Borderline Mean Pulmonary Artery Pressure in Patients With Systemic Sclerosis: Transpulmonary Gradient Predicts Risk of Developing Pulmonary Hypertension

Abstract

Objective. To determine whether patients with systemic sclerosis (SSc) and borderline mean pulmonary artery pressure (PAP) at cardiac catheterization are more likely to develop pulmonary hypertension (PH) than those in whom pulmonary pressure is normal.Methods. Patients with SSc in whom PH and significant interstitial lung disease had been excluded at baseline were enrolled in our prospective cohort. Analysis of followup data identified patients who met prespecified criteria prompting repeat catheterization to reassess for possible PH. Using Kaplan-Meier, receiver operating characteristic, and Cox regression methods, we studied the development of PH and death.Results. Of 228 patients in this study, 86 had borderline mean PAP (21-24 mm Hg) at baseline. Following prespecified criteria, 76 patients underwent repeat catheterization, and 29 of these developed PH. Two cases were related to disease of the left side of the heart. The average mean PAP increased from baseline (20.2 mm Hg) to followup (24.3 mm Hg) (P < 0.05 by Student's t-test). Patients with borderline mean PAP were more likely to develop PH than patients with mean PAP <20 mm Hg (P < 0.001 by log rank test, hazard ratio [HR] 3.7). A transpulmonary gradient (TPG) >11 mm Hg at baseline also predicted PH (P < 0.001 by log rank test, HR 7.9). Incident development of pulmonary arterial hypertension (PAH) was not benign, with a mortality of 18% within 3 years.Conclusion. Our findings indicate that borderline mean PAP and an elevated TPG in patients with SSc predict progression to PH. These patients should be monitored closely for the development of PH. Our findings indicate that catheterization data are useful in patients considered at risk of PAH.

Mild Pulmonary Hypertension Is Associated With Increased Mortality: A Systematic Review and Meta‐Analysis

“…Forty full‐text articles were assessed for eligibility (Figure 1). Fifteen (8 RHC and 7 echocardiography) studies were included in the meta‐analysis 2, 3, 4, 5, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20. The characteristics of the included studies are shown in Table 2.…”

“…Studies included in this analysis are Valerio et al,10 Heresi et al,11 Kovacs et al,5 Suzuki et al,12 Maron et al,3 Takahashi et al,13 Douschan et al,4 Assad et al,2 Abramson et al,14 Kjaergaard et al,15 Shalaby et al,16 Lam et al,17 Damy et al,18 Cabrita et al,19 and Choudhary et al 20. CI indicates confidence interval; M‐H, Mantel‐Haenszel; RHC, right heart catheterization.…”

Exercise pulmonary haemodynamics: a test in search of purpose

“…From VALERIO et al [12], we know that among scleroderma patients the likelihood of developing PH over 3 years is ∼18.5% among those with an mean Ppa of 21-24 mmHg, and only 5% among those with normal resting pressure. Given that 86% of the 21-24 mmHg population and 47% of those with normal resting pressures in this study had abnormal exercise responses, it is unlikely that the exercise response will help to significantly enrich the population requiring follow-up for the development of PH.…”

Is early diagnosis of pulmonary arterial hypertension possible in inflammatory rheumatic diseases? Experience from a single center in Turkey

“…The importance of reactive PH, borderline PH (mPAP between 21 and 24 mmHg), and the prognosis of patients with mildly elevated mPAP in connective tissue diseases has yet to be defined (6,28). In a recent study, SSc patients with borderline PH and an elevated trans-pulmonary gradient were found to be more likely to develop PH than patients with mPAP≤20 mmHg (29). As a result, these patients should be followed-up by experienced centers.…”