Guidelines for evaluating pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) can be difficult to diagnose because the symptoms are nonspecific and the physical findings are usually subtle (Table). In 2004, the American College of Chest Physicians (ACCP) published clinical practice guidelines for the diagnosis and management of PAH.¹ Highlights of the ACCP's recommendations for patient assessment include the following:

•Doppler echocardiography is indicated for patients in whom PAH is clinically suspected. This study is recommended to estimate pulmonary artery pressures (although it is not as precise as right heart catheterization) and to evaluate for left ventricular systolic or diastol- ic dysfunction, left-sided chamber enlargement, and valvular heart disease.

•Right heart catheterization is needed to confirm PAH and determine its severity and to establish the specific cause. It is also recommended to guide therapy.

•A workup for connective-tissue disease and HIV infection is appropriate for patients who have unexplained PAH.

•Ventilation-perfusion lung scanning is warranted to rule out chronic thromboembolic pulmonary hypertension. Normal results exclude the diagnosis.

•An assessment of sleep-disordered breathing is recommended for patients with PAH.

Additional studies that can be important in the workup include chest radiography, electrocardiography, and pulmonary function testing. Chest radiographic findings that are consistent with PAH include enlarged main and hilar pulmonary arterial shadows with concomitant attenuation of peripheral pulmonary vascular markings. Chest radiography is also important in the evaluation of associated problems, such as interstitial lung disease.

An algorithm for patient assessment is presented in the Figure.

REFERENCE

References:

1.

McGoon M, Gutterman D, Steen V, et al. Screening, early detection, and diagnosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines.

Chest

. 2004;126(1 suppl):14S-34S.