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Original Research: Pulmonary Vascular Disease |

Contemporary Trends in the Diagnosis and Management of Pulmonary Arterial HypertensionTrends in Pulmonary Arterial Hypertension: An Initiative to Close the Care Gap

Vallerie V. McLaughlin, MD, FCCP; Anatoly Langer, MD; Mary Tan, MSc; Philip J. Clements, MD, MPH; Ronald J. Oudiz, MD, FCCP; Victor F. Tapson, MD, FCCP; Richard N. Channick, MD, FCCP; Lewis J. Rubin, MD, FCCP; for the Pulmonary Arterial Hypertension-Quality Enhancement Research Initiative (PAH-QuERI) Investigators
Author and Funding Information

From the Pulmonary Hypertension Program (Dr McLaughlin), University of Michigan Health System, Ann Arbor, MI; The Canadian Heart Research Centre (Dr Langer and Ms Tan), Toronto, ON, Canada; David Geffen School of Medicine at UCLA (Dr Clements), Los Angeles, CA; Los Angeles Biomedical Research Institute at Harbor-UCLA Medical Center (Dr Oudiz), Los Angeles, CA; Duke University Medical Center (Dr Tapson), Durham, NC; Massachusetts General Hospital (Dr Channick), Boston, MA; and UC San Diego (Dr Rubin), La Jolla, CA.

Correspondence to: Vallerie V. McLaughlin, MD, FCCP, Pulmonary Hypertension Program, University of Michigan Cardiovascular Center, 1500 E Medical Center Dr, SPC 5853, Ann Arbor, MI 48109; e-mail: vmclaugh@umich.edu


A list of participating Pulmonary Arterial Hypertension-Quality Enhancement Research Initiative (PAH-QuERI) investigators and coordinators can be found in e-Appendix 1.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.

Funding/Support: This study was supported by Actelion Pharmaceuticals US, Inc. The PAH-QuERI project was conceived, designed, and coordinated by The Canadian Heart Research Centre, a federally incorporated, not-for-profit, academic research organization.


Chest. 2013;143(2):324-332. doi:10.1378/chest.11-3060
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Background:  The Pulmonary Arterial Hypertension-Quality Enhancement Research Initiative (PAH-QuERI) was created to help clinicians to implement a guidelines-based approach to the diagnosis and management of pulmonary arterial hypertension (PAH). Patients with PAH represent a heterogeneous population, and physician evaluation and treatment paradigms may vary considerably.

Methods:  Using an electronic data management system, participating physicians recorded data on diagnostic workup, disease management, and outcomes of patients with PAH. Queries were generated automatically following each follow-up visit if the tests recommended by the American College of Chest Physicians (ACCP) were not performed at least once.

Results:  Of 791 patients enrolled in PAH-QuERI, 77% were women; 64% received a diagnosis > 3 months prior to enrollment; 9% were in New York Heart Association functional class I, 39% in II, 48% in III, and 5% in IV; and the median age was 55 years (interquartile range, 45-66 years). At enrollment, all ACCP-recommended tests had been performed in only 6% of patients. The automated program generated 1,530 reminders for 642 patients (81%) with validated enrollment data. The proportion of recommended tests performed was 91% for CBC count, 91% for liver function test, 50% for connective tissue disease screen, 29% for HIV screen, 88% for chest radiograph, 82% for ECG, 97% for two-dimensional echocardiogram, 83% for pulmonary function tests, 41% for oximetry, 57% for ventilation/perfusion scan, 79% for 6-min walk distance, and 90% for right-sided heart catheterization. Regarding management, 78% of patients were on disease-specific therapy, and the use of these therapies tended to increase with the functional disability of the patient. One hundred seventy patients were taking calcium channel blockers, 91 specifically for PAH. Only six of 91 patients (7%) who received calcium channel blockers specifically for PAH had met the current guideline for acute vasoreactivity.

Conclusions:  When comparing reported clinical practice with ACCP guidelines-recommended strategies, a diagnostic care gap is apparent such that certain essential and recommended diagnostic tests may be underused despite the availability of detailed guidelines and reminders.

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