Raised N-TproBNP levels are directly related to the severity of PAH. In screening programs, SSc patients with an N-TproBNP in excess of 395 pg/mL have a very high probability of having pulmonary hypertension. Baseline and serial changes in N-TproBNP levels are highly predictive of survival. A 10-fold increase in N-TproBNP level on therapy is associated with a greater than three-fold increase in mortality, and may indicate therapeutic failure.

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.

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Objective. To construct a readily applicable formula for selecting patients with systemic sclerosis (SSc) for right-sided heart catheterization (RHC) based on the results of their pulmonary function tests (PFTs).Methods. The diagnostic value of PFT variables was quantified in 386 patients with SSc against data obtained from RHC.Results. We derived the following formula using data from 257 patients: predicted mPAP ‫؍‬ 136 -SpO 2 -0.25 ؋ DLCO % predicted, where mPAP is the mean pulmonary artery pressure, SpO 2 is the oxygen saturation as measured by pulse oximetry, and DLCO is the diffusing capacity for carbon monoxide. Conclusion. We derived and validated an easily applied formula for determining pulmonary function in patients with SSc that identifies subgroups with a low, average, or high prevalence of PH. It provides information that is complementary to echocardiography and that should improve the selection of patients for RHC.Pulmonary hypertension (PH) occurs in up to 12% of patients with systemic sclerosis (SSc) (1). Early identification and treatment of PH is important and may improve survival (2). Although right-sided heart catheterization (RHC) remains the gold standard test for diagnosing PH, it is invasive and is not ideal as a screening test. Pulmonary function tests (PFTs) are recommended as part of annual screening tests in patients with SSc. However, it is not clear which patients require RHC, based on the findings of the PFTs.In this study, we explored the utility of pulmonary function testing and assessment of oxygen saturation in detecting PH in a large cohort of patients with SSc who had undergone RHC. We sought to develop a formula based on oxygen saturation and PFT results that would help to select patients for whom RHC is required. In order to derive and validate the formula, we split our sample into separate derivation and validation cohorts. We compared our formula to other pulmonary function test-based formulae. We then assessed the diagnostic Supported by the Royal Free Hampstead NHS Trust.

Objective. Pulmonary arterial hypertension (PAH) is a severe complication of connective tissue diseases (CTDs). This study aimed to investigate the clinical and hemodynamic characteristics and survival of anti-U1 RNP-positive patients with CTD-associated PAH, with a focus on systemic sclerosis (SSc)-associated PAH.Methods. We implemented a prospective database that included patients with CTD-associated PAH for whom there were clinical, autoantibody, and mortality data. We compared clinical and hemodynamic characteristics to anti-U1 RNP antibody status. We then assessed whether anti-U1 RNP antibodies could be a prognostic factor in CTD-associated PAH with a focus on SScassociated PAH.Results. We studied a total of 342 patients with CTD-associated PAH, of whom 36 (11%) were anti-U1 RNP antibody positive. Anti-U1 RNP-positive patients were younger and less functionally impaired than were anti-U1 RNP-negative patients in CTD-and SScassociated PAH. Hemodynamic parameters were similar in anti-U1 RNP-positive and anti-U1 RNP-negative patients. In CTD-associated PAH, anti-U1 RNP positivity was associated with decreased mortality in univariable analysis (hazard ratio 0.34 [95% confidence interval 0.18-0.65], P < 0.001). In multivariable analysis, anti-U1 RNP positivity was also associated with decreased mortality (hazard ratio 0.44 [95% confidence interval 0.20-0.97], P 5 0.043) independently of age, sex, functional parameters, lung involvement, and hemodynamic parameters. Results were similar in SSc-associated PAH, although the association between anti-U1 RNP positivity and survival did not reach significance in univariable (hazard ratio 0.47 [95% confidence interval 0.22-1.02], P 5 0.055) and multivariable (hazard ratio 0.47 [95% confidence interval 0.20-1.11], P 5 0.085) analyses.Conclusion. Anti-U1 RNP positivity was associated with distinct clinical characteristics and survival in CTD-and SSc-associated PAH. While hemodynamic parameters were similar in anti-U1 RNP-positive and anti-U1 RNP-negative patients, our results suggest that anti-U1 RNP positivity could be a factor protecting against mortality in CTD-and SSc-associated PAH.

The prevalence of CAD in patients with SSc is similar and not greater to that expected in individuals without SSc.

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Accidental hypothermic cardiac arrest in a central European urban area is rare. Prognosis was excellent in patients where hypothermic cardiac arrest was caused by intoxication.