Elsevier

American Heart Journal

Volume 189, July 2017, Pages 167-176
American Heart Journal

Clinical Investigation
High-risk echocardiographic features predict mortality in pulmonary arterial hypertension

https://doi.org/10.1016/j.ahj.2017.04.013Get rights and content

Aims

Echocardiography is the most common imaging modality for assessment of the right ventricle in patients with pulmonary arterial hypertension (PAH). Echocardiographic parameters were identified as independent risk factors for mortality in the Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL) and other PAH cohorts. We sought to identify readily obtained echocardiographic features associated with PAH survival.

Methods and results

Retrospective analysis of 175 patients with Group 1 was performed. Baseline clinical and laboratory assessment including REVEAL risk criteria were obtained and standard 2-Dimensional and Doppler echocardiography performed at baseline was reviewed. Univariate and multivariate analyses of echocardiographic parameters were performed. Estimated right atrial pressure> 15 mmHg (HR 2.39, P = .02), tricuspid regurgitation ≥ moderate (HR 2.16, P = .04), and presence of pericardial effusion (HR 1.8, P = .05) were identified as independent, high-risk echocardiographic features in PAH. A validation cohort of 677 patients was identified and Kaplan–Meier survival analysis was performed in both cohorts. High-risk echocardiographic features stratified survival curves of both cohorts (P < .01 for all). The presence of 3 high-risk echocardiographic features greatly increased risk of 1-year (RR 4.86) and 3-year (RR 3.35) mortality (P < .05 for both).

Conclusion

Estimated right atrial pressure> 15, tricuspid regurgitation ≥ moderate, and presence of pericardial effusion are high-risk echocardiographic features in PAH. When seen in combination, these features greatly increase risk of mortality in PAH and may lead to more timely enhanced therapy for patients identified as having an increased risk for death.

Section snippets

Study design

In this multi-center, retrospective analysis, we performed a screening chart review of all patients with the diagnosis of PAH that were evaluated at Mayo Clinic Florida (MCF) between December 2003 and December 2012. Patients that met the 5th World Symposium Diagnostic criteria for Group 1 PAH based on clinical evaluation, echocardiography and RHC were included in the analysis cohort (N = 175).10 Comprehensive testing was performed and recorded at baseline. Group 1 PAH patients represented those

Clinical characteristics

The retrospective MCF cohort included 175 Group 1 PAH and is detailed in Table I. The average follow-up time was 141 ± 124 weeks. The population was predominately female and most patients carried a diagnosis of idiopathic or collagen vascular disease-associated PAH. Most patients reported baseline NYHA functional class 3 symptoms. Overall, the population had a calculated REVEAL score of almost 9, considered moderately high-risk. The composite endpoint of death or transplant occurred in 83 (47%)

Discussion

Echocardiography is generally the first line imaging modality for RV assessment due to its widespread availability. Quantitative assessment of size and function are limited at times by complex anatomy of the RV. A systematic approach for both qualitative and quantitative RV assessment has been proposed and has been incorporated into the routine assessment of many PAH patients, often including RV dimensions, FAC, RA size, TAPSE, and RIMP, also known as the Tei index,6 among others. Although

Limitations

An important limitation of this retrospective cohort analysis is the subjective nature of grading TR and the presence of pericardial effusion. Echocardiographic guidelines do provide some leeway for TR classification; however, grades of moderate or severe TR are supported by secondary findings such as high density TR Doppler signals and hepatic vein flow reversal. Unfortunately, an adequate TR Doppler envelope cannot always be obtained, even in patients with pulmonary hypertension.31

Conclusion

Standard echocardiography utilizing 2D and Doppler provides an indirect assessment of RV function that can be used to predict mortality in PAH patients. The presence of RAP >15 mmHg, TR severity ≥ moderate or pericardial effusion was independently associated with increased mortality; therefore, each was identified as a high-risk echocardiographic feature in PAH. When these features were seen in combination, PAH survival was further reduced. The presence of these high-risk echocardiographic

Author contributions

Dr Austin contributed to study design, data acquisition, analysis, interpretation, and drafting of the manuscript.

Dr Burger contributed to study design, data acquisition, interpretation, drafting of the manuscript, and critical revision based on content expertise.

Dr Kane contributed to data acquisition, analysis, interpretation, drafting of the manuscript, and critical revision based on content expertise.

Dr Safford obtained funding for the project, contributed to data acquisition,

Funding

This work was supported by the Center for Translational Science Activities grant support (UL1 TR000135).

Declaration of Helsinki

This retrospective study complies with the Declaration of Helsinki and has been approved by the Mayo Clinic Institutional Review Board as minimal risk (#12–004764); therefore, patient consent was not required.

Acknowledgements

We would like to thank the Center for Translational Science Activities grant support (UL1 TR000135) for assistance with this study.

References (31)

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Conflict of Interest: Christopher Austin, Charles Burger, Robert Safford, Joseph Blackshear, Ryan Ung, Jordan Ray, Ali Alsaad, Garvin Kane, and Brian Shapiro have no conflicts of interest.

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