Cardiac Catheterization versus Echocardiography for Monitoring Pulmonary Pressure: A Prospective Study in Patients with Connective Tissue Disease-Associated Pulmonary Arterial Hypertension.

Vasiliki Kalliopi Bournia,Petros P Sfikakis,Iraklis Tsangaris,Loukianos Rallidis,Frantzeska Frantzeskaki,Katerina Laskari,Panagiotis Karyofillis,Vassilis Voudris,Anastasia Anthi,Stylianos E Orfanos,Eftychia Demerouti,Stylianos Panopoulos,Panayiotis G Vlachoyiannopoulos,Dimitrios Konstantonis

doi:10.3390/diagnostics10010049

Abstract

Standard echocardiography is important for pulmonary arterial hypertension (PAH) screening in patients with connective tissue disease (CTD), but PAH diagnosis and monitoring require cardiac catheterization. Herein, using cardiac catheterization as reference, we tested the hypothesis that follow-up echocardiography is adequate for clinical decision-making in these patients. We prospectively studied 69 consecutive patients with CTD-associated PAH. Invasive baseline pulmonary artery systolic pressure (PASP) was 60.19 ± 16.33 mmHg (mean ± SD) and pulmonary vascular resistance (PVR) was 6.44 ± 2.95WU. All patients underwent hemodynamic and echocardiographic follow-up after 9.47 ± 7.29 months; 27 patients had a third follow-up after 17.2 ± 7.4 months from baseline. We examined whether clinically meaningful hemodynamic deterioration of follow-up catheterization-derived PASP (i.e., > 10% increase) could be predicted by simultaneous echocardiography. Echocardiography predicted hemodynamic PASP deterioration with 59% sensitivity, 85% specificity, and 63/83% positive/negative predictive value, respectively. In multivariate analysis, successful echocardiographic prediction correlated only with higher PVR in previous catheterization (p = 0.05, OR = 1.235). Notably, in patients having baseline PVR > 5.45 WU, echocardiography had both sensitivity and positive predictive values of 73%, and both specificity and negative predictive value of 91% for detecting hemodynamic PASP deterioration. In selected patients with CTD-PAH echocardiography can predict PASP deterioration with high specificity and negative predictive value. Additional prospective studies are needed to confirm that better patient selection can increase the ability of standard echocardiography to replace repeat catheterization.

Highlights

Pulmonary arterial hypertension (PAH), a severe complication of connective tissue disease (CTD), has been commonly described among systemic sclerosis (SSc) patients, with an estimated prevalence of 5–10% [1,2]
Comparing pulmonary artery systolic pressure (PASP) deterioration as measured by repeat right heart catheterization (RHC) to that estimated by repeat echocardiography, we found that in 74 out of 96 patients’
Using McNemar’s test for symmetry, we found no statistically significant difference in the ability of the two methods to detect ≥10% deterioration in PASP, when applied in the follow up of patients with CTD

Summary

Introduction

Pulmonary arterial hypertension (PAH), a severe complication of connective tissue disease (CTD), has been commonly described among systemic sclerosis (SSc) patients, with an estimated prevalence of 5–10% [1,2]. Less frequently, it can be a manifestation of systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD), dermatomyositis, polymyositis, Sjögren’s syndrome and rheumatoid arthritis [3]. Follow-up of CTD patients with PAH includes standard echocardiography and RHC every 6–12 months, or 3–6 months after changes in therapy, according to the 2015 European Society of Cardiology/European. Among the many parameters derived from standard echocardiography, Pulmonary Artery Systolic Pressure (PASP) remains the most commonly used

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