Studies assessing the quality of life (QoL) in patients with pulmonary arterial hypertension (PAH)

First author [reference]Sample sizePatient characteristicsTools used to assess QoLDomains measuredResultsComments
McCollister [24]n=55 in three study phases: n=25 in phase 1, n=20 in phase 2 and n=10 in phase 3
  • All PAH, WHO FC I–IV

Three-phase study: a concept-elicitation phase; second phase of two rounds of cognitive interviews; a final cognitive and usability interview phaseQuestionnaire assesses two domains: symptoms of PAH and impact of these symptoms on patients' lives
  • Development of the PAH-SYMPACT questionnaire, which was shown to capture symptoms and its impact

Chin et al. [23] validated the PAH-SYMPACT as the first disease-specific patient-reported outcome instrument
Taichman [4]Total n=155; completed n=55One-third had IPAH, WHO FC II/IIISF-36
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=27642% with IPAHLASA QoL items
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=53IPAH, SSc-PAH and anorexigen-related PAH
72% in NYHA FC III or IV
NHP questionnaire
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=10050% had IPAH
38% had WHO FC II and 62% had FC III
PHQ-8PHQ-8: lack of interest, feeling of depression, energy, sleep, appetite, feeling of guilt, lack of concentration, psychomotor agitation or retardationPHQ-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.