New guideline released on pharmacologic therapy for pulmonary arterial hypertension in adults
A new guideline was released last week on pharmacologic therapy for pulmonary arterial hypertension in adults.
A new guideline was released last week on pharmacologic therapy for pulmonary arterial hypertension (PAH) in adults.
The CHEST guideline was developed by a 12-member expert panel and was based on systematic reviews of English-language studies published between 1990 and November 2013. The panel issued guideline recommendations when evidence was available to support them and consensus statements based on expert opinion when evidence was lacking.
The guideline is intended for all clinicians caring for adults with PAH, including cardiologists, primary care internists, pulmonologists, and rheumatologists. The panel stressed that the guideline covers only patients with PAH and that the recommendations do not apply to patients with other kinds of pulmonary hypertension, such as that due to left-sided heart disease or chronic hypoxemic lung disease. The guideline was published online June 17 by CHEST.
The guideline panel suggested that severity of a PAH patient's disease be evaluated systematically and consistently with a combination of World Health Organization (WHO) functional class, exercise capacity, and echocardiographic, laboratory, and hemodynamic variables to help inform therapeutic decisions. It also suggested that all PAH patients be evaluated at a center with expertise in PAH diagnosis whenever possible, ideally before therapy is initiated, and that collaborative, closely coordinated care be provided by local physicians as well as PAH experts.
The panel suggested that treatment-naïve patients without symptoms and those at increased risk for PAH should be monitored for symptoms that would warrant initiation of pharmacotherapy and that contributing causes of pulmonary hypertension, such as sleep apnea and systemic hypertension, should be treated aggressively. Symptomatic patients with PAH and no contraindications should have acute vasoreactivity testing using a short-acting agent at an experienced center, the panel suggested, and a trial of oral calcium-channel blocker therapy should be considered in those who demonstrate acute reactivity and have no right-heart failure or contraindications. The panel also made specific recommendations for PAH pharmacotherapy in patients with WHO functional class II, III, and IV symptoms.
The guideline panel suggested that pregnancy be avoided in patients with PAH. It also suggested that patients avoid exposure to high altitude and use supplemental oxygen as needed during altitude exposure or air travel in order to maintain an oxygen saturation above 91%. Patients with PAH should maintain current immunization against influenza and pneumococcal pneumonia, the panel suggested, and should avoid nonessential surgery. When surgery is necessary, the panel suggested, it should be performed at a pulmonary hypertension center with a multidisciplinary approach and careful monitoring.
“No approved therapy for PAH has been shown to prevent progression of the underlying pulmonary vascular disease,” the guideline panel wrote. “PAH remains an incurable disease; currently clinicians attempt to manage it with pharmacotherapy. While we believe the outlook for a newly diagnosed patient with PAH has improved, it remains far from adequate or acceptable.” The panel called on researchers to help fill the gaps in evidence regarding clinical questions about PAH.
The complete guideline is available free of charge online.
Read more about diagnosing PAH in the primary care setting in ACP Internist's April 2013 cover story.