Which studies are performed in the workup of pulmonary arterial hypertension (PAH)?

Updated: Apr 25, 2018
  • Author: Kristin E Schwab, MD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Findings from the history, physical examination, chest radiography, and electrocardiography (ECG) may suggest the presence of pulmonary hypertension and right ventricular dysfunction. Two-dimensional transthoracic echocardiography (TTE) with Doppler analysis should be used as an initial screening measure to estimate the pulmonary artery pressure and assess ventricular function.

Right-sided cardiac catheterization is recommended as the confirmatory test for pulmonary hypertension. This can also be useful for assessment of the reversibility of pulmonary arterial hypertension (PAH) with vasodilatory therapy.

Further studies should then be performed to assess for the etiology of the pulmonary hypertension, as the etiology determines treatment options and prognosis. PAH is a diagnosis of exclusion, and so it is imperative that the practitioner first assess for WHO groups 2-5 pulmonary hypertension. Given this, European guidelines recommend first evaluating for significant group 2 or 3 disease by ordering a TTE, pulmonary function tests with arterial blood gas assessment, and chest imaging. [19]

If this workup is unrevealing, patients should then undergo ventilation-perfusion lung scanning to assess for group 4 disease. If defects are present, pulmonary angiography or spiral CT should be performed. This is crucial in all patients suspected of having PAH, as chronic thromboembolic pulmonary hypertension (CTEPH) is often curable by surgical endarterectomy.

Patients should also be screened clinically for possible nocturnal desaturation and obstructive sleep apnea.

Finally, laboratory studies should be performed in the appropriate clinical scenarios to evaluate for causes of WHO group 1 disease, or PAH.

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