PAH QuERI Study Statistics
PAH QuERI Study Progress
PAH QuERI Study Progression Updates
Early Discontinuation Status
Upcoming Events
Treatment of PAH
Data on disease-specific treatments
Demographics

Initial PAH Classification
Data on disease-specific treatments
Demographics
Disease Specific Treatments
Diagnostic Work Up of PAH
ACCP-recommended diagnostic tests
Limitations
Needs Assessment Survey

2008 Q3 - QUARTERLY UDPATE

This update will provide new and important data on the diagnostic and therapeutic aspects of the QuERI database that have been presented at the peer-reviewed National meetings this year.


PAH QuERI Study Statistics - site locations

FIRST PATIENT, FIRST VISIT: FIRST PATIENT, LAST VISIT: LAST PATIENT, FIRST VISIT: LAST PATIENT, LAST VISIT: 16 August, 2005 16 Nov, 2008 05 July, 2007 05 October, 2010 Out of 807 patients enrolled, 95% completed baseline visit, 81% completed 6 month follow-up visit, 72% completed 1 year follow-up visit, and 13% completed 2 year follow-up visit (13May08)

Click here to complete a short Needs Assessment Survey

For more information please contact:
Lianne Goldin | email: lgoldin@chrc.net
Tel: 416-977-8010 ext 22

PAH QuERI Study Progression Updates
Study Progression: Follow up visit date
achieved / actual visit completion (13May, 2008)




PAH QuERI Early Discontinuation Status
( 22 April, 2008: Total number of patients early terminated: 159 patients )

Treatment of PAH: Data from the Quality Enhancement Research Initiative
McLaughlin, Langer, Dragomir, Casanova, Tan, Oudiz, Clements, Tapson, Channick, Rubin

Implementation of the 2004 ACCP Recommendations for the Management of Pulmonary Arterial Hypertension (PAH) was studied in quality enhancement research initiative (QuERI) in PAH 517 patients among 52 US specialists.

Methods: Physicians were asked to enroll PAH patients (known or newly diagnosed) and provide data on their medical management.

Results: PAH was reported as idiopathic in 37%, familial in 3%, had associated conditions in 50% as follows: connective tissue disorders in 28%, drug exposure in 9%, congenital systemic-to-pulmonary shunt in 7%, portal hypertension in 4%, HIV in 3% and associated with significant venous or capillary involvement in <1%. WHO class was available in 471 patients: 42 were class I (9%), 182 class II (39%), 222 class III (47%), and 25 class IV (5%).

Data on disease-specific treatments was available in 450 patients (87%):

All of these therapies were more frequently used as symptom class worsened. Among 96 patients on CCB, only 27 had acute vasoreactivity testing performed; only 6 of these were reported to have ACCP-defined vasoreactivity (22% of those tested for vasoreactivity and only 6.3% of the total population). Recommended PAH therapy of anticoagulants (warfarin) was used in 38% of class II, 52% of class III and 44% of class IV (44% overall).

Diuretics were used in 40%, 53%, and 72% of class II, III, and IV, respectively. Modulators of renin system were used in 21% of patients and digoxin in 16%.

Summary and Conclusions: Medical management of PAH is complex with frequent use of multiple therapies. Disease specific therapies and diuretics are used more frequently as symptoms worsened. Vasoreactivity testing to direct CCB treatment was not performed in the majority of patients. Greater adherence to ACCP guidelines for vasoreactivity testing is recommended.

Click here to complete a short Needs Assessment Survey

Background
ACCP Guidelines for diagnosis and management of pulmonary arterial hypertension (PAH) were published in 2004. Applicability and use of these guidelines in general practice remains uncertain.

Purpose
To facilitate access to published PAH guidelines and to integrate them into physician's work environment through the use of electronic case report form as a reminder and a feedback mechanism.

PAH QuERI: Goals

PAH QuERI: Methodology

PAH QuERI: Inclusion Criteria

Results: Population and Variables
800 patients have been enrolled to date, however, the eCRF for only 564 have been signed off by the local PI and these data are presented here.

Diagnostic Work Up of PAH: Data from the Quality Enhancement Research Initiative
Oudiz, Langer, Dragomir, Casanova, Tan, McLaughlin, Clements, Tapson, Channick, Rubin

Implementation of the 2004 ACCP Recommendations for the Diagnosis and Differential Assessment of Pulmonary Arterial Hypertension (PAH) was measured as part of a quality enhancement research initiative (QuERI) in 517 patients with PAH among 52 US specialist physicians.

Methods: Physicians were asked to enroll PAH patients (known or newly diagnosed) and provide data on recommended diagnostic tests.

Results: (Median, 25th and 75th percentile): Patients enrolled were 55 years old (44, 66), 77% female, with a BMI of 27.5 kg/m² (23, 33), BP was 118/70 mmHg (104,130/61,78), and heart rate was 80 bpm (72,92). Most were functional class II or greater (91%) and 40% were using supplemental oxygen. PAH was reported as idiopathic in 37%, familial in 3%,and was associated with conditions in 50% as follows: connective tissue disorders (CTD) in 28%, drug exposure in 9%, congenital systemic-to-pulmonary shunt in 7%, portal hypertension in 4%, HIV in 3% and associated with significant venous or capillary involvement in <1%; the rest having been classified as "other".

WHO class was available in 471 patients: 42 were class I (9%), 182 class II (39%), 222 class III (47%), and 25 class IV (5%).

The frequency of recommended diagnostic tests was: chest x-ray: 82%, ECG: 72%, echocardiogram: 89%, right heart catheterization (RHC): 77%, pulmonary function testing: 77%, and V/Q scanning in only 52%. CTD screen: 53%, HIV: 36%, CBC: 89%, liver function: 93%. All three tests: CXR, ECG, and echocardiogram were performed in 344 out of 517 (67%) patients.

Conclusion: Physicians treating PAH report multiple etiologies requiring a comprehensive and multi-pronged approach to the diagnostic evaluation. This preliminary assessment suggests that certain essential diagnostic tests maybe underutilized: While ACCP guidelines recommend V/Q scanning to exclude correctable causes of PAH, and HIV testing is mandatory in all patients evaluated for PAH, these guidelines are not universally followed. Stricter adherence to guidelines may result in more optimal management of these high-risk patients.