Palliative care in pulmonary hypertension: A systematic review and meta-analysis

Table 1 Demographic characteristics of included studies

Ref.	Study design	Intervention	Sample size	Mean age
Rohlfing et al[8], 2023	Retrospective	N/A	92	N/A
Swetz et al[10], 2012	Cross-sectional	N/A	276	48.9
Penha et al[11], 2015	Retrospective	Senning	21	2.5
Chen et al[12], 2013	Prospective	Pulmonary artery denervation	13	40
Chiu et al[13], 2015	Retrospective	Atrial septostomy	32	23
Bobhate et al[14], 2021	Retrospective	Potts shunt	16	8
Baruteau et al[15], 2012	Retrospective	Potts shunt	8	< 18
Vonvisger et al[16], 2025	Prospective	Web-based meditation	9	N/A
Grady et al[17], 2016	Retrospective	Potts shunt	5	< 18
Lancaster et al[18], 2021	Prospective	Potts shunt	23	10.1
Baruteau et al[19], 2015	Retrospective	Potts shunt	24	8.1
Brown et al[21], 2023	Cross-sectional	N/A	18	53.5
Anand et al[24], 2020	Retrospective	N/A	682	62.1
Ivarsson et al[25], 2016	Qualitative	N/A	17	> 18
Hrustanovic-Kadic et al[26], 2021	Prospective	N/A	49	51.9
Brown et al[27], 2023	Qualitative	N/A	12	48.5
Sandoval et al[29], 2021	Retrospective	Atrial septostomy	34	35
Fenstad et al[30], 2014	Cross-sectional	N/A	79	N/A
Tye et al[32], 2024	Cross-sectional	N/A	84	35

N/A: Not applicable.

Table 2 Risk of bias assessment

Retrospective/prospective cohort and qualitative studies
Ref.	Selection				Comparability	Outcome			Total	Risk
	Representativeness of the exposed cohort	Selection of the non-exposed cohort	Ascertainment of exposure	Demonstration that outcome of interest was not present at start of study	Comparability of cohorts on the basis of the design or analysis	Assessment of outcome	Adequate follow-up	Adequacy of follow-up cohort
Rohlfing et al[8], 2023	1	1	1	1	1	1	1	1	8	Low
Penha et al[11], 2015	1	0	1	1	0	1	1	1	6	Medium
Chen et al[12], 2013	1	1	1	1	1	1	1	1	8	Low
Chiu et al[13], 2015	1	0	1	1	0	1	1	1	6	Medium
Bobhate et al[14], 2021	1	0	1	1	0	1	1	1	6	Medium
Baruteau et al[15], 2012	1	0	1	1	0	1	1	1	6	Medium
Vonvisger et al[16], 2025	1	1	1	0	2	1	1	1	8	Low
Grady et al[17], 2016	1	0	1	1	0	1	1	1	6	Medium
Lancaster et al[18], 2021	1	0	1	1	0	1	1	1	6	Medium
Baruteau et al[19], 2015	1	0	1	1	0	1	1	1	6	Medium
Anand et al[24], 2020	1	1	1	1	1	0	0	0	5	High
Ivarsson et al[25], 2016	1	0	1	1	1	0	1	0	5	High
Hrustanovic-Kadic et al[26], 2021	1	0	1	1	0	1	1	1	6	Medium
Brown et al[27], 2023	1	0	1	1	0	0	1	1	5	High
Sandoval et al[29], 2021	1	0	1	1	0	1	1	1	6	Medium
Cross sectional studies
Study	Selection				Comparability		Outcome
	Sample representativeness	Sample size justified	Non-respondents described	Exposure measurement	Group comparability	Additional confounder	Outcome assessment	Statistical test	Total	Risk
Swetz et al[10], 2012	1	0	1	2	0	0	1	1	6	Medium
Brown et al[21], 2023	1	0	0	1	0	0	1	0	3	High
Tye et al[32], 2024	1	0	1	1	0	0	1		4	High
Fenstad et al[30], 2014	1	0	1	1	0	0	1	1	5	High

Table 3 Study results and recommendations

Ref.	Barriers to PC	Study main results	Recommendations
Rohlfing et al[8], 2023	Infrequent referrals	92 of 1578 patients were referred to PC (5.8%); 43% were referred at their last visit prior to death; referrals were associated with: Increasing age per decade (HR = 1.35, 95%CI: 1.16-1.58); lower body mass index (HR = 0.97, 95%CI: 0.94-0.998); supplemental oxygen use (HR = 2.01, 95%CI: 1.28-3.16); parenteral prostanoid use (HR = 2.88, 95%CI: 1.84-4.51); worse quality of life, measured via lower physical (HR = 0.97, 95%CI: 0.95-0.99); mental (HR = 0.98, 95%CI: 0.96-0.995) scores on the 12-item Short Form Health Survey	Patients with PAH are infrequently referred to PC, even at centers of excellence. Referrals occur in sicker patients with lower quality of life scores, often close to the end of life. There needs to be earlier utilization based on predictive measures
Swetz et al[10], 2012	The perception that patients were not seriously ill; absence of PC recommendation from the healthcare team; confusion between PC and hospice; lack of understanding about what PC involves; concerns that starting PC would lead to discontinuation of PAH-specific treatments; hopelessness	High symptom burden, characterized by pain, fatigue, decreased overall quality of life, physical well-being, social activity, and emotional well-being	Improved patient education regarding all aspects of palliation is needed to improve adoption rates
Penha et al[11], 2015	N/A	Increased arterial oxygenation; decreased hematocrit	Palliative Senning procedure should be considered in patients with late diagnosis when severe; pulmonary vascular disease is already established
Chen et al[12], 2013	N/A	Improved 6-minute walk test and lower PAP	Further randomized study is required to confirm the efficacy of PADN
Chiu et al[13], 2015	N/A	No sig diff in PA pressure, biomarkers	Can be a successful bridge to lung transplant and RHF symptom alleviation when used in conjunction with appropriate medical treatment
Bobhate et al[14], 2021	N/A	12/16 patients survived the procedure; patients who did not survive the procedure were older, with severe right ventricular systolic dysfunction and functional class IV; patients who survived the procedure were followed up in the pulmonary hypertension clinic; of the 11/13 patients discharged after the operation, 11 showed sustained clinical, echocardiographic, and biochemical improvement, which reduced the need for pulmonary vasodilator therapy in 10/11 patients	Potts shunt/PDA stenting is feasible in patients with PAH; it can be done safely with an acceptable success rate
Baruteau et al[15], 2012	The term PC is associated with negative feelings and end-of-life care	Iso-systemic pulmonary artery pressures achieved; syncope and RHF failure signs disappeared; all children caught up to normal growth curves	Potts shunt should be considered as a first surgical or interventional step in the management of children with severe, drug-refractory PAH
Vonvisger et al[16], 2025	Patients withadvanced PH, burdened by symptoms, are less likelyto engage in a traditional, in-person, mindfulness-based program	Hr QOL improved in both the intervention and control groups; potential reduction in depressive symptoms and sleep disturbances	Useful complementary approach in PH management
Grady et al[17], 2016	N/A	Improved: Mechanical ventilation time; median hospital length of stay; overall complication rate	Potts shunt may serve as a valuable element in palliative strategies, potentially helping to postpone the need for lung transplantation while improving both survival and quality of life
Lancaster et al[18], 2021		Outcomes after Potts shunt was superior to lung transplant, including mechanical ventilation time (1.3 days vs 10.2 days, P ¼ 0.019), median hospital length of stay (9.8 days vs 34 days, P ¼ 0.012), and overall complication rate [35% (7/20) vs 81% (25/31), P ¼ 0.003]	Potts shunt yielded durable alleviation of supra-systemic RV pressures and improved functional capacity; was associated with no difference in survival when compared with a lung transplant was shown to be a viable component
Brown et al[21], 2023	Focus on staying alive; uncertainty about who would provide care; feeling that they were not ill enough to warrant such conversations	Emphasized the need for better education and communication	Palliation should be introduced early in treatment; patients should be approached by their PAH physicians about PC
Ivarsson et al[25], 2016	Lack of support	Three categories that describe patients’ experiences of support emerged: Support linked to healthcare, support linked to the private sphere, and support linked to persons outside the private sphere	Healthcare practitioners must work more collaboratively to detect patients’ needs for support and to develop the patient’s own skills to manage daily life. The PAH teams should tailor interventions to provide emotional, informational, and instrumental support and guidance to patients and their families
Hrustanovic-Kadic et al[26], 2021	More symptoms and disease impact led to more anxiety, depression, fear, and stress towards PC	REVEAL 2.0 and PPCI scores were useful in evaluating PC needs	These scores should be utilized
Sandoval et al[29], 2021	N/A	Clinical improvement seen in 88% of surviving patients, improved 6-minute walk test	The performance of AS at a relatively early stage of the disease may be beneficial
Fenstad et al[30], 2014	PAH patient or family was not agreeable to consultation; there is concern that palliative medicine consultation may be viewed by patients as “giving up hope”; PC consultation was necessary; confusion regarding PAH patients are not eligible to have PC if they continue to receive active therapies; simultaneous PAH aggressive treatment and PC is difficult; young age; the name “palliative” has a negative connotation	N/A	Efforts at integration of PC may be a means of improving QOL and may assist PAH providers in symptom management and complex communication issues
Tye et al[32], 2024	N/A	Patients with a higher WHO functional class and negative feelings (r = 0.333, P = 0.004); cognitive reaction to PC: Hopeless (r = 0.340, P = 0.003); supported (r = 0.258, P = 0.028); disrupted (r = 0.262, P = 0.025); perception of burden (r = 0.239, P = 0.041) are more receptive to PC; WHO class, N-terminal pro B-type natriuretic peptide, and 6-minute walking test distance were not associated with higher readiness for PC; in logistic regression analyses, patients with positive feelings (β = 2.240, P ≤ 0.05) and practical needs (β = 1.346, P ≤ 0.05) were more receptive to PC	Disease severity did not directly influence patients’ readiness for PC; patients with a positive outlook were more receptive to PC. This should be incorporated during the selection of PC

PC: Palliative care; HR: Hazard ratio; PAH: Pulmonary arterial hypertension; N/A: Not applicable; PAP: Pulmonary artery pressure; PADN: Pulmonary artery denervation; PA: Pulmonary artery; RHF: Right-sided heart failure; Hr QQL: Health-related quality of life; PH: Pulmonary hypertension; RV: Right ventricular; PPCI: Primary percutaneous coronary intervention; WHO: World Health Organisation; CI: Confidence interval.

Table 4 Barriers and facilitators to palliative care integration in pulmonary arterial hypertension

Category	Barriers	Facilitators
Systemic	Lack of programs tailored to rare diseases	Emerging care models aimed at integrating PC into PH
Clinician-level	Limited training; misconceptions about PC	Increased clinician education
Patient-level	Association with end-of-life care	Improved patient awareness and perception of PC
Interdisciplinary	Communication and coordination gaps	Enhanced interdisciplinary collaboration among teams

PC: Palliative care; PH: Pulmonary hypertension.