Faculty Opinions recommendation of Treatment of group I pulmonary arterial hypertension with carvedilol is safe.

Purpose: Goal directed management of left heart failure with an NT-proBNP target-based approach has some evidence of providing a survival benefit. To evaluate the potential utility of serial NT-proBNP measurements for goal-directed therapy in right heart failure we retrospectively assessed NT-proBNP as a predictor for survival in Group I pulmonary arterial hypertension (PAH) patients. Methods: We identified 103 Group I PAH patients from a pulmonary hypertension registry who had baseline elevated NT-proBNP prior to either the initiation or escalation of therapy and at least two serial NT-proBNP measurements. In a two-step process, we (1) estimated baseline NT-proBNP and slope (rate of change of NT-proBNP) with a linear mixed-effects model using all patient data and then (2) compared the power of serial versus single measurements in predicting survival with measured and model-derived values of baseline NT-proBNP with a Receiver Operative Characteristic (ROC) curve analysis . Survival was determined using the Kaplan-Meier methodology. Results: ROC curve analysis revealed significantly higher AUC for model-derived NT-proBNP values compared to the measured values (AUC: for baseline 0.74 vs 0.66, p= 0.009; for slope 0.78 vs 0.66, p= 0.02). Optimal cutpoints for prediction of survival on baseline NT-proBNP were 2012 (measured) vs. 1810 (model-derived) pg/mL. The optimal cutpoint for model-derived change in NT-proBNP was -0.004 log10pg/mL/month. Sensitivity, specificity, and negative predictive values for the three predictor variables were: 64%, 67%, 80% (measured baseline NT-proBNP), 61%, 80%, 81% (model-derived baseline NT-proBNP) and 73%, 57%, 85% (model-derived slope). Conclusions: In PAH patients, serial NT-proBNP measurements better predict survival than single measurements. This retrospective finding reveals that changes in NT-proBNP are associated with overall survival in PAH patients, and set initial target values for a pilot prospective study of NT-proBNP goal-directed therapy.

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Growth differentiation factor-15 levels in the blood around the pulmonary artery is associated with hospitalization for heart failure in patients with pulmonary arterial hypertension

Pulmonary Circulation ◽

10.1177/2045894020962948 ◽

2020 ◽

Vol 10 (4) ◽

pp. 204589402096294

Author(s):

Wei-Ting Chang ◽

Jhih-Yuan Shih ◽

Yu-Wen Lin ◽

Zhih-Cherng Chen ◽

Jun-Neng Roan ◽

...

Keyword(s):

Heart Failure ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Right Atrial ◽

Heart Catheterization ◽

Growth Differentiation Factor 15 ◽

Arterial Blood ◽

Differentiation Factor ◽

Group I ◽

Pulmonary Arterial

Despite no significant differences of growth differentiation factor-15 expressions in peripheral, right atrial, and right ventricular blood, in the pulmonary arterial blood, there was a significantly high level of growth differentiation factor-15 in Group I pulmonary arterial hypertension patients subsequently developing heart failure. During right heart catheterization, collecting pulmonary blood samples is suggested to measure growth differentiation factor-15.

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Demographic and clinical differences between idiopathic and scleroderma-related pulmonary arterial hypertension: Russian National Registry analysis

Systemic Hypertension ◽

10.26442/sg29142 ◽

2016 ◽

Vol 13 (2) ◽

pp. 65-72

Author(s):

N N Yudkina ◽

E G Valeeva ◽

I N Taran ◽

E V Nikolaeva ◽

V M Paramonov ◽

...

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

National Registry ◽

Functional Class ◽

Current Therapy ◽

Group I ◽

Pulmonary Arterial ◽

The Moment ◽

Registry Analysis

Pulmonary arterial hypertension associated with systemic sclerosis (SSc-PAH) and idiopathic pulmonary arterial hypertension (IPAH) belong to group I in the clinical classification of PH, but there is evidence for significant differences in their survival due to current therapy. Objective: the objective of this report is to compare pts with (SSc-PAH) and (IPAH) based on data of Russian National Registry. Patients and methods: in the study we included 52 pts with IPAH and 50 with SSc-PAH. There were no differences in functional class (FC). Diagnosis was based on RHC. Results. At the moment of diagnosis average age of patients with SSc-PAH was 15 year higher (p

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Electrocardiographic Changes in Patients Responding to Treatment with Group I Pulmonary Arterial Hypertension

Southern Medical Journal ◽

10.1097/smj.0000000000000021 ◽

2013 ◽

Vol 106 (11) ◽

pp. 618-623 ◽

Cited By ~ 2

Author(s):

David Snipelisky ◽

Charles Burger ◽

Brian Shapiro ◽

Fred Kusumoto

Keyword(s):

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Group I ◽

Electrocardiographic Changes ◽

Pulmonary Arterial

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The place of riociguat in the treatment of patients with pulmonary arterial hypertension associated with systemic connective tissue diseases

Kardiologiia ◽

10.18087/cardio.2020.9.n1189 ◽

2020 ◽

Vol 60 (9) ◽

pp. 92-101

Author(s):

N. A. Shostak ◽

A. A. Klimenko ◽

N. A. Demidova

Keyword(s):

Pulmonary Arterial Hypertension ◽

Connective Tissue ◽

Arterial Hypertension ◽

Lupus Erythematosus ◽

Soluble Guanylate Cyclase ◽

Connective Tissue Diseases ◽

Genetically Engineered ◽

Group I ◽

Endogenous Nitric Oxide ◽

Pulmonary Arterial

Pulmonary hypertension (PH) can develop in different systemic autoimmune rheumatic diseases (SARD), such as systemic scleroderma (SSD), systemic lupus erythematosus, rheumatoid arthritis, and mixed connective tissue disease In most cases, patients with SARD develop WHO group I PH (pulmonary arterial hypertension associated with systemic connective tissue diseases, PAH-SCTD). General prevalence of this pathology reaches 15 cases per million adults. Most cases of PAH-SCTD are induced by SSD. Survival of PAH-SCTD patients is generally lower than survival of patients with other forms of LAH. Treatment of any SARD, including in LAH, implies a complex approach using glucocorticoids, disease-modifying anti-rheumatic drugs (cyclophosphamide, methotrexate, azathioprine, and others), and genetically engineered biologics. Specific targeted therapy is indicated for most patients with PAH-SCTD. The representative of a new class (soluble guanylate cyclase (sGC) stimulators), riociguat, has been approved for the treatment of PAH. This drug has a unique double mechanism of action: (i) sGC sensibilization to endogenous nitric oxide (NO) by stabilizing the NO-sGC bond; and (ii) direct, NO-independent sGC stimulation. For patients with PAH-SCTD, riociguat is the major alternative to phosphodiesterase-5 inhibitors both as monotherapy and combination therapy.

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Abstract 20267: Diminished Levels of Circulating Bone Morphogenetic Protein 9 Identify Pulmonary Arterial Hypertension Associated With Liver Disease

Circulation ◽

10.1161/circ.130.suppl_2.20267 ◽

2014 ◽

Vol 130 (suppl_2) ◽

Author(s):

Ivana Nikolic ◽

Samuel D Paskin-Flerlage ◽

Rajeev Malhotra ◽

Lai-Ming Yung ◽

Raymond T Chung ◽

...

Keyword(s):

Liver Disease ◽

Pulmonary Arterial Hypertension ◽

Arterial Hypertension ◽

Bone Morphogenetic Protein ◽

Strong Predictor ◽

Healthy Individuals ◽

Group I ◽

Morphogenetic Protein ◽

Pulmonary Arterial ◽

Circulating Levels

Background: Dysregulated bone morphogenetic protein (BMP) signaling is thought to contribute to the pathogenesis of pulmonary arterial hypertension (PAH). BMP9 can be detected in the circulation at physiologically active concentrations, and is thought to serve as a vascular endothelial quiescence factor. Aim: We hypothesized that circulating levels of BMP9 might be associated with altered endothelial homeostasis, and could help predict the presence of PAH or distinguish between PAH of distinct etiologies. Methods: Circulating levels of BMP9, and its biological inhibitor, soluble Endoglin (sEng), were measured in the plasma of 96 patients with various etiologies of pulmonary hypertension and 28 healthy individuals using enzyme-linked immunoassays. Results: Patients with Group I PAH (n=44) had significantly lower levels of BMP9 compared to healthy individuals (188 ± 22 pg/mL vs 254 ± 17 pg/mL, mean ± SEM, p=0.001). BMP9 was significantly decreased in PAH associated with connective tissue disease (p=0.02), but was most profoundly decreased in patients with portopulmonary hypertension (82 ± 27 pg/mL, p<0.0001 vs controls). BMP9 was a strong predictor of concomitant liver disease (ROC AUC=0.920), correlating positively with serum albumin and platelet counts, and negatively with INR, total bilirubin, cardiac index and MELD score among Group I PAH patients (

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