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Improving Pulmonary Hypertension Care

Improving Pulmonary Hypertension Care

The success of treatment for pulmonary hypertension (PH), including pulmonary arterial hypertension (PAH), is dependent on early detection and an accurate diagnosis. “PH treatments are typically directed toward the underlying cause, but there can be overlap between various patient groups,” says Mardi Gomberg-Maitland, MD, MSc. “This overlap can complicate diagnostic approaches, therapeutic choices, and anticipated outcomes.” Patients are often referred to PH centers with incomplete evaluations. “PH centers offer important benefits to patients because they have clinicians and nurse teams who are dedicated and trained in caring for these patients,” says Dr. Gomberg-Maitland. “Unfortunately, patients are often referred late in the course of illness. Some are given PAH-specific medications without appropriate testing or indications.” Furthermore, PAH medications are expensive and difficult to manage. Data on Pulmonary Hypertension Diagnosis & Referral In JAMA Internal Medicine, Dr. Gomberg-Maitland, Cherylanne Glassner-Kolmin, BS, and colleagues had a study published that assessed PH diagnosis accuracy in patients referred to PH centers. “We also wanted to look at the appropriateness of use of PAH-specific medications based on current guidelines,” says Glassner-Kolmin. According to findings, 68% of patients were referred by cardiologists or pulmonologists to PH centers and 61% had late-stage disease. One-third of patients who received a definitive PAH diagnosis before their referral received a misdiagnosis. “We also found that many patients were treated contradictorily to published recommendations, either having not undergone a heart catheterization, having been inappropriately prescribed medications, or both,” says Glassner-Kolmin. Dr. Gomberg-Maitland says major efforts have been made to educate medical professionals about PH, but misdiagnoses and inappropriate treatment are still occurring. “Inexperienced clinicians should be cautious of giving patients a...

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...
Diagnosis & Treatment Often Delayed in Hypertension Patients

Diagnosis & Treatment Often Delayed in Hypertension Patients

Results of a national study indicate that 20% of all patients with pulmonary arterial hypertension (PAH) suffer with the disease for more than 2 years before obtaining an accurate diagnosis and proper treatment. “For a lot of patients, that means the treatment is more difficult and the damage is irreversible,” said lead author Lynnette Brown, MD, PhD, a pulmonologist and researcher from Intermountain Medical Center. “Finding out which patients are getting a delayed diagnosis is the first step in identifying them earlier, when treatment is easier and hopefully more effective.” Among the nearly 3,000 adults with PAH who participated in the study, those with symptom onset before age 36 displayed the highest likelihood of delayed recognition (PAH diagnosis, start of PAH-specific therapy, or diagnosis by right-sided heart catheterization). History of obstructive airway disease and sleep apnea were also independently associated with a delayed recognition of PAH. Dr. Brown and colleagues also found that a 6-minute walk distance of less than 250 meters, right atrial pressure less than 10 mmHg, and pulmonary vascular resistance of less than 10 Wood units were associated with delayed recognition, whereas sex, race/ethnicity, and geographic region were not. “We have a lot more medications available to fight pulmonary arterial hypertension, but we can’t use them all if we don’t get to patients early enough in the course of the disease,” said co-author, Gregory Elliott, MD, chairman of the department of medicine at Intermountain Medical Center. “If we can treat these patients sooner, we may find that we can improve survival.” Drs. Elliot and Brown hope the study results provide guidance in diagnosing PAH. “If a...

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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