Practice Guidelines

ACCP Updates Guidelines for the Treatment of PAH

SHERRI DAMLO

Pulmonary arterial hypertension (PAH) is often a progressive disorder that results in right ventricular dysfunction and impairment in activity tolerance, and it can lead to right-heart failure and death. PAH can occur in patients with other underlying medical conditions. Although the pathogenesis is complex and not fully understood, it includes environmental and genetic factors that alter vascular function and structure. In response to dramatic advances in the treatment of PAH in recent years, the American College of Chest Physicians (ACCP) has updated its 2004 guidelines with evidence-based treatment recommendations for the management of this disorder.

Treatment

Developments in the treatment of PAH are rapidly advancing, so physicians should consider a variety of factors for patients with the disorder (Figure 1), including cardiopulmonary hemodynamics, signs and symptoms of right-heart failure, drug-drug interactions, side effects, and New York Heart Association functional class (Table 1). Quantifying functional class may be difficult because it varies among patients and among health care professionals, and it may not always correlate with other indices of disease severity. However, functional class does correlate with outcomes in patients with idiopathic PAH.

functional class ii

Sildenafil (Revatio) and intravenous and subcutaneous treprostinil (Remodulin) are the only therapies approved for functional class II patients with PAH. The ACCP recommends sildenafil as the first choice for most of these patients because of its relative effectiveness and ease of administration.

Table 1. Summary of Updated ACCP Recommendations for the Treatment of Pulmonary Arterial Hypertension

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functional class iii

The U.S. Food and Drug Administration has approved five drugs for the treatment of functional class III patients with PAH: bosentan (Tracleer), sildenafil, intravenous epoprostenol (Flolan, brand no longer available in the United States), iloprost (Ventavis), and subcutaneous and intravenous treprostinil. The ACCP recommends that bosentan or sildenafil be considered for "early" functional class III patients. Therapeutic decisions must be based on clinical judgment, and the relative toxicities of each drug and the specific situation and preferences of the patient should be considered (e.g., patients with liver abnormalities may respond better to sildenafil; those with ocular disease may be better candidates for bosentan). Patients with more advanced PAH may require treatment with a prostanoid (e.g., intravenous epoprostenol, treprostinil), iloprost, or subcutaneous treprostinil.

functional class iv

All currently labeled therapies are approved for patients with functional class IV PAH, but the ACCP recommends treatment with intravenous epoprostenol for patients who are critically ill. Because there are limited data on which to base treatment decisions for patients with functional class IV symptoms, oral, inhaled, or subcutaneous agents generally should not be used as first-line therapy.

Conclusions

To determine the appropriate therapy, recommendations should be based on each patient's specific situation. Cost may also be a factor when considering choice of therapy. Physicians should look for contributing factors and underlying causes, and a thorough diagnostic evaluation should be performed in these patients. However, because of the complexity of the diagnostic evaluation and treatment options, the ACCP recommends that physicians consider referring patients with PAH to a specialized health care facility.

Practice Guideline Briefs

CDC Evaluates the Effect of Revised Guidelines on the Prevention of Group B Streptococci Disease

In 2002, the Centers for Disease Control and Prevention (CDC), the American College of Obstetricians and Gynecologists, and the American Academy of Pediatrics revised guidelines for the prevention of perinatal group B streptococci (GBS) disease, which included recommending that health care professionals screen patients to identify candidates for prophylaxis. The CDC has reviewed surveillance data to compare the rates of neonatal and pregnancy-related GBS disease before and after the new guideline was released. Data were reviewed from the two years before (2000 to 2001) and after (2003 to 2005) the publication of the guideline revisions.

From 2003 to 2005, the average incidence of early-onset neonatal GBS disease was 33 percent less than in the pre-guideline revision period; however, although the incidence decreased steadily among white infants between 2003 and 2005, it increased by 70 percent during the same period in black infants. In the years after the guideline revisions, rates of pregnancy-related and late-onset neonatal GBS disease remained stable compared with baseline rates.

Although further research is needed, the authors conclude that these data highlight the need for strategies to reduce the rate of neonatal GBS disease among black infants, evaluation of missed opportunities for prevention, and continued monitoring of disease trends.

AMBER HUNTZINGER

CDC Reports a Decrease in the Incidence of Breast Cancer

Although breast cancer is the most commonly diagnosed cancer in U.S. women, the Centers for Disease Control and Prevention (CDC) reports that the incidence of breast cancer has decreased since 1999. Data from population-based cancer registries show that invasive breast cancer decreased annually between 1999 and 2003; and, after increasing between 1999 and 2002, noninvasive breast cancer decreased between 2002 and 2003.

Data were collected from the CDC's National Program of Cancer Registries and the National Cancer Institute's Surveillance, Epidemiology, and End Results program. The largest decline occurred between 2002 and 2003 (6.1 percent for invasive disease and 2.7 percent for noninvasive disease). The decrease in breast cancer occurred across several age, cancer stage, and racial groups, with the most significant decrease occurring in women 50 years and older. Factors that may have contributed to the decrease in breast cancer include mammography screening, a decrease in the use of hormone therapy, and differences in risk-factor prevalence, diet, and lifestyle.

AMBER HUNTZINGER

Answers to This Issue's Clinical Quiz Q1. C Q2. C Q3. C Q4. A Q5. B Q6. C Q7. A, D Q8. A, C Q9. A, B Q10. A, B, C

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