Studies assessing the quality of life (QoL) in patients with pulmonary arterial hypertension (PAH)
| First author [reference] | Sample size | Patient characteristics | Tools used to assess QoL | Domains measured | Results | Comments |
| McCollister [24] | n=55 in three study phases: n=25 in phase 1, n=20 in phase 2 and n=10 in phase 3 |
| Three-phase study: a concept-elicitation phase; second phase of two rounds of cognitive interviews; a final cognitive and usability interview phase | Questionnaire assesses two domains: symptoms of PAH and impact of these symptoms on patients' lives |
| Chin et al. [23] validated the PAH-SYMPACT as the first disease-specific patient-reported outcome instrument |
| Taichman [4] | Total n=155; completed n=55 | One-third had IPAH, WHO FC II/III | SF-36 SGRQ | SF-36 assesses physical function, bodily pain, general health, vitality and social, functional and mental health SGRQ assesses symptoms, activity and impacts (psychosocial), as well as total score | SF-36: impaired QoL in every domain SGRQ: abnormally elevated scores (indicating a worse QoL) were seen in assessments of patient symptoms, activity and the impact of disease | These scores indicate a poor physical function, mental health, increased body pain and decreased general and social wellbeing; all correlating with a lower QoL No correlation was observed between haemodynamic measurements and QoL scores |
| Swetz [11] | Total n=276 | 42% with IPAH | LASA QoL items CAMPHOR | LASA assesses physical, spiritual, emotional, intellectual and overall wellbeing CAMPHOR assesses energy, breathlessness, mood, total symptoms, function and QoL | LASA: 40% patients had score ≤5 (0 being the worst and 10 the best) CAMPHOR score centred around 50th percentile on each scale for QOL | Patients with PAH have large symptom burden that affects QoL and persist even with PAH treatment |
| Shafazand [3] | Total n=53 | IPAH, SSc-PAH and anorexigen-related PAH 72% in NYHA FC III or IV | NHP questionnaire CHQ HADS | NHP: physical mobility, pain, sleep, social isolation, emotional reactions and energy CHQ: dyspnoea, fatigue, emotional function and mastery HADS: screen for anxiety and depression | NHP: moderate to severe impairment in all domains CHQ: moderate impairment in all domains assessed HADS: moderate or severe levels of anxiety and depression were reported by 20.5% and 7.5% of participants, respectively | Impairment in multiple QoL domains, although the anxiety and depression scores were within the range of normal responses |
| McCollister [10] | Total n=100 | 50% had IPAH 38% had WHO FC II and 62% had FC III | PHQ-8 | PHQ-8: lack of interest, feeling of depression, energy, sleep, appetite, feeling of guilt, lack of concentration, psychomotor agitation or retardation | PHQ-8 scores showed that 15% of patients had score of ≥10, suggestive of major depression 40% had score of between 4 and 9, corresponding to mild to moderate depressive symptoms | Higher prevalence of depression disorders in PAH patients than those found in general population, patients with other medical disorders and patients with left heart failure |
WHO: World Health Organization; FC: functional class; PAH-SYMPACT: Pulmonary Arterial Hypertension-Symptoms and Impact; IPAH: idiopathic PAH; SF-36: 36-item Short Form Health Survey; SGRQ: St George's Respiratory Questionnaire; LASA: Linear Analogue Self-Assessment; CAMPHOR: Cambridge Pulmonary Hypertension Outcome Review; SSc: systemic sclerosis; NYHA: New York Heart Association; NHP: Nottingham Health Profile; CHQ: Congestive Heart Failure Questionnaire; HADS: Hospital Anxiety and Depression Scale; PHQ-8: Patient Health Questionnaire.