Table 3

Summary of studies

StudyCountryAimN% femaleAge (mean unless stated) and rangeEthnicityYears since diagnosis (mean unless stated) and rangeWHO classification of PH described by authorsWHO functional classData collection and analysis
Alami et al 26 FranceTo explore adult’s experiences of PH and identify potential improvements in care166349
24–75		NRNR75%=idiopathic PAH
25%=heritable PAH		I=0%
II=38%
III=50%
IV=12%		Semi-structured interviews; grounded theory
Armstrong et al 28*UKTo investigate participant’s experiences of being diagnosed with PH306056.3
26–80		NR<1 to >10Group 1 PAH (86%)
37%=idiopathic PAH
23%=connective tissue disease-associated PAH
17%=congenital heart disease-associated PAH
3%=portopulmonary hypertension
3%=heritable PAH
3%=drugs/toxins-associated PAH
Group 3 PH-lung disease (3%)
Group 4 CTEPH (4%)
NR (3%)		I=0%
II=30%
III=63%
IV=7%		Semi-structured interviews; thematic analysis
Carroll et al 24 USATo understand the motivations of why patients with PH partake in randomised controlled trials2685Median 56
25th percentile 41
75th percentile 61		65% white,
19% black, 15% Hispanic		Median 5.5
25th percentile 4
75th percentile 11		Group 1 PAH (85%)
50%=idiopathic PAH
23%=connective tissue-associated PAH
4%=heritable PAH
4%=congenital heart disease-associated PAH
4%=portopulmonary
Other/unknown 15%		I–III=100%Randomised participants to review a hypothetical randomised controlled trial that did or did not allow continuation of pre-existing PAH therapies followed by semi-structured interviews; thematic analysis
Chiang et al 30 TaiwanTo explore treatment experiences in individuals with PH177653.6
27–84		NR6.4
2–15		Form of PH not specifiedNRSemi-structured interviews; thematic analysis
Flattery et al 20 USATo describe patients experiences of PAH and explore the impact117358.5
40–72		‘Most were white’ (p. 101)3.5
0.5–14		Group 1 PAH
55%=idiopathic PAH
18%=systemic lupus erythematosis-associated PAH
9%=mixed connective tissue disease
9%=portopulmonary hypertension;
9%=other PAH
Not specified (45%)		NRSemi-structured interviews; Colaizzi’s approach to phenomenological analysis
Goddard et al 21 UKTo use narrative analysis to explore the impact of PH125020–79NRNRGroup 1 PAH (100%)NRUnstructured narrative interview; narrative analysis
Hall et al 31 CanadaTo describe patient’s experiences of adjusting to PH and continuous intravenous epoprostenol77155
43–70		NR3
0.5–7		Group 1 PAH (100%)NRSemi-structured interviews; constant comparison method
Ivarsson, Ekmehag B27+SwedenTo describe patient’s experience of information provision in PH177656
28–73		NR5
1–12		Group 1 PAH (71%)
35%=idiopathic PAH
24%=systemic sclerosis-associated PAH
12%=PAH
Group 4 CTEPH (29%)
11% treated by pulmonary endarterectomy		NRSemi-structured interviews; content analysis
Ivarsson, Ekmehag B34+SwedenTo describe patient’s experiences of support in PH177656
28–73		NR5
1–12		Group 1 PAH (71%)
35%=idiopathic PAH
24%=systemic sclerosis-associated PAH
12%=PAH
Group 4 CTEPH (29%)
11% treated by pulmonary endarterectomy		NRSemi-structured interviews; content analysis
Keen et al 6 UKTo explore the nature of physiotherapy for PH in patients36744–66NR8–10Pulmonary hypertension Breakdown of classification not specifiedNRSemi-structured interviews; framework analysis
Kingman et al 29 International study—Europe, Asia, North America, South AmericaTo examine patient’s perspectives of PH, including impact of living with PH, management and treatment397519–91NRNRGroup 1 PAH (87%)
Group 4 CTEPH (13%)		I=5%
II=36%
III=44%
IV=15%		Video of participants in their home, field notes and participant’s diaries; ethnography
Lee et al 33 International studyTo investigate different data sources to better understand perspectives of those with PH1087 from online discussion board;
27 from archival data		NRNRNRNRGroup 1 PAH (100%)NRCollected from online discussion boards and Food and Drug Administration archival data; NR
Lo et al 17ˆCanadaTo examine the presentation of existential distress in those with PAH307752
24–77		57% white,
20% South Asian, 3% black, 3% Arab, 3% Indigenous, 13% other		6.3
0.5–24		Group 1 PAH (93%)
40% PAH associated with CTD
Group 2 PH left heart disease (7%)		I=10%
II=60%
III=23%
IV=7%		Semi-structured interview; thematic analysis
Matura et al 35 International studyTo examine how people with PH are using online discussion boards549
data available for n=155		9245.6
19–78		NRNRForm of PH not specified but n=95 used prostanoid therapy, which is approved for group 1 PAHParticipants who self-identified with PH group I–V were includedData were collected from online discussion board posted by individuals with PH; qualitative descriptive methodology
Martin et al 37ˆCanadaTo identify avoidant techniques in patients with PH when discussing difficult topics associated with the disease307752
24–77		57% white6.3
0.5–24		Group 1 PAH (100%)
40% PAH associated with connective tissue disease		II=60%Semi-structured interview; qualitative content analysis
McDonough, Matura LA23 USATo explore symptoms and their impact on participants with PH experience107065
38–81		80% white
10% Asian-American
10% other		5.3
1–10		Group 1 PAH (100%)I=10%
II=10%
III=70%
IV=10%		Semi-structured interviews; qualitative descriptive methodology
Muntingh et al 25 The NetherlandsTo explore mental health needs in patients with PH248326–69NR0–16Group 1 PAH (100%)NRSemi-structured interviews; mixed methods—constant comparison method
Uhlenbusch, Löwe B (2019)32 GermanyTo explore burden of living with a rare disease, including PH4NRNRNRNRGroup 1 PAH (100%)NRFocus groups; content analysis
Yorke J, Armstrong I36*UKTo explore participant’s experiences of daily life with PH306056.3
26–80		NR<1 to >10Group 1 PAH (89%)
37%=idiopathic PAH
23%=connective tissue disease-associated PAH
17%=congenital heart disease-associated PAH
3%=heritable PAH
3%=portopulmoary hypertension
3%=drugs/toxins-associated PAH
Group 3 PH-lung (3%)
Group 4 CTEPH (7%)
NR 3%		I=0%
II=30%
III=63%
IV=7%		Semi-structured interviews; thematic analysis

  • *, +, ˆ same sample investigated across studies.

  • CTEPH, Chronic thromboembolic pulmonary hypertension; n, number; NR, not reported; PAH, pulmonary arterial hypertension; PH, pulmonary hypertension.;