q108 Q2 Q3 Q4 Q1 2009 Q2 2009 Q3 2009 Q4 2009
 
Welcome to the 2009 Q2 PAH QuERI Quarterly Update,
Please select a category below or scroll down to read the newsletter.
_________________________________________________________________________________


Introduction
Etiology of the PAH among patients
Baseline Characteristics
Functional Class

 


Disease Specific Treatment
Calcium Channel Blockers
Summary


Needs Assessment Survey


 


Q2 2009 Update Treatment of PAH:
Data from the Quality Enhancement
Research Initiative

The real-world implementation of the 2004 ACCP Recommendations for the Management of Pulmonary Arterial Hypertension (PAH) continues to be studied in the PAH Quality Enhancement Research Initiative (QuERI).

The PAH QuERI is a large scale, multi-disciplinary initiative designed to encourage excellence in the long-term PAH care setting. The innovative design of the PAH QuERI integrates guidelines and clinical care delivery to better identify and implement evidence-based practice in routine health care settings. Below is a summary of the processes undertaken.



US medical practitioners (pulmonologists, cardiologists, rheumatologists) were asked to enroll either known or newly diagnosed patients with PAH into the PAH QuERI database and provide data on guideline recommended diagnostic tests. The enrollment is now complete with 787 patients (baseline and up to 1 year follow up data). The figures summarized here represent the most recent data cut after completion of the second quarter of this year. These findings have recently been presented as an abstract at the 2009 American College of Cardiology conference in Orlando:

1.

McLaughlin VV, Langer A, Dragomir A, Casanova A, Altshuler M, Oudiz RJ, Mathier M, Clements P, Tapson VF, Channick RN, Rubin LJ. Treatment of pulmonary arterial hypertension: Data from the Quality Enhancement Research Initiative. J Am Coll Cardiol 2009;53(10, Suppl. A):A455.
2.

Mathier MA, Oudiz RJ, Langer A, Dragomir A, Casanova A, Altshuler M, Clements P, Tapson VF, Channick RN, Rubin LJ, McLaughlin VV. Mortality in patients with pulmonary arterial hypertension in the modern era: Data from the quality enhancement research initiative. J Am Coll Cardiol 2009;53(10, Suppl. A):A449.


The table below summarizes the etiology of the PAH among those patients who were enrolled and have been followed in the QuERI.

Etiology

 N=787
Idiopathic 290 (37.3%)
Familial 18 (2.3%)
Associated conditions 466 (59.9%)
- Connective tissue 236 (30.3%)
- Congenital shunt 55 (7.1%)
- Portal hypertension 30 (3.9%)
- HIV infection 31 (4.0%)
- Drug exposure 58 (7.5%)
- Other associated conditions 98 (12.6%)
Primary venous or capillary involvement 4 (0.5%)


The baseline characteristics of these patients are summarized in the table below.

Parameter Median (25th, 75th), %
Age, y 55 (45, 66)
Female 78%
BMI, kg/mē 27.6 (23.3, 33.1)
BP, mmHg SBP
DBP 		118 (104,130)
70 (61,78)
HR, bpm 81 (72,92)
> WHO functional class II 91%
Supplemental oxygen use 44%


The functional class in these patients as recorded at baseline and more recently at 6 months of follow up was:

Class Enrolment n=787 6 Month n = 629
I 8.6% 10.1%
II 38.8% 41.3%
III 47.5% 43.7%
IV 5.1% 4.9%

Thus, comparing the changes in the functional class from baseline to six months, we observed the following:

Changed from Baseline to
6 months		 N (%)
Same 319/478 (66.7%)
Improved 90/478 (18.8%)
Worsened 69/478 (14.4%)

Next Table >> Disease Specific Treatment

 





The disease specific treatment is shown at baseline and during follow up as monotherapy, followed by a combination therapy, and finally as an overall use.

  Baseline Follow Up
Monotherapy n = 787 n =669
PGI2 59 (7.5%) 33 (4.9%)
PDE5i 87 (11.1%) 64 (9.6%)
ERA 187 (23.8%) 140 (20.9%)

  Baseline Follow Up
Combination Therapy n = 787 n =669
PGI2 and PDE5i 71 (9.0%) 76 (11.8%)
PGI2 and ERA 66 (8.4%) 72 (10.8%)
PDE5i and ERA 94 (11.9%) 119 (17.8%)
PGI2 and PDE5i and ERA 64 (8.1%) 105 (15.7%)

  Baseline Follow Up
Overall use n = 787 n =669
PGI2 (%) 260 (33.0%) 286 (42.8%)
PDE5i 316 (40.2%) 364 (54.4%)
ERA 411 (52.2%) 436 (65.2%)
Any one above 628 (79.8%) 609 (91.0%)



We then focused on 166 patients who were treated with calcium channel blockers to assess whether a vasoreactivity testing as recommended by the ACCP 2004 guidelines was performed in these patients.

The results were as follows:

  n (%)
Right Heart Cath 150 (90.4)
Vasoreactivity test done 55 (33.1)
Vasoreactivity test positive(*) 11 (20.0)
  (*) mPAP decrease >=10 mmHg and mPAP at maximum dose of vasodilator <=40 mmHg and cardiac output (litres/min) unchanged or with positive increase; proportion out of patients with vasoreactivity test done

It is important to note that out of 166 patients on calcium channel blockers, 82 (49.4%) were being treated specifically for PAH (10.4% of all patients). Thus, if we only focus on these 82 patients we found the following results:

  n (%)
Right Heart Cath 77 (93.9)
Vasoreactivity test done 29 (35.4)
Vasoreactivity test positive(*) 9 (31.0)



Importantly, therefore, of those on calcium channel blockers as a PAH specific therapy (n=82), only 9 (11.0%) had vasoreactivity test positive.

Recommended PAH therapy of anticoagulants (warfarin) was used in 21.9% of class I, 24.8% of class II, 29.2% of class III and only 17.1% of class IV (26.2% overall).

Diuretics were used in 26.6% of class I, 30% of class II, 39.1% of class III and 34.3% of class IV. Modulators of the renin system were used in 8.4% of patients and digoxin in 10.5%.

As always, it is important to acknowledge the following limitations when looking at this data:

•

Physician and patient enrollment bias
• Self-reported data
• Reporting influenced by the data entry mechanism itself
• Experience limited to mostly community sites
• Dynamic pattern of data in relation to practice enhancement aspect of the program (main purpose of QuERI)
• Study design does not allow evaluation of a cause-effect relationship

Summary

In summary, most patients with PAH remain in functional class II and III over a short period of follow up as reported so far. Calcium channel blockers are still used in a sizable number of patients even though only a fraction of patients in whom calcium channel blockers are used specifically for PAH meet the criteria for a responder as recommended by the ACCP 2004 Guidelines. Additionally, the use of other generally recommended therapies such as diuretics (50%) and warfarin (40%) was lower than expected.

Click here to complete a short
Needs Assessment Survey