Abstract
Providing prognostic information is important when counseling patients and planning treatment strategies in chronic thromboembolic pulmonary hypertension (CTEPH). The aim of this study was to assess the prognostic value of gold standard imaging of cardiac structure and function using cardiac magnetic resonance imaging (CMR) in CTEPH. Consecutive treatment-naive patients with CTEPH who underwent right heart catheterization and CMR between 2011 and 2017 were identified from the ASPIRE (Assessing-the-Specturm-of-Pulmonary-hypertensIon-at-a-REferral-center) registry. CMR metrics were corrected for age and sex where appropriate. Univariate and multivariate regression models were generated to assess the prognostic ability of CMR metrics in CTEPH. Three hundred and seventy-five patients (mean+/-standard deviation: age 64+/-14 years, 49% female) were identified and 181 (48%) had pulmonary endarterectomy (PEA). For all patients with CTEPH, left-ventricular-stroke-volume-index-%predicted (LVSVI%predicted) (p = 0.040), left-atrial-volume-index (LAVI) (p = 0.030), the presence of comorbidities, incremental shuttle walking test distance (ISWD), mixed venous oxygen saturation and undergoing PEA were independent predictors of mortality at multivariate analysis. In patients undergoing PEA, LAVI (p < 0.010), ISWD and comorbidities and in patients not undergoing surgery, right-ventricular-ejection-fraction-%predicted (RVEF%pred) (p = 0.040), age and ISWD were independent predictors of mortality. CMR metrics reflecting cardiac function and left heart disease have prognostic value in CTEPH. In those undergoing PEA, LAVI predicts outcome whereas in patients not undergoing PEA RVEF%pred predicts outcome. This study highlights the prognostic value of imaging cardiac structure and function in CTEPH and the importance of considering left heart disease in patients considered for PEA.
Highlights
Pulmonary hypertension (PH) is heterogeneous and treatment depends on the underlying cause [1]
We have highlighted the importance of considering left heart disease in patients considered for pulmonary endarterectomy (PEA), by showing the prognostic value of LAVI in patients undergoing PEA, whilst confirming the value of established prognostic markers of right ventricular (RV) function in patients with chronic thromboembolic pulmonary hypertension (CTEPH) who do not undergo PEA
In this study we have shown that a number of measures reflecting RV function, including RVESVI%pred which is strongly prognostic in pulmonary arterial hypertension (PAH) [15, 25] and RVEF (%pred), were predictors of mortality in the whole cohort (Supplementary Table 1) and in those not undergoing PEA (Table 5), at univariate analysis
Summary
Pulmonary hypertension (PH) is heterogeneous and treatment depends on the underlying cause [1]. Chronic thromboembolic pulmonary hypertension (CTEPH) is a potentially curable form of PH and a recent meta-analysis has identified a cumulative incidence of 2.9% in patients surviving an acute pulmonary embolism [2]. It can present as PH with no previous evidence of venous thromboembolism [3]. It is characterized by nonresolution of thrombus and remodeling of the pulmonary arteries resulting in PH and right ventricular (RV) dysfunction [4] and without treatment a poor prognosis. Patients with CTEPH are presenting with comorbidities and additional information that could aid decision making would be helpful
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