Fast Five Quiz: Pulmonary Arterial Hypertension

Zab Mosenifar, MD


June 14, 2019

Findings from the history, physical examination, chest radiography, and ECG may suggest the presence of pulmonary hypertension and right ventricular dysfunction. Two-dimensional transthoracic echocardiography 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 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. European guidelines recommend first evaluating for significant group 2 or 3 disease by ordering a transthoracic echocardiogram, pulmonary function tests with arterial blood gas assessment, and chest imaging.

If this workup is unrevealing, patients should then undergo ventilation-perfusion lung scanning. If defects are present, pulmonary angiography or spiral CT scanning 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.

Read more on the workup of PAH.


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