Borderline Pulmonary Arterial Pressure Research Articles

Pulmonary hypertension screening in patients with systemic sclerosis, in a tertiary center, in Turkey; a cross-sectional original study

Aim: Development of Pulmonary Hypertension (PH) in Systemic Sclerosis (SS) significantly reduces the survival of the disease and early diagnosis and treatment is very important.The aim of this study was to investigate the presence of PH in patients who were followed and treated by rheumatology clinic with the diagnosis of SS and who did not have a known diagnosis of PH.Materials and Methods: This cross-sectional study was completed with 51 patients with SS and a control group of 51 volunteers with similar characteristics in terms of gender and comorbidity. Demographic, laboratory and echocardiographic data were recorded.Results: The median age of the patients with systemic sclerosis was 53 (46-60) years and the control group was 50 (45-55) years. 42 (82.4%) of the SS patients were female and 39 (76.5%) of the control group were female. Right heart catheterization was performed to 3 patients with high pulmonary artery pressure (>40 mmHg) on transthorasic echocardiography. Group 1 PH was diagnosed in two of three patients (3.9%); group 2 PH was diagnosed in one of three patients (1.9%).Conclusion: In our study, we detected pulmonary hypertension in 5.8% of 51 patients with systemic sclerosis in a tertiary center. Although these patients have undergone PH screening at certain frequencies, it is noteworthy that we achieved this finding. We believe that we have detected patients with pulmonary systolic pressure at the border and showing rapid progression. Our study supports the more frequent screening of SS patients with borderline pulmonary artery pressure elevation.

Reduced diffusing capacity for carbon monoxide predicts borderline pulmonary arterial pressure in patients with systemic sclerosis.

Early intervention in pulmonary arterial hypertension associated with systemic sclerosis (SSc) may improve its prognosis. We aimed to establish an algorithm to detect mean pulmonary artery pressure (mPAP) > 20mmHg using non-invasive examinations in SSc patients by modifying the DETECT algorithm. This study included SSc patients who underwent right heart catheterization (RHC) in our hospital during 2010-2018. Following variables were assessed for performance to predict mPAP ≥ 25mmHg or > 20mmHg; anti-centromere or U1-RNP antibody, plasma BNP level, serum urate level, right axis deviation, forced vital capacity (FVC)/diffusing capacity for carbon monoxide (DLCO) ratio, and tricuspid regurgitation velocity. Of 58 patients enrolled in this study, 24 had mPAP of ≥ 25mmHg and 9 had mPAP of 21-24mmHg. Among variables tested, only FVC/DLCO elevated similarly in patients with mPAP of ≥ 25mmHg (median 2.5) and those with mPAP of 21-24mmHg (median 2.5) compared to those with mPAP of ≤ 20mmHg (median 1.5). Given the particularly good correlation between DLCO and mPAP of > 20mmHg, each variable was weighted according to its odds ratio and the total weighted score was calculated. The total weighted score exhibited a good predictive performance for mPAP of > 20mmHg with its sensitivity of 87.5% and specificity of 92%. Among conventional risk factors for PAH, decreased DLCO may predict mPAP > 20mmHg with priority in SSc patients. Weighting DLCO may improve the performance of screening algorithm for early SSc-PAH.

Pulmonary arterial hypertension associated with connective tissue diseases: A review focusing on distinctive clinical aspects.

Recent studies have clarified that pulmonary arterial hypertension associated with connective tissue diseases (CTD-PAH) has some distinctive clinical aspects from other PAH, such as high prevalence, venous and cardiac involvement, less favourable outcome, helpfulness of detection algorithm, response to immunosuppression, pre-PAH conditions in borderline pulmonary arterial pressure and coexistence of interstitial lung disease. In this review, by focusing on these distinctive aspects, we discuss how to provide an efficacious and safe management of CTD-PAH and garner attention to areas where further evidence is desired.

GW27-e0047 Characteristics of Cardiopulmonary Exercise Tests in Patients with Borderline Pulmonary Arterial Pressure

GW27-e0047 Characteristics of Cardiopulmonary Exercise Tests in Patients with Borderline Pulmonary Arterial Pressure

Survival in patients with borderline pulmonary arterial pressure

Background: Pulmonary hypertension (PH) is defined as an elevation of mean pulmonary arterial pressure (mPAP) ≥25 mmHg. Normal mPAP is considered to be 14 ± 3 mmHg. The prognostic relevance of mPAP between 21 and 25 mmHg is unknown.

Borderline pulmonary arterial pressure in systemic sclerosis patients: a post-hoc analysis of the DETECT study.

IntroductionPatients with mean pulmonary artery pressures (mPAP) of 21 to 24 mm Hg have a so-called borderline elevation of mPAP (BoPAP)—a condition thought to represent early-stage pulmonary arterial vasculopathy. Based on the DETECT study, this post-hoc analysis examined patient characteristics of systemic sclerosis (SSc) patients with normal mPAP, BoPAP and elevated mPAP, fulfilling pulmonary arterial hypertension (PAH) criteria.MethodsAdult patients with a duration of SSc more than 3 years, a diffusing capacity of the lung for carbon monoxide less than 60% predicted, and no previous diagnosis of any form of pulmonary hypertension (PH) underwent screening tests followed by right heart catheterization. Subjects were divided into three groups: normal mPAP, BoPAP, and PAH. Exploratory comparative and binary logistic regression analyses were performed for the BoPAP versus normal mPAP and PAH versus BoPAP groups.ResultsOf 244 patients evaluated, 148 (60%) had normal mPAP, 36 (15%) had BoPAP, and 60 (25%) had definite PAH. Univariable logistic regression (ULR) showed the mean tricuspid regurgitation velocity in patients with BoPAP to be intermediate between normal mPAP and PAH. In the ULR analyses BoPAP versus normal mPAP and PAH versus BoPAP, the statistically significant predictors were, amongst others: demographic, clinical, pulmonary function, echocardiographic and hemodynamic variables.ConclusionsIn this exploratory post-hoc analysis of the DETECT study population patients with BoPAP could be distinguished from patients with normal mPAP and PAH, and it appears that BoPAP may be an intermediate stage on the continuum between normal PA pressures and PAH.Electronic supplementary materialThe online version of this article (doi:10.1186/s13075-014-0493-1) contains supplementary material, which is available to authorized users.

Characterization of Patients With Borderline Pulmonary Arterial Pressure

BACKGROUND:Resting mean pulmonary artery pressure (mPAP) values between 20 and 25 mm Hg are above normal but do not fulfill the criteria for pulmonary hypertension (PH). The clinical relevance of such borderline hemodynamics is a matter of discussion.METHODS:We focused on patients who underwent right-sided heart catheterization during rest and exercise for symptoms indicative of PH or due to underlying disease associated with an increased risk for pulmonary arterial hypertension and characterized the patients according to their resting mPAP. Patients with manifest PH (mPAP ≥ 25 mm Hg) were excluded.RESULTS:We included 141 patients, 32 of whom presented with borderline hemodynamics (20 < mPAP < 25 mm Hg). Borderline patients were older (65.8 ± 12.5 years vs 57.3 ± 12.5 years,P= .001) and more often had cardiac comorbidities (53% vs 15%,P< .001) or decreased lung function (47% vs 16%,P< .001) as compared with patients with resting mPAP < 21 mm Hg. After correction for age, borderline patients had significantly increased pulmonary vascular resistance (2.7 ± 0.7 Wood units vs 1.8 ± 0.8 Wood units,P< .001) and mPAP/cardiac output (CO) and transpulmonary gradient/CO slopes (bothP< .001) as well as lower peak oxygen uptake (16.9 ± 4.6 mL/min/kg vs 20.9 ± 4.7 mL/min/kg,P= .009) and 6-min walk distance (383 ± 120 m vs 448 ± 92 m,P= .001). During follow-up (4.4 ± 1.4 years), the mortality rate of borderline patients vs patients with resting mPAP < 21 mm Hg was 19% vs 4%.CONCLUSIONS:In patients undergoing right-sided heart catheterization with exclusion of manifest PH, borderline elevation of pulmonary arterial pressure is associated with cardiac and pulmonary comorbidities, decreased exercise capacity, and a poor prognosis.

Clinical characterization and survival of patients with borderline elevation in pulmonary artery pressure.

Normal resting mean pulmonary artery pressure (PAP) is 8-20 mmHg. Pulmonary hypertension is defined as mean PAP of ≥25 mmHg. Borderline PAP levels of 21-24 mmHg are of unclear significance. We sought to determine the clinical characteristics and survival of subjects with mean PAP of 21-24 mmHg. We examined 1,491 patients enrolled in the Cleveland Clinic Pulmonary Hypertension Registry between February 1990 and May 2012 with baseline right heart catheterization. The relationship between PAP and all-cause mortality was assessed by Cox models and a tree-based analysis. Sixty-three patients had borderline PAP (underlying conditions: 12 left heart disease, 20 respiratory disease, 17 connective-tissue disease, 4 others, and 10 none). We then compared 3 groups: borderline PAP without heart or lung disease ([Formula: see text]), normal PAP without heart or lung disease ([Formula: see text]), and category 1 pulmonary arterial hypertension (PAH; [Formula: see text]). Borderline-PAP patients had levels of hemodynamic and functional compromise between those for normal-PAP patients and those for patients with PAH. Borderline PAP was associated with increased mortality compared to normal PAP (hazard ratio: 4.03 [95% confidence interval: 0.78-20.80], [Formula: see text]). A tree-based analysis demonstrated almost identical cut points in mean PAP (≤20, 21-26, and ≥27 mmHg) associated with differential survival ([Formula: see text]). Connective-tissue disease and an elevated transpulmonary gradient were predictors of worse survival in the borderline-PAP population. Borderline PAP elevation is associated with decreased survival, particularly in the context of connective-tissue disease and an elevated transpulmonary gradient.

Pulmonary arterial hypertension therapy may be safe and effective in patients with systemic sclerosis and borderline pulmonary artery pressure

Borderline pulmonary arterial hypertension (PAH), characterized by a marked exercise-induced increase in pulmonary artery pressure (PAP) with normal resting values, may precede overt PAH in systemic sclerosis (SSc). We undertook the present study to investigate whether PAH treatment is safe in these patients and might attenuate hemodynamic progression. SSc patients with borderline PAH underwent right heart catheterization at baseline, after a 12-month observation period, and subsequently after 6 months of bosentan therapy. Changes in mean PAP at 50W during the observation period versus during therapy were compared. Ten patients completed the study. Mean PAP at rest, at 50W, and during maximal exercise increased significantly during the observation period (mean ± SD increases of 2.5 ± 3.0 mm Hg [P = 0.03], 4.0 ± 2.9 mm Hg [P = 0.002], and 6.8 ± 4.1 mm Hg [P = 0.0005], respectively) and tended to decrease during the treatment period (decreases of 2.5 ± 3.9 mm Hg [P = 0.07], 1.5 ± 4.5 mm Hg [P = 0.32], and 1.8 ± 7.0 mm Hg [P = 0.43], respectively). The changes during the observation period versus the therapy period were significantly different (P = 0.03 at rest, P = 0.01 at 50W [primary end point], and P = 0.02 during maximal exercise). The changes in resting pulmonary vascular resistance were also significantly different during the observation period (increase of 8 ± 25 dynes · seconds · cm(-5) ) versus during the therapy period (decrease of 45 ± 22 dynes · seconds · cm(-5) ) (P < 0.0005). Changes in resting pulmonary arterial wedge pressure were not significantly different between the observation period and the treatment period, despite the significant increase during the observation period (2.6 ± 2.5 mm Hg [P = 0.01]). No relevant adverse effects were reported. In SSc patients with borderline abnormal pulmonary hemodynamics, resting and exercise PAP may increase significantly within 1 year of observation. Bosentan might be safe and effective to attenuate these changes. Randomized controlled trials are warranted to confirm the exploratory findings of this hypothesis-generating pilot study.

Borderline Pulmonary Arterial Pressure Is Associated with Decreased Exercise Capacity in Scleroderma

Pulmonary arterial hypertension is associated with impaired exercise capacity and decreased survival in patients with scleroderma. Randomized controlled studies showed significant benefit of targeted therapies in patients with a resting mean pulmonary arterial pressure (MPAP) greater than 25 mm Hg. The clinical relevance of pulmonary arterial pressure values in the upper normal range is unknown. To examine the clinical relevance of pulmonary arterial pressure in scleroderma patients. After a noninvasive screening program, 29 patients with systemic sclerosis without significant lung fibrosis and without known pulmonary arterial hypertension underwent right heart catheterization and simultaneous cardiopulmonary exercise test. A six-minute walk distance (6MWD) was determined within 48 hours. A resting MPAP above the median (17 mm Hg) was associated with decreased 6MWD (396 +/- 71 vs. 488 +/- 76 m; P < 0.005) and peak Vo(2) (76 +/- 11% vs. 90 +/- 24%; P = 0.05). Resting pulmonary vascular resistance was inversely correlated with 6MWD (r = 0.45; P < 0.05). At 25 and 50W, MPAP above the median (23 and 28 mm Hg) was associated with decreased 6MWD (P < 0.005; P < 0.0005). At peak exercise, MPAP showed no association with 6MWD or peak Vo(2); however, cardiac index was positively (r = 0.45; P < 0.05) and pulmonary vascular resistance was negatively correlated with 6MWD (r = -0.38; P < 0.05). MPAP and resistance in the upper normal range at rest and moderate exercise are associated with decreased exercise capacity and may indicate early pulmonary vasculopathy in patients with systemic sclerosis. Investigations on the prognostic and therapeutic implications of such borderline findings are warranted. Clinical trial registered with http://www.clinicaltrials.gov (NCT00609349).