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Closing the Gaps in PAH Management

Although the management of pulmonary arterial hypertension (PAH) has changed dramatically thanks to advances in therapy, the disease continues to be progressive and often fatal. In 2004, the American College of Chest Physicians (ACCP) published its most recent guidelines for diagnosing and managing PAH. After these guidelines were released, the Quality Enhancement Research Initiative (QuERI) was established to help clinicians implement ACCP guideline-based approaches to the diagnosis and management of PAH. While updated guidelines have emerged, the recommended tests for diagnostic assessments of PAH have not differed significantly since 2004. “There’s a need for better understanding of the management, treatment, and progression of PAH,” says Vallerie V. McLaughlin, MD. “People with PAH are a diverse patient group, leading to considerable variation in how physicians evaluate and treat the condition. It’s important to identify areas of care where improvement is required. The PAH-QuERI initiative is intended to help physicians optimize use of the ACCP guidelines and deliver evidence-based care in real-life healthcare settings.” Diagnostic Tests for PAH Largely Underutilized In an issue of Chest, Dr. McLaughlin and colleagues evaluated clinician adherence to the ACCP guidelines when managing patients enrolled in PAH-QuERI. Participating physicians recorded data on diagnostic work-up, disease management, and outcomes of PAH patients. Queries were generated automatically following pre-scheduled follow-up visits if the tests recommended by ACCP were not performed at least once. “Certain essential and recommended diagnostic tests appear to be underutilized despite the availability of detailed guidelines and reminders.” —Vallerie V. McLaughlin, MD At the beginning of the study period, all of the ACCP-recommended tests had been performed in only 6% of PAH patients. The automated program...

A New Consensus on PAH

Pulmonary arterial hypertension (PAH) is a complex, multidisciplinary disorder resulting from restricted flow through the pulmonary arterial circulation; this results in increased pulmonary vascular resistance and, ultimately, right heart failure. PAH has been thought of as a rare disease, but recent evidence suggests that the incidence may be higher than what has been previously reported. The American College of Cardiology Foundation (ACCF) and the American Heart Association (AHA) issued their first expert consensus document on PAH. Published in both the April 28, 2009 issues of Circulation and the Journal of the American College of Cardiology, the guideline is intended to inform practitioners about the evolving areas of clinical practice and/or technologies widely available. The document also includes a table on prognostic factors for PAH in addition to diagnostic and treatment algorithms. The Details of PAH The prognosis of PAH is poor, with about a 15% mortality rate within 1 year on modern therapy. The ACCF/AHA guideline outlines predictors of a poor prognosis, including advanced functional class, poor exercise capacity, high right atrial pressure, significant right ventricular (RV) dysfunction, RV failure, low cardiac index, elevated brain natriuretic peptide, and the scleroderma spectrum of diseases. Idiopathic PAH (IPAH) appears to be the most common type of PAH; it’s more common in younger women who tend to present with nonspecific symptoms (eg, shortness of breath or fatigue). Familial PAH is often the result of a mutation in the BMPR2 gene, which is found in up to 25% of patients with IPAH. PAH is also associated with: Congenital heart disease. Connective tissue diseases. Drugs and toxins. HIV. Portal hypertension. Hemoglobinopathies. Patients with a...
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