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. 2024 Feb 10;14(1):e12343.
doi: 10.1002/pul2.12343. eCollection 2024 Jan.

Using PAH-SYMPACT to assess quality of life in patients with pulmonary hypertension associated with chronic lung disease

Morgan E Bailey  1 , Louise Durst  2 , Hector R Cajigas  1 , Garvan C Kane  2 , Michael J Krowka  1 , Sudhir S Kushwaha  2 , Robert B McCully  2 , Joseph G Murphy  2 , Yogesh N Reddy  2 , Robert P Frantz  2 , Hilary M DuBrock  1
Affiliations

Using PAH-SYMPACT to assess quality of life in patients with pulmonary hypertension associated with chronic lung disease

Morgan E Bailey et al. Pulm Circ. .

Abstract

Chronic lung disease (CLD) is the second leading cause of pulmonary hypertension (PH) and is associated with significant morbidity and mortality. Although PH associated with CLD (PH-CLD) leads to impaired health-related quality of life (HRQOL), there are no validated tools to assess HRQOL in PH-CLD. The Pulmonary Arterial Hypertension-Symptoms and Impact Questionnaire (PAH-SYMPACT) is an HRQOL instrument aimed at assessing the symptoms and impact of PH on overall function and well-being. We performed a single-center prospective cohort study using PAH-SYMPACT scores to compare symptoms, exercise capacity and HRQOL in patients with PAH and PH-CLD. One hundred and twenty-five patients (99 patients with idiopathic/heritable PAH and 26 with PH-CLD) completed the PAH-SYMPACT questionnaire which consists of 22 questions that assess HRQOL across four domains: cardiopulmonary (CP) symptoms, cardiovascular (CV) symptoms, physical impact (PI), and cognitive/emotional (CE) impact. Higher scores indicate worse HRQOL. We compared patients with PAH and PH-CLD using a Wilcoxon rank sum or chi-squared test as appropriate. Multivariate linear regression analysis was used to assess the relationship between PH classification and SYMPACT scores. Compared to PAH, patients with PH-CLD were older, more likely to use oxygen and had worse functional class and exercise capacity. While there was no significant difference between the two groups in CP, CV, or CE domain scores, patients with PH-CLD had significantly worse PI scores by univariate (1.79 vs. 1.13, p < 0.001) and multivariate analysis (1.61 vs. 1.17, p = 0.02) and overall worse SYMPACT scores (1.19 vs. 0.91, p = 0.03). In conclusion, patients with PH-CLD have worse HRQOL as assessed by the PAH-SYMPACT questionnaire versus patients with PAH. Although PAH-SYMPACT has not been validated in PH-CLD, the results of this study can guide clinicians in understanding the symptoms and impact of PH-CLD relative to PAH.

Keywords: PAH‐SYMPACT; patient‐reported outcomes; pulmonary arterial hypertension; quality of life.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
Mean scores for individual questions within the symptom and impact domains of the PAH‐SYMPACT questionnaire compared between patients with PAH and PH‐CLD. Higher scores indicate worse health‐related quality of life. Error bars indicate standard deviation. PAH, pulmonary arterial hypertension; PAH‐SYMPACT, PAH‐symptoms and impact; PH‐CLD, pulmonary hypertension associated with chronic lung disease; SOB, shortness of breath.
Figure 2
Figure 2
Scatter plot showing the relationship between PAH‐SYMPACT symptom (a) and impact (b) domain scores and 6‐min walk distance among patients with PH‐CLD. Corresponding correlation coefficient and p‐values are also shown. PAH, pulmonary arterial hypertension; PAH‐SYMPACT, PAH‐symptoms and impact; PH‐CLD, pulmonary hypertension associated with chronic lung disease.
Figure 3
Figure 3
Analysis of covariance depicting the relationship between pulmonary vascular resistance and mean cardiovascular symptom domain (a) and cognitive and emotional impact domain (b) scores for patients with pulmonary arterial hypertension and pulmonary hypertension associated with chronic lung disease.
See this image and copyright information in PMC

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