Background Although pulmonary arterial hypertension (PAH) is a relatively rare disease, it occurs relatively frequently as a complication of connective tissue diseases (CTDs), particularly systemic sclerosis (SSc), mixed connective tissue disease (MCTD), and systemic lupus erythematosus (SLE). PAH is defined as a mean pulmonary artery pressure (PAP) over the 25 mmHg at rest. Right heart catheterization (RHC) is strongly required to evaluate the PAP. However, RHC is invasive and it is also difficult to perform frequently, therefore, alternative noninvasive methods would be required. In this study, we investigated non invasive evaluation of PAH in patient with CTDs.
Objectives To determine the non invasive evaluation of PAH in patient with CTDs.
Methods The study subjects were selected from authors’ hospital who fulfilled the CTDs criteria (43 with MCTD, 30 with SSc, 24 with SLE), as well as those with a estimated PAP at rest of 40 mmHg or more were chosen from patients who underwent Doppler echocardiography. Diameters of the main pulmonary artery (d-mPA), right pulmonary artery (d-rPA), and ascending aorta (d-aAO) on thoracic computed tomography (CT) were measured. Moreover, serum brain natriuretic peptide (BNP) levels and % vital capacity (%VC), % forced vital capacity (%FVC), and % diffusing capacity for carbon monoxide (%DLCO) with pulmonary function tests were assessed. The relationship between these results and estimated PAP measured by Doppler echocardiography was evaluated. Static analyses were performed to determine differences in descriptive characteristics between groups.
Results There were significant correlations between d-rPA/d-aAO and estimated PAP measured by Doppler echocardiography. Moreover, combination of d-rPA/d-aAO and serum BNP levels were significantly useful and using multiple linear regression, it may possible for predict the PAP by calculating formula: [Estimated PAP =77-25.2(d-aAO/d-rPA)+0.02BNP]. On the other hand, %VC/%DLCO and %FVC/%DLCO were useful for predict the existence of PAH but no correlations with estimated PAP.
Conclusions It is suggested that a combination of d-rPA/d-aAO measured by CT and serum BNP levels are more closely related to estimated PAP measured by Doppler echocardiography and useful in the follow up of patients with PAH associated with CTDs.
Disclosure of Interest None Declared
Statistics from Altmetric.com
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.