Cardiac MRI Metrics Help Stratify Mortality Risk in Pulmonary Arterial Hypertension - Pulmonology Advisor

Cardiac MRI Metrics Help Stratify Mortality Risk in Pulmonary Arterial Hypertension - Pulmonology Advisor

Cardiac MRI Metrics Help Stratify Mortality Risk in Pulmonary Arterial Hypertension - Pulmonology Advisor

Posted: 06 Nov 2019 03:30 AM PST

In patients with pulmonary arterial hypertension (PAH), cardiac magnetic resonance imaging (MRI) metrics are capable of identifying individuals at low risk for 1-year mortality, making cardiac MRI an effective additive risk stratification tool in this population, according to study results published in the American Journal of Respiratory and Critical Care Medicine.

The ASPIRE (Assessing the Spectrum of Pulmonary Hypertension Identified at a Referral Centre) cardiac MRI database was used to identify consecutive patients with PAH (n=438) undergoing cardiac MRI between April 2012 and March 2017. After imaging, operators who were blinded to patient diagnoses and cardiac catheter results analyzed imaging. Investigators identified thresholds in a discovery cohort, which were then assessed in a test cohort.

Cardiac MRI parameters were evaluated in the discovery cohort for their associated risk for 1-year mortality. Both univariate and multivariate analyses of demographics, hemodynamics, and MRI parameters were performed among the whole cohort. To determine whether cardiac MRI could be an additional risk stratification tool, the researchers also calculated the REVEAL 2.0 Risk Score and number of low-risk criteria by the French Pulmonary Hypertension Registry in the incident population. Electronic data from the National Health Service Personal Demographics Service provided mortality data.

A total of 63% of patients were stratified as low risk (<5%) of 1-year mortality, using a right ventricular end systolic volume index percentage predicted threshold of 227%. A left ventricular end diastolic volume index of 58 mL/m2 was also capable of identifying 34% of patients at low risk for 1-year mortality. Several right ventricular ejection fraction (RVEF) thresholds were also established for identifying 1-year mortality risk.

An RVEF of >54% identified 21% of patients as low risk, an RVEF of 37% to 54% identified 43% of patients as intermediate risk, and an RVEF of <37% identified 36% of patients as high risk. The use of right ventricular end systolic volume index percent predicted improved risk stratification for 1-year mortality when used with the REVEAL 2.0 risk score calculator or a modified French Pulmonary Hypertension registry approach.

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Limitations of the study included the relatively small sample size, as well as the lack of external validation of the MRI metrics for predicting 1-year mortality in patients with PAH.

The findings from this study provide clinical relevance for the care of the patients with PAH, the researchers wrote, as the "[i]dentification and maintenance of a low-risk status remains the goal of current treatment strategies."

Reference

Lewis RA, Johns CS, Cogliano M, et al. Identification of cardiac MRI thresholds for risk stratification in pulmonary arterial hypertension [published online October 24, 2019]. Am J Respir Crit Care Med. doi:10.1164/rccm.201909-1771OC

Topics:

Pulmonary Arterial Hypertension Pulmonary Hypertension

Macitentan safe, effective for PAH associated with systemic sclerosis - Healio

Posted: 22 Oct 2019 12:00 AM PDT

NEW ORLEANS — New real-world data demonstrate safety and effectiveness of macitentan for the treatment of pulmonary arterial hypertension associated with systemic sclerosis, with outcomes consistent with those in idiopathic and heritable PAH, researchers reported at the CHEST Annual Meeting.

Using the OPUS Registry and OrPHeUS cohort study, which provide information on patients with PAH who are newly started on macitentan (Opsumit, Actelion Pharmaceuticals), researchers analyzed follow-up data on 659 patients with PAH associated with systemic sclerosis. The researchers then compared outcomes in this population with those of 2,283 patients with idiopathic PAH or heritable PAH.

Treatment discontinuation was reported in 30.2% of patients with PAH associated with systemic sclerosis and 32.8% of patients with idiopathic and heritable PAH. In each group, discontinuation was related to an adverse event in about 17% of patients. Alanine aminotransferase (ALT) or aspartate aminotransferase (AST) three or more times the upper limit of normal occurred in 3.9% and 2.7%, respectively, and ALT/AST three or more times the upper limit of normal and total bilirubin two or more times the upper limit of normal occurred in 0.5% and 0.3%, respectively, according to the results.

At 12 months, the Kaplan-Meier survival estimate was 92% for patients with PAH associated with systemic sclerosis and 93% for those with idiopathic and heritable PAH.

New real-world data demonstrate safety and effectiveness of macitentan for the treatment of pulmonary arterial hypertension associated with systemic sclerosis, with outcomes consistent with those in idiopathic and heritable PAH, researchers reported at the CHEST Annual Meeting.

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One or more hospitalizations occurred in 40% of patients with PAH associated with systemic sclerosis and 36.3% with idiopathic and heritable PAH, according to the results.

At initiation of macitentan, patients with PAH associated with systemic sclerosis had a median age of 64 years, nearly three-quarters had WHO functional class III or IV PAH, 61% were receiving another PAH therapy and 45% had been diagnosed within 6 months. Overall median exposure to macitentan was 14.7 months among patients with PAH associated with systemic sclerosis and 13.2 months in those with idiopathic or heritable PAH.

"These data add to the body of evidence supporting the use of macitentan for the treatment of PAH-systemic sclerosis," Vallerie McLaughlin, MD, FCCP, director of the pulmonary hypertension program and the Kim A. Eagle MD Endowed Professor of Cardiovascular Medicine at University of Michigan, and colleagues concluded. – by Katie Kalvaitis

Reference:

McLaughlin V. Pulmonary Hypertension: Characteristics and Treatment. Presented at: CHEST Annual Meeting; Oct. 19-23, 2019; New Orleans.

Disclosure: McLaughlin reports she consults for Acceleron, Actelion, Arena, Bayer, Caremark and United Therapeutics, and receives research grants from Acceleron, Actelion, Arena, Bayer, Gilead and Sonovie.

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