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. 2020 Feb 15;201(4):458-468.
doi: 10.1164/rccm.201909-1771OC.

Identification of Cardiac Magnetic Resonance Imaging Thresholds for Risk Stratification in Pulmonary Arterial Hypertension

Robert A Lewis  1   2 , Christopher S Johns  2 , Marcella Cogliano  2 , David Capener  2 , Euan Tubman  2 , Charlie A Elliot  1 , Athanasios Charalampopoulos  1 , Ian Sabroe  1   2 , A A Roger Thompson  1   2 , Catherine G Billings  1   2 , Neil Hamilton  1 , Kathleen Baster  3 , Peter J Laud  3 , Peter M Hickey  1   2 , Jennifer Middleton  1   2 , Iain J Armstrong  1 , Judith A Hurdman  1 , Allan Lawrie  2 , Alexander M K Rothman  1   2   4 , Jim M Wild  2 , Robin Condliffe  1 , Andrew J Swift  2   4 , David G Kiely  1   2   4
Affiliations

Identification of Cardiac Magnetic Resonance Imaging Thresholds for Risk Stratification in Pulmonary Arterial Hypertension

Robert A Lewis et al. Am J Respir Crit Care Med. .

Abstract

Rationale: Pulmonary arterial hypertension (PAH) is a life-shortening condition. The European Society of Cardiology and European Respiratory Society and the REVEAL (North American Registry to Evaluate Early and Long-Term PAH Disease Management) risk score calculator (REVEAL 2.0) identify thresholds to predict 1-year mortality.Objectives: This study evaluates whether cardiac magnetic resonance imaging (MRI) thresholds can be identified and used to aid risk stratification and facilitate decision-making.Methods: Consecutive patients with PAH (n = 438) undergoing cardiac MRI were identified from the ASPIRE (Assessing the Spectrum of Pulmonary Hypertension Identified at a Referral Center) MRI database. Thresholds were identified from a discovery cohort and evaluated in a test cohort.Measurements and Main Results: A percentage-predicted right ventricular end-systolic volume index threshold of 227% or a left ventricular end-diastolic volume index of 58 ml/m2 identified patients at low (<5%) and high (>10%) risk of 1-year mortality. These metrics respectively identified 63% and 34% of patients as low risk. Right ventricular ejection fraction >54%, 37-54%, and <37% identified 21%, 43%, and 36% of patients at low, intermediate, and high risk, respectively, of 1-year mortality. At follow-up cardiac MRI, patients who improved to or were maintained in a low-risk group had a 1-year mortality <5%. Percentage-predicted right ventricular end-systolic volume index independently predicted outcome and, when used in conjunction with the REVEAL 2.0 risk score calculator or a modified French Pulmonary Hypertension Registry approach, improved risk stratification for 1-year mortality.Conclusions: Cardiac MRI can be used to risk stratify patients with PAH using a threshold approach. Percentage-predicted right ventricular end-systolic volume index can identify a high percentage of patients at low-risk of 1-year mortality and, when used in conjunction with current risk stratification approaches, can improve risk stratification. This study supports further evaluation of cardiac MRI in risk stratification in PAH.

Keywords: disease severity; imaging; prognosis; pulmonary arterial hypertension; risk stratification.

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Figures

Figure 1.
Figure 1.
Flowchart showing patients included and reasons for exclusion. mPAP = mean pulmonary artery pressure; MRI = magnetic resonance imaging; PAWP = pulmonary arterial wedge pressure; RHC = right heart catheterization.
Figure 2.
Figure 2.
Histograms displaying quintile groups and percentage of mortality at 1 year for cardiac magnetic resonance imaging variables (discovery). Blue dashed lines represent division into categories of risk. *Division into risk categories was not performed. %pred = percentage predicted for age and sex; LVEDVi = left ventricular end-diastolic volume index; RVEDVi = right ventricular end-diastolic volume index; RVEF = right ventricular ejection fraction; RVESVi = right ventricular end-systolic volume index.
Figure 3.
Figure 3.
Histograms displaying percentage mortality at 1 year for derived thresholds for cardiac magnetic resonance imaging variables (test cohort). % pred = percentage predicted for age and sex; LVEDVi = left ventricular end-diastolic volume index; RVEF = right ventricular ejection fraction; RVESVi = right ventricular end-systolic volume index.
Figure 4.
Figure 4.
(A–F) Kaplan–Meier survival curves for RVESVi %pred at baseline (test cohort) (A), RVESVi %pred at follow-up cardiac magnetic resonance imaging (MRI) (B), transition of risk between baseline and follow-up cardiac MRI for RVESVi %pred (C), RVEF at baseline (test cohort) (D), RVEF at follow-up cardiac MRI (E), and transition of risk between baseline and follow-up cardiac MRI for RVEF (F). %pred = percentage predicted for age and sex; MRI = magnetic resonance imaging; RVEF = right ventricular ejection fraction; RVESVi = right ventricular end-systolic volume index.
Figure 5.
Figure 5.
Impact of adding cardiac magnetic resonance imaging to REVEAL 2.0 and a modified FPHR (French Pulmonary Hypertension Registry) approach to European Society of Cardiology and European Respiratory Society guidelines for risk stratification. Green indicates low risk of 1-year mortality (<5%), amber indicates intermediate risk (5–10%), and red indicates high risk (>10%). Mod. = modified; MRI = magnetic resonance imaging; PAH = pulmonary arterial hypertension; REVEAL = North American Registry to Evaluate Early and Long-Term PAH Disease Management; RVESVi% = percentage-predicted right ventricular end-systolic volume index.
Figure 6.
Figure 6.
Receiver operating characteristic curves showing potential added value of cardiac magnetic resonance imaging in conjunction with the REVEAL 2.0 risk score calculator (left) and modified FPHR (French Pulmonary Hypertension Registry) approach to risk stratification (right), using an RVESVi percentage-predicted threshold of 227%. %pred = percentage predicted for age and sex; REVEAL = North American Registry to Evaluate Early and Long-Term PAH Disease Management; ROC = receiver operating characteristic; RVESVi = right ventricular end-systolic volume index.
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