Welcome to the 2010 Quarterly Update,
Please select a category below or scroll down to read the newsletter.
_________________________________________________________________________________
Important Results from
Published Registry Databases
The PAH QuERI is an on-going large program in the United States in the area of pulmonary hypertension with the following overall strategy:
PAH QuERI Quality Enhancement Research Initiative
The following algorithm has been recommended for evaluation of patients with PAH:
•
QuERI is a large scale, multi-disciplinary quality improvement research initiative designed to ensure excellence in long-term care of patients with PAH
•
QuERI represents an innovative integration of guidelines and clinical care delivery, to identify and implement evidence based practices in real life health care settings.
The Quarterly Updates are a useful tool developed for clinicians with an interest in the management of pulmonary hypertension. They contain succinct and relevant updates on new developments and publications in the area of pulmonary hypertension.
Please visit www.mdprimer.com for a web-cast presentation from the 2010 Educational Satellite Symposium entitled:
"Tips and Pearls: Cardiology for the Clinician: Lessons in Heart Failure, Pulmonary Hypertension and Diagnostic Conundrums.
Chaired by Dr. Milton Packer, this program addressed common challenges in clinical cardiology with a goal to provide solutions, practical risk stratification models and innovative approaches including the complexity and significance of right versus left heart disease.
Sunday, September 12, 2010 | 1:00 pm - 3:00 pm 2010 Update:
Tips and Pearls in PH for the
Heart Failure Clinician
San Diego Convention Center, San Diego, California
Ballroom 6B - Upper Level
Faculty: Vallerie McLaughlin, MD ~ Program Chair , Milton Packer, MD
Paul Forfia, MD , Teresa De Marco, MD
Sunday, November 2, 2010 Contemporary Trends in the Diagnosis and Management of PAH: An Initiative to Close the Care Gap (Poster Presentation at CHEST 2010)
Vancouver, BC ; Convention Centre Clinical Resource Centre
Sunday, November 14, 2010 | 7:00 pm - 9:30 pm Cardiology for the Clinician: Pearls for Practice
Heart Failure, Pulmonary Hypertension and Diagnostic Conundrums
Sheraton Chicago Hotels and Towers, Chicago, Illinois
Sheraton Chicago Ballroom 4 & 5
Faculty: Milton Packer, MD ~ Program Chair, Paul Forfia, MD, Vallerie V. McLaughlin, MD, Blase A. Carabello, MD, Myung H. Park, MD
An important report from the French Network on Pulmonary Hypertension Registry was published this month in the Circulation Journal (Humbert et al, Survival in Patients With Idiopathic, Familial, and Anorexigen-Associated Pulmonary Arterial Hypertension in the Modern Management Era. Circulation. 2010;122:156-163). A review and analysis is summarized below.
The PAH QuERI initiated patient enrolment in 2004 and included patients with all types of PAH diagnosis. The patients in the French Network's Registry were enrolled between 2002 and 2003, making the report very relevant to our discussion.
Unlike the PAH QuERI, the French Network's Registry enrolled patients from 17 academic centers only, however, the definition used for PAH was very similar: mean pulmonary arterial pressure 25 mm Hg at rest and pulmonary artery wedge pressure 15 mm Hg (measured during right heart catheterization). An additional requirement in the PAH QuERI was a PVR > 3. The French Network's Registry patient follow up was for 3 years and the PAH QuERI is currently in the final stages of completing the 3 year patient follow up.
In total, 354 patients with PAH were enrolled into the French Network's Registry. Incident cases (n=56) were defined as patients who received a diagnosis of PAH confirmed by right heart catheterization during the recruitment phase of the study (October 2002 to October 2003). Prevalent cases were defined as patients diagnosed before the start of the study.
The important baselines and treatment features of the French Network's Registry are shown below in Table 1:
Table 1
Incident Population (n=56)
Combined Analysis Population (n=190)
Age, y
54 (18)
52.5 (16.1)
Female:male
34:22
120:70
Female, %
61
63
Female:male ratio
1.5:1
1.7:1
Diagnosis, %
Idiopathic PAH
87.5
80.5
Familial PAH
5.4
6.3
Anorexigen-associated PAH
7.1
13.2
NYHA FC, %
I/II
21.4
17.4
III
66.1
68.4
IV
12.5
14.2
6WMD, m
306 (137)
311 (115)
Hemodynamics
RAP, mm Hg
8 (5)
9 (6)
mPAP, mm Hg
52 (13)
54 (13)
PAOP, mm Hg
8 (3)
8 (3)
CO, L/min
3.8 (1.2)
3.9 (1.3)
Cardiac index, L/min/m2
2.2 (0.6)
2.2 (0.7)
PVR, Wood units
12.9 (6.2)
13.1 (6.1)
PVRi, Wood units/m2
21.9 (10.1)
22.4 (10.1)
Treatment at study inclusion, %
Conventional therapy only
23.2
29.5
Epoprostenol
17.9
14.7
Bosentan
42.9
35.3
Sildenafil
7.1
2.1
Iloprost
5.3
2.1
Combination of targeted therapies
3.6
12.6
Beraprost
0
2.6
Treprostinil
0
0.5
Conventional therapy, %
Warfarin
94.6
90.5
Diuretics
83.9
69.5
Oxygen
41.1
30.3
Calcium channel blockers
21.4
13.8
Time from diagnosis to study entry (prevalent patients only, n=134), mo
It must be noted that the use of calcium channel blockers appears higher than expected and the use of the so called "targeted" or disease specific therapy was lower than expected particularly the use of combination therapy.
The figures in Table 1 demonstrate the Kaplan-Meier estimates of survival among the French Registry participants according to baseline NYHA functional class in the panel above.
The Kaplan-Meier estimates of survival among these patients with idiopathic, familial, and anorexigen-associated PAH stratified according to the baseline 6 minute walk are shown in the panel below..
Older age, female gender, functional class, 6 minute walk and the hemodynamics were shown to be of statistically significant predictive values with respect to survival
A, Kaplan-Meier estimates of survival among the combined population of patients with idiopathic, familial, and anorexigen-associated PAH stratified according to baseline NYHA FC
The results of the French Network's Registry provide clinicians with an important current understanding of this rare disease, especially with respect to the poor outcome associated with PAH and the difficulty in improving it.
Early diagnosis is clearly critical and the PAH QuERI is addressing how we, as clinicians, can facilitate an earlier and more guidelines oriented approach to this challenge.
While effective therapies are now available, their appropriate use and timely initiation appear to lag, particularly in the community centers.
Innovative physician and patient based programs are required to improve outcome in these high-risk patients.
Important Results 
Needs Assessment Survey 
