of life. PAH is a condition associated with symptoms that may severely affect patient’s QoL. Awareness of having a rare disease of poor prognosis, with limited therapeutic options and symptoms restricting daily activities may cause anxiety, panic, depression, anger, and hopelessness. Current European Society of Cardiology guidelines suggest that psychosocial support should be considered in patients with PAH (with recommendation class IIa and level of evidence C). PAH is a progressive disease, leading to a gradual reduction in exercise tolerance and significantly decreasing the HRQoL. A recent development of PAH-specific therapies has significantly improved patients’ survival and rate of clinical deterioration. It may be useful to consider patient’s personal outcomes in predicting treatment benefits and decision making. JRCD 2014; 2 (1): 5–8

physical activity, QoL, questionnaire, depression, CAMPHOR

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