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Grand Rounds | At the Northwestern University Feinberg School of Medicine| Clinician's Corner

Pulmonary Hypertension

Sanjiv J. Shah, MD
JAMA. 2012;308(13):1366-1374. doi:10.1001/jama.2012.12347.
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Published online

Pulmonary hypertension (PH), defined as elevated pulmonary artery pressure, is common in the general population and associated with increased mortality. Accordingly, physicians commonly encounter patients with dyspnea, exercise intolerance, and/or right heart failure who have elevated pulmonary artery systolic pressure (PASP) on echocardiography. Although pulmonary arterial vasodilators may often be considered in this setting, these drugs have been predominantly tested in the subset of PH patients with pulmonary arterial hypertension (PAH). Elevated PASP alone is not sufficient for the diagnosis of PAH, and secondary causes of PASP elevation, most commonly left heart disease, are far more prevalent than isolated PAH. Treatment of this more common group of patients with PH due to left heart disease is challenging because there are few evidence-based treatment options, and pulmonary vasodilator therapy may lead to worsening symptoms. Therefore, improving symptoms and avoiding adverse outcomes in patients with PH requires the following: (1) understanding the optimal use of echocardiography for the diagnosis of PH; (2) recognizing the utility and proper interpretation of invasive hemodynamic testing prior to starting pulmonary vasodilator therapy; (3) differentiating PAH from pulmonary venous hypertension due to left heart disease; and (4) understanding the appropriate treatment strategies for PH and resultant right heart failure.

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Figure 1. Pulmonary Hypertension Diagnostic and Categorization Algorithm
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The initial goal of the algorithm is to determine whether PH is likely present based on echocardiography and whether the PH could be due to a common cause. If PH is present and the cause is not apparent, if symptoms persist despite treatment of the suspected underlying cause, if pulmonary arterial hypertension is suspected, or if treatment with pulmonary vasodilators is being considered, further evaluation is necessary. At this point, additional testing to determine the etiology of PH, evaluation of functional capacity, and invasive hemodynamic testing are all indicated—with the ultimate goals of confirming the PH diagnosis, differentiating pulmonary arterial from pulmonary venous hypertension, determining type of PH, and choosing initial therapy. WHO indicates World Health Organization. aBased on the results of initial testing, consider further evaluation with high-resolution chest computed tomography (CT), CT angiography, or transesophageal echocardiography when indicated.

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Figure 2. Case Patient's Echocardiogram Demonstrating Useful Parameters for the Evaluation of Right Ventricular Size and Function
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Sample echocardiographic imaging from the case patient. A, Global right ventricular (RV) systolic function can be assessed by RV fractional area change (reference value, >0.35). B, Longitudinal RV systolic function can be assessed by tricuspid annular plane systolic excursion (normal, >1.6 cm) and tissue Doppler peak systolic velocity of the basal RV free wall (reference value, >10 cm/s). These echocardiographic images were obtained 6 months after treatment and demonstrate near-normalization of RV systolic function in the case patient. Data for reference values are obtained from Rudski et al.31 See of the case patient's baseline echocardiogram and the echocardiographic findings of 2 additional patients for comparison.

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