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

Abstract

BACKGROUND

Pulmonary arterial hypertension (PAH) is a debilitating and progressive pulmonary pathology that often leads to death. Guidelines recommend the use of palliative care (PC) early in the treatment course to ease the burden of symptoms; however, uptake remains low.

AIM

To evaluate barriers to PC uptake and determine its association with mortality in patients with PAH.

METHODS

All studies discussing PC in PAH were selected in our review and analysis. Clinical and cross-sectional studies were included. Barriers were described in a qualitative fashion. A random-effects meta-analysis was also conducted, in which the odds ratio for mortality was pooled and reported, along with 95% confidence intervals.

RESULTS

A total of 19 studies were included in the review. The most common barriers identified included feeling like the patients were “not sick enough”, belief that PC is only appropriate for end-of-life care, belief that it would burden family members, and general feelings of hopelessness. Physicians identified structural causes, such as a lack of funding and low levels of PC-related knowledge, as barriers to recommending PC. The meta-analysis showed no statistically significant difference in mortality across four included studies (Log odds ratio = 0.89, 95% confidence intervals: -3.06-1.28). Heterogeneity was high (I² = 80.32%).

CONCLUSION

Uptake of PC in PAH is low due to patient and physician-level barriers, which can be overcome with systematic PC integration. Long-term studies are also needed to investigate the impact of PC on outcomes in PAH, as the current limited data show no significant difference.

Key Words: Palliative care; Pulmonary arterial hypertension; Barriers; Underutilization; Outcomes

Core Tip: There are significant patient and physician-level barriers hindering the uptake of palliative care in pulmonary arterial hypertension, including feelings of hopelessness, depression, sadness, lack of funding, and lack of knowledge. These psychological and systemic challenges collectively limit timely referral and integration of palliative services within routine pulmonary arterial hypertension management. A meta-analysis of four studies reveals no statistically significant difference in mortality with palliative care (Log odds ratio = 0.89, 95% confidence intervals: -3.06-1.28).

INTRODUCTION

Pulmonary hypertension (PH) is a progressive, debilitating pulmonary vascular disease that can occur secondary to underlying cardiac or pulmonary diseases but is often idiopathic in origin[1,2]. Pulmonary arterial hypertension (PAH) is associated with a significant symptom burden. Patients commonly report dyspnea on exertion, fatigue, exertional chest pain, pre-syncope, or syncope[3-5]. PAH substantially impacts physical and emotional health, often leading to increased psychological stress, resulting in a marked reduction in quality of life. It is a significant contributor to increased morbidity and mortality[2].

For chronic progressive diseases such as PAH, palliative care (PC) is seen as a viable option that can potentially increase comfort in refractory and severe cases. PC is a form of multidisciplinary end-of-life care that aims to relieve physical and psychological symptoms, thereby improving overall quality of life. The American College of Chest Physicians recommends the incorporation of PC services into the management of PAH patients[6]. There are various types of PC available for PAH patients, ranging from invasive (Potts shunt being the most common) to non-invasive, such as counseling and physician and family-led discussions, and have been known to improve overall symptoms and quality of life[7].

Despite guideline recommendations, recent evidence from the Pulmonary Hypertension Association Registry highlights stark underutilization of PC for PAH patients, with only 10% being referred despite significant symptoms[8]. Specialist-led PC is also utilized in a minority of patients despite showing significant benefits in care and patient experiences[7]. This underutilization has been attributed to several barriers, including limited awareness and access. Therefore, we conducted this in-depth systematic review to describe the role and effectiveness of PC in PAH management, highlight specific palliative interventions, describe patient perspectives on PAH, and identify patient and physician-level factors that have increased barriers to adoption and access despite apparent benefits. To our knowledge, this is the first meta-analysis to evaluate the outcomes of PC in PAH patients.

MATERIALS AND METHODS

Search strategy and study selection

We searched multiple databases, including PubMed, Scopus, EMBASE, and Web of Science, to identify studies published from database inception up to May 2025. The following search string was used: [“palliative care” (MeSH Terms) OR “palliative care” (All Fields)] AND [“pulmonary hypertension” (MeSH Terms) OR “pulmonary hypertension” (All Fields) OR “PAH” (All Fields) OR “pulmonary arterial hypertension” (All Fields)]. Additionally, results for the search string: “(palliative care) AND (pulmonary hypertension)” were also reviewed to include any studies missed in the search. Two independent reviewers (Shaikh A and Rab AU) screened all possible studies for inclusion at the title and abstract level, followed by the evaluation of full texts. A third independent reviewer (Ali W) resolved all conflicts.

Eligibility criteria

For the purposes of this review, “palliative care” was defined broadly to include both specialist palliative care (i.e., care delivered by dedicated PC teams or specialists) and primary palliative care (i.e., basic symptom management and supportive care provided by non-specialist clinicians). Clinical and qualitative studies, including observational studies, randomized controlled trials, and some cross-sectional studies that discussed any aspect of PC in PAH and provided patient or physician-level data, were included. Studies mentioning symptom burden, patient and clinician perspectives, and barriers to care were also included. Studies that did not report patient-level data and were published in any language other than English, as well as those focused on non-human and non-adult participants, were all excluded. Other relevant studies were also identified through a manual search of secondary sources, including references to initially identified articles, to identify any studies missed by the literature searches. When an abstract from a meeting and a full article referred to the same study, only the full article was included in the analysis.

Data extraction

Two independent reviewers extracted all data from eligible studies on a pre-specified data collection form. Extracted data included the following items: Study design, sample size, methods of palliation, barriers to palliation, patient perspectives, outcomes, main study results, and recommendations.

Statistical analysis

Continuous variables were expressed as means ± SD, and categorical variables were expressed as frequencies and percentages. All qualitative data were reported in tables. Outcomes were expressed as odds ratios (ORs) with their 95% confidence intervals as calculated for each study after preparing a 2 × 2 contingency table of PC and non-PC patients according to events and non-events. ORs were pooled across studies using the random-effects model. To assess heterogeneity within studies, the I² statistic was calculated. The I² statistic represents the proportion of heterogeneity of effects across studies that cannot be attributed to sampling variability. Values greater than 50% denoted significant heterogeneity owing to differences in study populations, protocols, interventions, and outcomes. Publication bias was not assessed for as the minimum number of studies required to conduct a funnel-plot-based assessment is 10[9].

Analyses were conducted using Microsoft Excel Office 16 (Microsoft Corporation, Redmond, WA, United States) and STATA version 17 (StataCorp, College Station, TX, United States). A risk of bias assessment of the included studies was conducted using the Newcastle-Ottawa scale. This systematic review has been reported according to the recommendations set forth by the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) work groups.

RESULTS

A total of 19 studies were included in our analysis. Figure 1 shows the PRISMA study selection chart for the systematic review. The median sample size was 22, with the largest sample size being 682 patients in one study. The mean age of patients was 33.2 ± 21.6, with a mean of 68.3% of patients being female. 9 (47.4%) studies were retrospective cohort studies, 4 (21.1%) were prospective, 4 (21.1%) were cross-sectional, and 2 (10.5%) were qualitative. Table 1 shows all demographic characteristics of the included studies. Results for the risk of bias, which was found to be moderate in most included studies, are presented in Table 2.

Figure 1 Prisma flow diagram for study selection.

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

Burden of symptoms

Patients with PAH reported a wide range of symptoms. Adult patients with PAH reported experiencing dyspnea, fatigue, cyanosis, and chest pain[10-12]. Right-sided heart failure (RHF) and syncope were also reported in most patients[13,14]. In pediatric populations, growth retardation, asthenia, syncope, heart failure, and hemoptysis were most noted[15].

Methods of palliation

Palliative interventions were primarily of two types: Non-invasive and invasive. Noninvasive methods reported included patient-physician discussions to aid in coping. The most commonly discussed non-invasive method was web-based meditation sessions[16]. Invasive methods were much more commonly discussed. Amongst them, the most reported invasive methods included Potts shunting, atrial septostomy, and PAD denervation[12-14,17-19]. One study also described palliative Senning as a reasonable option[11].

Effectiveness of palliation

Effectiveness varied according to the method of palliation. Studies focusing on invasive methods of palliation have shown a significant decrease in pulmonary artery pressure, improved arterial oxygen saturation, and children overcoming growth retardation, with a significant reduction in signs and symptoms of RHF and syncope post-intervention[11,19]. Lung transplants led to reduced mechanical ventilation times, hospital stay durations, and post-treatment complications[18]. Additionally, improvement in functional tests such as the 6-minute walk test was also seen[12]. Some studies reported an increase in health-related quality of life[16]. Table 3 shows all study outcomes and recommendations.

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.

Barriers to the uptake of PC

Patient-level barriers to the uptake of PC included misconceptions that PC was only appropriate for end-of-life care, a belief that they were “not sick enough”, and concern about burdening family members[5,17]. There was also a negative association with the term palliative, with patients believing that it meant they were closer to dying[16,17]. Another reason identified was an excessive burden of symptoms in late-stage patients[13]. Physicians tended to utilize PC care mostly in the context of end-of-life care and hospice referrals. Most physicians reported similar patient-level barriers to uptake, such as patients “not feeling sick enough” or feeling like “giving up hope”, which were the two most common reasons for denial of PC. A third of physicians felt comfortable engaging in PC discussions but did not deem it necessary for certain PAH patients, and the rest expressed hesitation. There was also miscommunication and a lack of awareness regarding certain principles of PC among physicians. Physicians also cited young patient age and high rates of denial among these patients as reasons not to recommend PC. Health system level limitations were also identified, including a lack of funding and support, curbing the utilization of PC[17,18].

Systemic challenges represent significant obstacles to the widespread adoption of PC for PH patients. Due to the rarity of PH, most healthcare systems lack tailored PC programs for this patient population. The specialized nature of PH care, requiring experienced providers and intricate therapies, has led to a compartmentalization of care that excludes palliative services. Moreover, reimbursement structures and healthcare funding often fail to prioritize PC services in chronic disease management, further limiting their integration into standard PH care. The limited availability of interdisciplinary teams, especially in smaller hospitals or non-urban areas, exacerbates the issue by restricting access to necessary resources and collaborative approaches.

Outcomes

A total of four studies reported events and non-events related to mortality, comparing the PC group with the non-PC group. A meta-analysis showed no difference in mortality between PC and non-PC groups (Log OR = 0.89, 95% confidence intervals: -3.06-1.28). Heterogeneity was high (I² = 80.32%). Figure 2 depicts the meta-analysis.

Figure 2 Meta-analysis. PC: Palliative care; CI: Confidence interval.

DISCUSSION

PC in PAH can potentially become a significant component of regular PAH care. Palliative interventions may be categorized based on their intended timing and role. Studies show that non-invasive approaches, including communication support and psychosocial interventions, are more aligned with chronic symptom management, improving patient outlook and overall quality of life[20,21]. On the other hand, invasive procedures, such as Potts shunting and atrial septostomy, are often used as acute bridge therapies to stabilize severely ill patients or delay lung transplantation, significantly improving symptoms and quality of life[22,23]. Yet uptake remains extremely low[24]. Our findings shed light on the possible reasons for this disparity.

Firstly, we found that there are significant patient- and physician-level challenges that lower the utilization of PC. Most patient-level challenges stem from a lack of knowledge and feelings of hopelessness surrounding PC. Patients and their families often feel as if relying on PC will mean relinquishing control, giving up, and being ineligible for regular PAH care. Therefore, PC denials are frequent[10,25]. Additionally, symptom burden and decreased quality of life also lead to greater denials of PC, with patients who have a more positive outlook being keener on receiving PC. However, a lack of symptoms can also lead patients to decide that PC is not yet needed[16,26].

On the other hand, physicians reported that the lack of objective criteria for referrals, patient-specific assessments not necessitating PC referral, and system-based limitations, such as a lack of funding, were causes for underutilization[27]. While patient-specific approaches in referring for PC should always play a role, earlier integration can improve outcomes and quality of life[24]. Patient education can also play a significant role in improving PC adoption through regular, detailed discussions with patients and their family members, focusing on alleviating concerns and explaining what PC means. One of the barriers identified simply stemmed from negative feelings associated with the term “palliation”[19,28]. Table 4 summarizes the overarching barriers encountered in PC uptake and what could be done to facilitate their utilization.

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.

Secondly, in every case, the adoption of PC led to an improvement in outcomes. There was marked improvement in 6-minute walk tests, dyspnea, RHF symptoms, arterial oxygenation, growth curves in children, syncope, hematocrit, mechanical ventilation, hospital duration of stay, complication rate, pulmonary artery pressure, time to lung transplant, and mood symptoms such as depression and sadness[11,12,17-19,28,29]. This marked improvement over invasive procedures, namely Potts shunting and atrial septostomy, makes the case for integrating PC with PAH, which would not only help achieve better outcomes but also facilitate systematic integration, allowing for discussion with patients[30].

However, it must be noted that the results of our meta-analysis did not show any difference in outcomes between invasive PC procedures and those who did not receive any PC or directly received lung transplants. Yet, the overall decrease in symptom burden and donor shortages for transplantation still makes a case for utilizing invasive PC in patients, especially those waiting for transplantation or suffering from severe disease burden[31,32]. Additionally, the lack of difference in outcomes could also have occurred due to the inclusion of very limited studies with extremely small sample sizes. Additional long-term studies and pooled analyses will help in defining outcomes with PC in the future.

This review has several limitations. Firstly, due to a lack of quantitative data, we were unable to assess outcomes other than mortality in our meta-analysis. Additionally, given the small number of included studies, the results may be considered less generalizable and exploratory in nature. Secondly, heterogeneity was high, and this may be attributed to differences in study designs, patient populations, type and timing of palliative care interventions, outcome definitions, and small sample sizes. These factors likely introduced clinical and methodological variability across studies, which limits the interpretability of pooled mortality effects. Additionally, 6 out of the 19 included studies were judged to be high risk, which may have biased our results. Furthermore, exclusion of non-English studies may have introduced language bias. Although the meta-analysis included fewer than 10 studies, precluding the creation of a funnel plot, the risk of publication bias cannot be ruled out. Moreover, individual small-scale clinical data in the form of case reports and small case series were excluded, which may have omitted useful clinical insights, especially given the rarity of PAH. Finally, this review was not registered in the International Prospective Register of Systematic Reviews, as it began as a narrative review. The shift to a systematic review and meta-analysis occurred later, once sufficient data became available. While the lack of a pre-specified protocol introduces potential reporting bias, the review was conducted in line with PRISMA guidelines to ensure transparency.

CONCLUSION

PC in PAH is underutilized due to key patient-level barriers, which can be overcome with systematic PC integration with regular PAH care and detailed patient education on PC. Additionally, future long-term studies are needed to investigate the impact of PC on outcomes in PAH, as the current limited data show no statistically significant difference. Yet, improvement in overall quality of life is marked, and early uptake of PC can lead to decreased symptom burden and improved mood symptoms.