Better Risk Assessment Needed to Improve Care in PAH, International Survey Shows

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.

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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.”