Better Risk Assessment Needed to Improve Clinical Care in PAH, Survey Says - Pulmonary Hypertension News

Better Risk Assessment Needed to Improve Clinical Care in PAH, Survey Says - Pulmonary Hypertension News


Better Risk Assessment Needed to Improve Clinical Care in PAH, Survey Says - Pulmonary Hypertension News

Posted: 31 Jul 2019 06:00 AM PDT

While physicians worldwide are conducting multi-parametric risk assessments to evaluate pulmonary arterial hypertension (PAH), they are not doing so to the extent recommended by current guidelines, results from an international survey show.

The survey found that PAH evaluations in clinical practice were not performed to the levels recommended by the European Society of Cardiology (ESC) and the European Respiratory Society (ERS).

These findings were reported in the study "Assessment of Risk of Disease Progression in Pulmonary Arterial Hypertension: Insights from an International Survey of Clinical Practice," published in the journal Advances in Therapy.

PAH is a progressive and debilitating disease characterized by increased pulmonary vascular resistance and pulmonary arterial pressure. With no cure available, PAH can lead to right heart failure, and ultimately death.

Given its severity and life-threatening potential, it is crucial to identify patients who may be at risk to develop PAH. That allows those diagnosed to start treatment early to prevent the fast progression of the disease.

Based on growing clinical evidence, leading PAH experts have recommended regular multi-parametric risk assessment to predict patients' outcomes, and establish the best treatment course. Although the guidelines clearly "mandate for risk assessment in the management of PAH," it remains unclear how this is being implemented in clinical practice.

Cello Health, in collaboration with Actelion Pharmaceuticals, conducted an international survey to explore this issue.

The study included 90 physicians — of whom 52 were cardiologists, and 38 pulmonologists — in France, Germany, Italy, and the U.S. The physicians provided clinical information about 623 people with PAH, of which 53.6% cases were of unknown cause (idiopathic PAH) and 30.5% were associated with another condition. A total 9.1% of patients had PAH induced by a medicine or toxin, and 6.7% of the cases were of genetic nature.

In the survey, researchers evaluated how often physicians evaluated all nine parameters required for risk assessment. These include clinical signs of right heart failure, progression of symptoms, syncope (sudden drop of blood pressure), heart imaging, and blood flow dynamics (hemodynamics). The other parameters are World Health Organization (WHO) functional class, exercise capacity assessed by the 6-minute walk distance test (6MWD) and cardiopulmonary exercise testing (CPET), and NT-proBNP plasma levels.

Physicians most commonly evaluated progression of symptoms (91%), according to their survey responses. The CPET parameter was less assessed, at 54%, the survey showed.

In addition, the risk assessments — done by evaluation of the different parameters — were performed more frequently at an initial consultation than at any follow-up visit, the physicians reported.

Comparison of physicians' risk assessment and calculated risk scores, as defined in the ESC and ERS guidelines based on all parameters available, showed that they were both able to identify PAH patients at intermediate risk.

In contrast, there was poor concordance between the two risk assessment approaches concerning low- and high-risk groups. Among patients judged to be at low risk by physicians, approximately 80% were calculated as being at higher risk. Meanwhile, 69% of the individuals categorized as high risk by physicians were calculated as being at low risk.

These results were independent of the physicians' geographical locations. Indeed, physicians located in Europe and in the U.S. showed similar characterization trends.

Overall, the findings demonstrate that "there are differences between physicians' … judgement of patient risk, and patient risk as calculated using an objective algorithm," the researchers said.

It remains unclear what could be contributing to the lack of agreement. However, the survey team believes the differences could be due, at least in part, to the reduced number of parameters that are being measured and used by physicians to accurately stratify PAH risk.

In addition, physicians may be using thresholds from parameters different to those recommended in the guidelines. They also may be adding additional information — such as a patient's age, gender, and comorbidities, or other disorders — to build their risk assessment profile.

The survey showed that "multi-parametric risk assessment is being performed in clinical practice." However, it is not always applied to "the extent recommended in the 2015 ESC/ERS guidelines."

Pulmonary Arterial Pressure Above 30 mmHg Linked to Increased Mortality - Pulmonary Hypertension News

Posted: 08 Jul 2019 12:00 AM PDT

An estimated right ventricular systolic pressure (eRVSP) — a measure of the pressure inside the artery that supplies blood to the lungs (pulmonary artery) — greater than 30.0 mmHg is related to an increased risk of mortality in patients with pulmonary hypertension (PH), according to a new Australian study.

The study, "Threshold of Pulmonary Hypertension Associated With Increased Mortality," was published in the Journal of the American College of Cardiology.

Currently, there are significant limitations in therapies available to patients with PH. Consequently, a diagnosis of PH is associated with poor long-term outcomes. Even patients with mild PH are at an increased risk of mortality — one study found that mild PH patients have 1.52 times the risk of mortality compared to the general population.

Borderline PH is defined as having an average pulmonary arterial pressure between 20 and 25 mmHg. Given the rapid progression of borderline PH to clinically define PH (defined as pulmonary arterial pressure greater than 25 mmHg), researchers are starting to recognize the importance of identifying patients who are at increased risk of progressing, and those who could benefit from disease-specific therapies.

Hence, there is a need to identify "optimal cutoffs," or thresholds, for pulmonary arterial pressure that can be easily used to identify at-risk populations with PH. "There is increasing evidence that current thresholds for diagnosing pulmonary hypertension underestimate the prognostic impact of PH," the researchers said.

The researchers conducted a study (ACTRN12617001387314) to determine the long-term outcomes and prognostic impact of increasing pulmonary pressures in patients with PH within the National Echocardiography Database of Australia cohort.

Echocardiography is the first-line diagnostic and screening tool for PH, as it can help assess eRVSP levels. Using echocardiography, researchers were able to examine the association between short- and long-term mortality with increasing pulmonary pressures.

Researchers evaluated eRVSP levels for 74,405 men and 83,437 women who were, on average, 65.6 years of age.

Overall, results indicated that 17,955 (11.4%) patients had mild PH (40-49 mmHg), 7,016 (4.4%) patients had moderate PH (50-59 mmHg), and 4,515 (2.9%) patients had severe PH (60 mmHg or more).

Researchers confirmed previous data by showing that 9.4% of patients with eRVSPs higher than 40 mmHg had almost three times the risk for 5-year mortality. Additionally, an increased mortality risk in patients with mild or borderline PH was found.

After conducting statistical analysis and adjusting the results for age, sex, and evidence of left heart disease, researchers were able to establish a clear and consistent threshold of increased mortality that corresponded to an eRVSP of 30.0 mmHg or above.

In fact, patients with eRVSP levels between 30.0-39.9 mmHg were, after adjusting for other factors, 1.4 to 1.9 times more likely to die compared to patients with lower eRVSP levels.

"Our unique findings suggest that those currently considered to be at intermediate risk for underlying PH have a significant risk for mortality once they reach the threshold of eRVSP [higher than] 30.0 mm Hg," the researchers concluded.

The team said that "future studies should characterize patients with borderline PH on the basis of eRVSP to determine the mechanisms of excess mortality, and evaluate the efficacy of therapeutic interventions to prolong survival."

Inoperable Chronic Thromboembolic Pulmonary Hypertension: BPA vs Riociguat - Pulmonology Advisor

Posted: 04 Jul 2019 12:00 AM PDT

Compared with riociguat therapy, balloon pulmonary angioplasty (BPA) may be associated with greater improvements in exercise tolerance and pulmonary hemodynamics, with the exception of cardiac index and cardiac output, according to a study published in Clinical Cardiology. Overall, both therapies were well tolerated, but findings should be confirmed by additional studies.

This meta-analysis compared the safety and efficacy of BPA with riociguat therapy in patients with inoperable chronic thromboembolic pulmonary hypertension. Searches in the ClinicalTrials.gov, Embase, and PubMed databases found 23 clinical trials of these treatments, with a total 1454 participants (BPA; n=631 and riociguat; n=823). The Newcastle-Ottawa Scale for the assessment of nonrandomized study quality in meta-analyses was used to assess risk for bias.

When compared with riociguat therapy, BPA was associated with greater improvements in right atrial pressure (mean difference [MD], -3.53 mm Hg [95% CI, -4.85 to -2.21 mm Hg] vs MD, -1.05 mm Hg [95% CI, -1.82 to -0.29 mm Hg]), mean pulmonary artery pressure (MD, -15.02 mm Hg [95% CI, -17.32 to −12.71 mm Hg] vs MD, -4.19 mm Hg [95% CI, -5.58 to -2.80 mm Hg]), pulmonary vascular resistance (standard MD, -1.32 Woods [95% CI, -1.57 to -1.08 Woods] vs standard MD, -0.65 Woods [95% CI, -0.79 to -0.50 Woods]), New York Heart Association functional class (RR, 6.78 [95% CI, 3.14-14.64] vs RR, 1.49 [95% CI, 1.07-2.07]), and 6-minute walk test distance (MD, 71.66 m [95% CI, 58.34-84.99 m] vs MD, 45.25 m [95% CI, 36.51-53.99 m]).

However, the increase in cardiac output was greater with riociguat than with BPA (MD, 0.78 L/min [95% CI, 0.61-0.96 L/min] vs MD, 0.33 L/min [95% CI, 0.06-0.59 L/min]), and there was no significant difference in cardiac index between BPA and riociguat (MD, 0.40 L/min/m2 [95% CI, 0.21-0.58 L/min/m2] vs MD, 0.40 L/min/m2 [95% CI, 0.26-0.54 L/min/m2]).

The most common BPA complications were pulmonary edema (0.8%-28.6%) and pulmonary injury (0.3%-5.6%), and the most common riociguat adverse events were dizziness, headache, hypotension, and nasopharyngitis.

The study investigators concluded, "Overall, both BPA and riociguat were well tolerated. However, our findings need to be confirmed with further multicenter randomized control trials…and prospective observational studies."

Reference

Wang W, Wen L, Song Z, Shi W, Wang K, Huang W. Balloon pulmonary angioplasty vs riociguat in patients with inoperable chronic thromboembolic pulmonary hypertension: a systematic review and meta-analysis [published online June 12, 2019]. Clin Cardiol. doi:10.1002/clc.23212

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This article originally appeared on The Cardiology Advisor

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