Original Research
Pulmonary Artery Strain Predicts Prognosis in Pulmonary Arterial Hypertension

https://doi.org/10.1016/j.jcmg.2023.02.007Get rights and content

Abstract

Background

Current cardiac magnetic resonance (CMR) imaging in pulmonary arterial hypertension (PAH) focuses on measures of ventricular function and coupling.

Objectives

The purpose of this study was to evaluate pulmonary artery (PA) global longitudinal strain (GLS) as a prognostic marker in patients with PAH.

Methods

The authors included 169 patients with PAH from the ASPIRE (Assessing the Spectrum of Pulmonary hypertension Identified at a REferral centre) and INITIATE (Integrated computatioNal modelIng of righT heart mechanIcs and blood flow dynAmics in congeniTal hEart disease) registries, and 82 normal controls with similar age and gender distributions. PA GLS was derived from CMR feature tracking. Right ventricular measurements including volumes, ejection fraction, and right ventricular GLS were also derived from CMR. Patients were followed up a median of 34 months with all-cause mortality as the primary endpoint. Other known risk scores were collected, including the REVEAL (Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management) 2.0 and COMPERA (Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension) 2.0 scores.

Results

Of 169 patients (mean age: 57 ± 15 years; 80% female), 45 (26.6%) died (median follow-up: 34 months). Mean PA GLS was 23% ± 6% in normal controls and 10% ± 5% in patients with PAH (P < 0.0001). Patients with PA GLS <9% had a higher risk of mortality than those with PA GLS ≥9% (P < 0.001), and this was an independent predictor of mortality in PAH on multivariable analysis after adjustment for known risk factors (HR: 2.93; P = 0.010). Finally, in patients with PAH, PA GLS provided incremental prognostic value over the REVEAL 2.0 (global chi-square; P = 0.001; C statistic comparison; P = 0.030) and COMPERA 2.0 (global chi-square; P = 0.001; C statistic comparison; P = 0.048).

Conclusions

PA GLS confers incremental prognostic utility over the established risk scores for identifying patients with PAH at higher risk of death, who may be targeted for closer monitoring and/or intensified therapy.

Section snippets

Study population

In this multicenter study, data from 169 patients diagnosed with PAH following multimodal imaging assessment and right heart catheterization were retrieved from the ASPIRE (Assessing the Spectrum of Pulmonary hypertension Identified at a REferral centre)6 and INITIATE (Integrated computatioNal modelIng of righT heart mechanIcs and blood flow dynAmics in congeniTal hEart disease)7 registries. The diagnostic criteria for PAH was based on guidelines1: mean pulmonary artery pressure >25 mm Hg,

Baseline demographic and clinical variables

Patient demographic, clinical, and CMR characteristics of the study population are shown in Table 1. Seventy and 90 patients had idiopathic PAH and PAH associated with connective tissue disease (CTD), respectively. Sixty-one percent (103/169) of patients were in World Health Organization functional class III-IV, and the median REVEAL 2.0 and COMPERA 2.0 were 8 and 3, respectively. Patients with PAH had significantly higher RV EDV index and ESV index and lower right ventricular ejection fraction

Discussion

Our study has demonstrated an approach for identifying patients with PAH at high risk of death using PA GLS from CMR feature tracking. This is one of the largest studies to examine PA strain and long-term outcomes in a characterized adult PAH cohort that combined 2 contemporary PAH registries. We have demonstrated that PA GLS reflects a combination of both PA stiffness and RV function. We show for the first time that impairment of PA GLS is a significant and independent predictor of mortality

Conclusions

We propose PA GLS as a noninvasive approach for identifying patients with PAH who are at high risk of death and who may benefit from intensified therapy and/or closer surveillance. Our study has demonstrated that PA GLS has significant prognostic utility over standard risk score as well as RV volume and functional indices, which supports its potential role in the clinical management of patients with PAH.

COMPETENCY IN MEDICAL KNOWLEDGE: In the present study, we demonstrated the prognostic value

Funding Support and Author Disclosures

This study has received funding support from the National Medical Research Council of Singapore (NMRC/OFIRG/0018/2016). Dr Kiely has received personal fees for consultancy work, educational talks and participation in steering committees from Acceleron, Ferrer, GSK, MSD, and Janssen; and has received funding from the National Institute for Health and Care Research, Sheffield Biomedical Research Centre. The funder had no role in the design and conduct of the study; collection; management,

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  • The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the Author Center.

    Drs Zhong, Leng, and Alabed contributed equally to this paper.

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