Clinical InvestigationHigh-risk echocardiographic features predict mortality in pulmonary arterial hypertension
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)
- et al.
The REVEAL registry risk score calculator in patients newly diagnosed with pulmonary arterial hypertension
Chest
(2012) - et al.
Definitions and Diagnosis of Pulmonary Hypertension
J Am Coll Cardiol
(2013) - et al.
Updated clinical classification of pulmonary hypertension
J Am Coll Cardiol
(2009) - et al.
Research electronic data capture (REDCap)--a metadata-driven methodology and workflow process for providing translational research informatics support
J Biomed Inform
(2009) - et al.
Recommendations for evaluation of the severity of native valvular regurgitation with two-dimensional and Doppler echocardiography
J Am Soc Echocardiogr
(2003) - et al.
Recommendations for chamber quantification: a report from the American Society of Echocardiography's Guidelines and Standards Committee and the Chamber Quantification Writing Group, developed in conjunction with the European Association of Echocardiograph
J Am Soc Echocardiogr
(2005) - et al.
Echocardiographic assessment of estimated right atrial pressure and size predicts mortality in pulmonary arterial hypertension
Chest
(2015) - et al.
American Society of Echocardiography clinical recommendations for multimodality cardiovascular imaging of patients with pericardial disease: endorsed by the Society for Cardiovascular Magnetic Resonance and Society of Cardiovascular Computed Tomography
J Am Soc Echocardiogr
(2013) - et al.
Frequency and severity of tricuspid regurgitation determined by Doppler echocardiography in primary pulmonary hypertension
Am J Cardiol
(2003) - et al.
Assessment of right ventricular function using two-dimensional echocardiography
Am Heart J
(1984)
Right ventricular strain for prediction of survival in patients with pulmonary arterial hypertension
Chest
Right atrial size and tricuspid regurgitation severity predict mortality or transplantation in primary pulmonary hypertension
J Am Soc Echocardiogr
Pericardial effusions in pulmonary arterial hypertension: characteristics, prognosis, and role of drainage
Chest
Frequency and prognostic significance of pericardial effusion in primary pulmonary hypertension
Am J Cardiol
Incidence and significance of pericardial effusion in patients with pulmonary arterial hypertension
Can J Cardiol
Cited by (10)
Prognostic Value of Echocardiographic Variables Prior to and Following Initiation of Parenteral Prostacyclin Therapy: An Observational Study
2022, ChestCitation Excerpt :The large number of candidate variables included also makes it possible that some false-positive findings may have occurred. Despite these limitations, our study is the largest study assessing the prognostic value of echocardiographic parameters in patients with PAH on parenteral prostacyclin therapy, and the only study thus far to assess the value of posttreatment echocardiograms in assessing survival for patients with PAH on parenteral therapy (and only one of a few to assess posttreatment echocardiograms vs PAH outcomes with any therapy).4,6-20 Finally, use of echocardiography in the assessment of prognosis does not eliminate the need for periodic hemodynamic reassessment, as these measures are most often complementary in assessing overall response to therapy.38
Noninvasive Risk Score to Screen for Pulmonary Hypertension With Elevated Pulmonary Vascular Resistance in Diseases of Chronic Volume Overload
2021, American Journal of CardiologyCitation Excerpt :In this study, TRV, right ventricular function, and TAPSE strongly associated with PH-PVR. These markers of right ventricular systolic function each variably associate with outcomes in PH.10,15-19 In contrast, an abnormal left ventricular ejection fraction associated with lower odds of PH-PVR; this finding likely reflects the substantial prevalence of isolated post-capillary PH in patients with heart failure with a reduced ejection fraction.20 This combination of left- and right-sided echocardiography measurements improved the accuracy of screening for PH-PVR.
Interstitial Lung Disease and Other Pulmonary Manifestations in Connective Tissue Diseases
2019, Mayo Clinic ProceedingsCitation Excerpt :Conversely, patients with scleroderma-related PAH fare worse, particularly men older than 60 years with low systemic blood pressure (systolic <110 mm Hg) or 6-minute walk distance (<165 m) and severely abnormal hemodynamics (eg, right atrial pressure of 20 mm Hg).126 Echocardiography may provide a simplified approach to prognostication.127 The presenting features of CTD-ILD comprise a broad spectrum of clinical, imaging, and pathologic patterns.
Risk stratification in adult and pediatric pulmonary arterial hypertension: A systematic review
2022, Frontiers in Cardiovascular Medicine
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.