Cardiac magnetic resonance findings predicting mortality in patients with pulmonary arterial hypertension: a systematic review and meta-analysis

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Abstract

Objectives

To provide a comprehensive overview of all reported cardiac magnetic resonance (CMR) findings that predict clinical deterioration in pulmonary arterial hypertension (PAH).

Methods

MEDLINE and EMBASE electronic databases were systematically searched for longitudinal studies published by April 2015 that reported associations between CMR findings and adverse clinical outcome in PAH. Studies were appraised using previously developed criteria for prognostic studies. Meta-analysis using random effect models was performed for CMR findings investigated by three or more studies.

Results

Eight papers (539 patients) investigating 21 different CMR findings were included. Meta-analysis showed that right ventricular (RV) ejection fraction was the strongest predictor of mortality in PAH (pooled HR 1.23 [95 % CI 1.07–1.41], p = 0.003) per 5 % decrease. In addition, RV end-diastolic volume index (pooled HR 1.06 [95 % CI 1.00–1.12], p = 0.049), RV end-systolic volume index (pooled HR 1.05 [95 % CI 1.01–1.09], p = 0.013) and left ventricular end-diastolic volume index (pooled HR 1.16 [95 % CI 1.00–1.34], p = 0.045) were of prognostic importance. RV and LV mass did not provide prognostic information (p = 0.852 and p = 0.983, respectively).

Conclusion

This meta-analysis substantiates the clinical yield of specific CMR findings in the prognostication of PAH patients. Decreased RV ejection is the strongest and most well established predictor of mortality.

Key Points

• Cardiac magnetic resonance imaging is useful for prognostication in pulmonary arterial hypertension .

• Right ventricular ejection fraction is the strongest predictor of mortality.

• Serial CMR evaluation seems to be of additional prognostic importance.

• Accurate prognostication can aid in adequate and timely intensification of PAH- specific therapy.

Electronic supplementary material

The online version of this article (doi:10.1007/s00330-016-4217-6) contains supplementary material, which is available to authorized users.

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Most cited references 34

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To characterize mortality in persons diagnosed with primary pulmonary hypertension and to investigate factors associated with survival. Registry with prospective follow-up. Thirty-two clinical centers in the United States participating in the Patient Registry for the Characterization of Primary Pulmonary Hypertension supported by the National Heart, Lung, and Blood Institute. Patients (194) diagnosed at clinical centers between 1 July 1981 and 31 December 1985 and followed through 8 August 1988. At diagnosis, measurements of hemodynamic variables, pulmonary function, and gas exchange variables were taken in addition to information on demographic variables, medical history, and life-style. Patients were followed for survival at 6-month intervals. The estimated median survival of these patients was 2.8 years (95% Cl, 1.9 to 3.7 years). Estimated single-year survival rates were as follows: at 1 year, 68% (Cl, 61% to 75%); at 3 years, 48% (Cl, 41% to 55%); and at 5 years, 34% (Cl, 24% to 44%). Variables associated with poor survival included a New York Heart Association (NYHA) functional class of III or IV, presence of Raynaud phenomenon, elevated mean right atrial pressure, elevated mean pulmonary artery pressure, decreased cardiac index, and decreased diffusing capacity for carbon monoxide (DLCO). Drug therapy at entry or discharge was not associated with survival duration. Mortality was most closely associated with right ventricular hemodynamic function and can be characterized by means of an equation using three variables: mean pulmonary artery pressure, mean right atrial pressure, and cardiac index. Such an equation, once validated prospectively, could be used as an adjunct in planning treatment strategies and allocating medical resources.

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Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL).

Raymond Benza, Dave Miller, Mardi Gomberg-Maitland … (2010)

Factors that determine survival in pulmonary arterial hypertension (PAH) drive clinical management. A quantitative survival prediction tool has not been established for research or clinical use. Data from 2716 patients with PAH enrolled consecutively in the US Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) were analyzed to assess predictors of 1-year survival. We identified independent prognosticators of survival and derived a multivariable, weighted risk formula for clinical use. One-year survival from the date of enrollment was 91.0% (95% confidence interval [CI], 89.9 to 92.1). In a multivariable analysis with Cox proportional hazards, variables independently associated with increased mortality included pulmonary vascular resistance >32 Wood units (hazard ratio [HR], 4.1; 95% CI, 2.0 to 8.3), PAH associated with portal hypertension (HR, 3.6; 95% CI, 2.4 to 5.4), modified New York Heart Association/World Health Organization functional class IV (HR, 3.1; 95% CI, 2.2 to 4.4), men >60 years of age (HR, 2.2; 95% CI, 1.6 to 3.0), and family history of PAH (HR, 2.2; 95% CI, 1.2 to 4.0). Renal insufficiency, PAH associated with connective tissue disease, functional class III, mean right atrial pressure, resting systolic blood pressure and heart rate, 6-minute walk distance, brain natriuretic peptide, percent predicted carbon monoxide diffusing capacity, and pericardial effusion on echocardiogram all predicted mortality. Based on these multivariable analyses, a prognostic equation was derived and validated by bootstrapping technique. We identified key predictors of survival based on the patient's most recent evaluation and formulated a contemporary prognostic equation. Use of this tool may allow the individualization and optimization of therapeutic strategies. Serial follow-up and reassessment are warranted. Clinical Trial Registration- URL: http://www.clinicaltrials.gov. Unique identifier: NCT00370214.

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Author and article information

Contributors

Gertjan Tj. Sieswerda: +31 (0) 88 755 3688 , G.T.Sieswerda@umcutrecht.nl

Journal

Journal ID (nlm-ta): Eur Radiol

Journal ID (iso-abbrev): Eur Radiol

Title: European Radiology

Publisher: Springer Berlin Heidelberg (Berlin/Heidelberg )

ISSN (Print): 0938-7994

ISSN (Electronic): 1432-1084

Publication date (Electronic): 4 February 2016

Publication date PMC-release: 4 February 2016

Publication date (Print): 2016

Volume: 26

Issue: 11

Pages: 3771-3780

Affiliations

[1 ]Department of Cardiology, AHMaZON Centre for Adult Congenital Heart Disease, University Medical Centre Utrecht, Radboud University Medical Centre Nijmegen and St. Antonius Hospital Nieuwegein, PO Box 85500, 3508 GA Utrecht, The Netherlands

[2 ]Department of Cardiology, Erasmus Medical Centre, Rotterdam, The Netherlands

[3 ]Department of Radiology, University Medical Centre Utrecht, Utrecht, The Netherlands

[4 ]Department of Clinical Epidemiology, Erasmus Medical Centre, Rotterdam, The Netherlands

Article

Publisher ID: 4217

DOI: 10.1007/s00330-016-4217-6

PMC ID: 5052291

PubMed ID: 26847041

SO-VID: c8298221-1889-48eb-8558-ae6a1f6b5776

License:

Open Access This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License (http://creativecommons.org/licenses/by-nc/4.0/), which permits any noncommercial use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.

History

Date received : 17 August 2015

Date revision received : 4 January 2016

Date accepted : 13 January 2016

Custom metadata

ScienceOpen disciplines: Radiology & Imaging

Keywords: pulmonary arterial hypertension,prognosis,magnetic resonance imaging,meta-analysis,mortality

Data availability:

ScienceOpen disciplines: Radiology & Imaging

Keywords: pulmonary arterial hypertension, prognosis, magnetic resonance imaging, meta-analysis, mortality

Cardiac magnetic resonance findings predicting mortality in patients with pulmonary arterial hypertension: a systematic review and meta-analysis

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Abstract

Objectives

Methods

Results

Conclusion

Key Points

Electronic supplementary material

Related collections

Stroke MRI

Most cited references 34

Survival in patients with primary pulmonary hypertension. Results from a national prospective registry.

ESC Guidelines for the management of grown-up congenital heart disease (new version 2010).

Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL).

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