Welcome to the 2009 Q4 PAH QuERI Quarterly Update,
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This last issue of the Quarterly Update in 2009 will focus on providing you with an updated summary on the utilization of diagnostic techniques amongst community and academic sites participating in the PAH QuERI.
PAH QuERI is a Quality Enhancement Research Initiative that aims to improve the management of patients with PAH based on the American College of Chest Physicians (ACCP) guidelines. which were published in 2004. .
The steps of the QuERI can be summarized as follows:
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Link guidelines and risk based management with actual patient care
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Monitor outcomes and provide feedback to improve care
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Ensure dynamic (over a period of time) and interactive environment (allowing for change)
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Use data for future research direction and guidelines development
In the PAH QuERI, physicians were asked to enroll existing or newly diagnosed patients with PAH and provide data on recommended diagnostic tests. Participating physicians were also asked to provide six-month and annual follow-up data over three years.
Prior Quarterly Updates have focused on providing summary data on etiology and classification of the enrolled patients, their baseline clinical characteristics and the overall management. Thus, this Quarterly Update will focus specifically on the diagnostic work up and the evidence based approach upon which it is built and compare the use of these diagnostic approaches as seen from the PAH QuERI data base.
The following is the summary of the recommended
non-invasive tests:
One of the least performed tests among those recommended by the ACCP guidelines was the ventilation and perfusion scan (V/Q Scan). This is of particular interest since the V/Q Scan is an inexpensive and widely available diagnostic procedure that can be helpful in identifying a reversible or surgically manageable disease etiology such as thrombo-embolism.
Determinants of survival in PAH are:
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Etiology
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Severity of hemodynamic abnormalities
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Exercise capacity
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Echocardiographic Indices
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Biomarkers
A few of the guideline mandated diagnostic tests that have been shown to provide prognostic value were performed frequently, such as echocardiography:
On the other hand, it is interesting to note that the exercise capacity as measured by the six minute walk was performed in only three quarters of the patients despite clear evidence of its importance for risk stratification: + click to view larger
Another interesting and apparent underutilization of diagnostic testing is related to the use of serum biomarkers. Survival in patients with PAH secondary to scleroderma is known to be particularly poor as shown below:
However, as can be seen from the results below, the testing for connective tissue disease was done in only about half of the patients. Moreover, a test for a significant etiology such as HIV was done in only about a third of the patients despite clear guidelines and widespread availability of the testing technology. + click to view larger
It is also important to note that the use of other serum biomarkers for testing has been captured in the PAH QuERI database, including BNP and Tn which have been demonstrated to have a prognostic value in patients with PAH as shown below. These findings will be reported in future Quarterly Updates.
It is important to acknowledge the limitations of the PAH QuERI related observations:
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Physician and patient enrollment bias
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Self-reported data
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Reporting influenced by the data entry mechanism itself
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Experience limited to mostly community sites
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Dynamic pattern of data in relation to practice enhancement aspect of the program (main purpose of QuERI)
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Study design does not allow evaluation of a cause-effect relationship
In Summary:
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PAH management requires a comprehensive and multi-pronged diagnostic evaluation
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This preliminary PAH QuERI assessment suggests that certain essential diagnostic tests such as HIV testing, CTD screening, and V/Q for excluding a treatable cause of pulmonary hypertension are underutilized
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Given the treatment implications of a positive HIV test or V/Q scan, stricter adherence to guidelines may result in more optimal management of these high-risk patients.
In Conclusion:
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Despite obvious and significant limitations, the PAH QuERI database allows for a real world assessment of the diagnosis, management and outcomes in PAH patients
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When PH exists, an appropriate diagnostic evaluation must be done, with consideration for several etiologies, guided by specific clinical circumstances
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Greater adherence to guidelines may improve outcomes in these high-risk patients.
Introduction 
Needs Assessment Survey 
