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ACCF/AHA issue consensus information regarding pulmonary hypertension

Tuesday, May 19 2009 | Comments

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What's This? The American College of Cardiology Foundation and the American Heart Association released their first clinical expert consensus document on pulmonary hypertension, a complex, multidisciplinary disease.

The organizations provided an overview of the condition, its diagnosis, and treatment to inform clinical practice, since there is not sufficient evidence or experience to conduct the formal practice guidelines process in this area.

They explained that pulmonary arterial hypertension (PAH) results when restricted flow through the pulmonary arterial circulation leads to increased pulmonary vascular resistance and eventually right heart failure. Several pathogenic pathways are implicated in PAH development.

Previously, PAH was considered rare. More recent evidence from a French registry suggests that it occurs in about 15 people per 1 million, with idiopathic PAH being the most common in the registry and more prevalent in women. Familial PAH can be inherited from a mutation in the bone morphogenic protein receptor-2.

Patients suspected of having pulmonary hypertension should undergo an echocardiogram, and evaluating for other etiologies such as thromboembolic disease are also warranted. Complete right heart catheterization can confirm PAH, and acute vasodilator testing can be useful in that it has prognostic value.

PAH has a poor prognosis, with approximately 15% of patients dying within just 1 year on modern treatment. Several factors are predictive of poor prognosis, including advanced functional class, poor exercise capacity (on 6-minute walk test or cardiopulmonary exercise test), high right atrial pressure, significant right ventricular dysfunction or failure, low cardiac index, elevated brain natriuretic peptide, and an underlying scleroderma spectrum of disease diagnosis.

The group provided details about the appropriate use of warfarin, oxygen, diuretics, calcium channel blockers, intravenous epoprostenol (the only therapy shown to prolong survival), treprostinil, iloprost, endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and lung transplantation. Treatment response can be objectively measured through improvement in exercise capacity, hemodynamics, and survival.
 
Patients should be reassessed every 3 to 6 months, depending on the severity of their condition, the groups advised.

In terms of other conditions, the organizations noted that pulmonary hypertension associated with elevated left heart filling pressures will be much more frequent than PAH in cardiology and pulmonology practices. Treatment should be focused on the underlying left heart disease, and "[e]xperts caution against widespread treatment for non-PAH pulmonary hypertension until clinical trial data indicate whether such patients benefit from them."

PAH in pediatric patients and in those with congenital heart disease was also discussed.

This expert consensus document was published online March 30 in the Journal of the American College of Cardiology and the journal Circulation.

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