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

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

Combination Pharmacotherapy in the Treatment of Pulmonary Arterial Hypertension

2013 ◽  
Vol 26 (1) ◽  
pp. 18-28 ◽  
Author(s):  
Kimberly L. Tackett ◽  
Gregory V. Stajich
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Combination Therapy ◽  
Arterial Hypertension ◽  
Therapeutic Option ◽  
Symptom Control ◽  
Right Ventricular Dysfunction ◽  
Functional Class ◽  
Current Therapy ◽  
Therapeutic Benefits ◽  
Pulmonary Arterial

Pulmonary arterial hypertension (PAH) is a disorder of the small pulmonary arteries characterized by progressive fibrotic and proliferative changes that result in an increased pulmonary vascular resistance. It is a progressive and debilitating disease that leads to right ventricular dysfunction, impairment in activity tolerance and eventually right-sided heart failure, and premature death. The treatment goals for PAH include improvement in symptoms, improvement in functional class and exercise class, decreased morbidity, and preventing mortality. Combination therapy in the treatment of PAH is an emerging therapeutic option. Combining therapies with differing mechanisms of action will maximize therapeutic benefits such as symptom control and increased rate of survival. The updated 2007 American College of Chest Physicians evidence-based clinical practice guidelines recommend combination therapy in functional classes III and IV if there is no improvement with current therapy or if there is deterioration in class. PAH monotherapy has been shown to improve symptoms, but the patients’ hemodynamic parameters may not be normalized, leading to further pulmonary vascular remodeling. Combination therapy offers an additional option for those patients who are unable to stabilize on monotherapy.

scholarly journals Combination therapy with pulmonary arterial hypertension targeted drugs and immunosuppression can be a useful strategy for sarcoidosis-associated pulmonary hypertension: a case report

2020 ◽  
Author(s):  
Keiko Sumimoto ◽  
Yu Taniguchi ◽  
Noriaki Emoto ◽  
Ken-Ichi Hirata
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Functional Class ◽  
Targeted Drugs ◽  
Appropriate Treatment ◽  
Pulmonary Arterial ◽  
Group 5 ◽  
Underlying Mechanisms

Abstract Background Pulmonary hypertension (PH) is one of the significant complications of sarcoidosis. In the clinical classification of PH proposed in the recent world symposium of PH 2018, sarcoidosis-associated PH is classified in Group 5. The mechanisms of sarcoidosis-associated PH are very heterogeneous. There is no evidence of effective treatment for this condition. Case summary We report a case of a 46-year-old female who developed severe pulmonary hypertension due to sarcoidosis. Her haemodynamics, exercise capacity, and functional class remarkably improved after the treatment with pulmonary arterial hypertension (PAH) targeted drugs including macitentan and tadalafil and secondary immunosuppressive therapy with corticosteroids. Discussion This case emphasizes the importance of considering the underlying mechanisms of sarcoidosis-associated PH in order to choose appropriate treatment.

scholarly journals Pulmonary arterial hypertension in Russia: six-year observation analysis of the National Registry

2019 ◽  
Vol 91 (1) ◽  
pp. 25-31 ◽  
Author(s):  
I E Chazova ◽  
O A Arkhipova ◽  
T V Martynyuk
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Congenital Heart ◽  
Heart Defects ◽  
Connective Tissue Diseases ◽  
National Registry ◽  
Current Therapy ◽  
Pulmonary Arterial

Aim. Assess the prevalence, clinical course, current therapy, and mortality in patients with pulmonary arterial hypertension (PAH) in the National Registry. Materials and methods. In the prospective study we included patients over 18 years of age with diagnosed PAH [idiopathic PAH (IPAH); Drug - and Toxin-Induced Pulmonary Hypertension; inherited PAH; PAH associated with congenital heart disease (PAH-CHD); PAH associated with systemic connective tissue disease (PAH-CTD); PAH associated with HIV infection (PAH-HIV); with portal pulmonary hypertension (portoPAH)]. The observation was carried out in 15 expert centers of Russia from 01.01.2012 to 31.12.2017. Results. Our registry included 470 patients with PAH: IPAH - 41.5%, PAH-CHD - 36%, PAH-CTD - 19.5%, inherited PAH - 0.4%, portoPAH - 1.9%, PAH-HIV - 0.4%, Drug - and Toxin-Induced PAH - 0.4%. The prevalence among women was 84%. The mean age at the time of patient enrollment in the registry for the overall group of PAH was 42.7±15.3 years. The distance in the 6-minute walking test was 361.3±129.3 m. Among all patients with PAH, 65% had functional class (FC) III/IV at the time of diagnosis, among IPAH - 62%. 69.9% received PAH-specific therapy, of which 62.1% - monotherapy, 32.7% - dual combination therapy, and 5.2% triple therapy. Sildenafil is the most commonly prescribed drug in the regimen of monotherapy. 31.6% of patients were treated with bosentan, 6.4% - riociguat, 3.4% - ambrisentan, 2.1% - macitentan and 2.0% iloprost. Survival of patients with PAH was 98.9% at 1 year of follow-up, 94.1% at 3 years and 86.0% at 5 years. Conclusion. The registry data indirectly indicates the need to increase efforts aimed at improving the diagnosis of systemic connective tissue diseases in adults, as well as congenital heart defects in children for timely surgical treatment. In recent years, PAH-specific drugs of the new generation have been introduced into clinical practice, but currently in Russia there are no parenteral prostanoids, which are recommended for the most severe patients.

scholarly journals Switching Strategy in the Management of Patients with Pulmonary Arterial Hypertension. The REPLACE Study Results

2020 ◽  
Vol 16 (6) ◽  
pp. 1009-1016
Author(s):  
E. V. Filippov
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Soluble Guanylate Cyclase ◽  
Functional Class ◽  
Patient Response ◽  
Risk Of Mortality ◽  
Study Results ◽  
Pulmonary Arterial ◽  
Phosphodiesterase Type ◽  
Dual Mechanism

The article is devoted to topical issues of management of patients with pulmonary arterial hypertension and strategies for switching to riociguat therapy in patients with insufficient clinical response against the background of phosphodiesterase type 5 (PDE-5) inhibitors. The role of the NO metabolic pathway in the development of pulmonary arterial hypertension (PAH) was shown. From the standpoint of pathogenesis, the importance of the effect of medication on this pathway through soluble guanylate cyclase (sGC) has been assessed. The effects of the drug riociguat, the only member of the sGC stimulant class approved for the treatment of PAH, are associated with a dual mechanism of action: riociguat sensitizes the sGC enzyme to endogenous NO by stabilizing the NO-sGC bond, and also directly stimulates the latter through another site of the bond, regardless of NO. The favorable efficacy and safety profile of riociguat has been demonstrated in the PATENT-1,2 studies. During therapy, patients showed an improvement in exercise tolerance, functional class, and a number of other indicators. Studies that indicate the feasibility of using a change in therapy in clinical practice are reviewed. In particular, the article provides a detailed analysis of the REPLACE study. It demonstrated a significantly greater likelihood of achieving clinical improvement and a significant decrease in the rate of development of clinical deterioration when switching patients with PAH from PDE-5 inhibitors to riociguat. This therapy was well tolerated and can be considered as a strategy for managing patients at intermediate risk with insufficient patient response to PDE-5 inhibitors. In conclusion, the need to plan the need for specific therapy for pulmonary arterial hypertension in the region is noted in order to rapidly escalate therapy to achieve a low risk of mortality in these patients.

scholarly journals Triple combination therapy with macitentan, riociguat, and selexipag in a patient with idiopathic pulmonary arterial hypertension (functional class III)

Kardiologiia ◽  
2021 ◽  
Vol 61 (10) ◽  
pp. 104-107
Author(s):  
A. A. Proshkina ◽  
N. A. Tsareva ◽  
G. V. Nekludova ◽  
S. N. Avdeev
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Combination Therapy ◽  
Arterial Hypertension ◽  
Clinical Manifestations ◽  
Functional Class ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Triple Combination ◽  
Class Iii ◽  
Triple Combination Therapy ◽  
Pulmonary Arterial

The article presents a clinical case of successful triple combination therapy in a female patient with functional class III idiopathic pulmonary arterial hypertension. Supplementing the previous macitentan and riociguat treatment with selexipag reduced the severity of clinical manifestations of pulmonary hypertension. Also, the treatment efficacy was demonstrated by improvement of laboratory and instrumental indexes. Time-related changes were evaluated at 3 months after initiation of the selexipag treatment.

Abstract 12455: Evaluation of N-Terminal Pro-B-type Natriuretic Peptide as a Therapeutic Response Biomarker in Group I Pulmonary Arterial Hypertension

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Aaron M Wolfson ◽  
Micheal L Maitland ◽  
Vasiliki Thomeas ◽  
Cherylanne Glassner ◽  
Mardi Gomberg-Maitland
Keyword(s):  
Heart Failure ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Roc Curve ◽  
Curve Analysis ◽  
Roc Curve Analysis ◽  
Goal Directed Therapy ◽  
Group I ◽  
Target Values ◽  
Pulmonary Arterial

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.

scholarly journals Oral treprostinil in transition or as add-on therapy in pediatric pulmonary arterial hypertension

2019 ◽  
Vol 9 (3) ◽  
pp. 204589401985647 ◽  
Author(s):  
D. Dunbar Ivy ◽  
Jeffrey A. Feinstein ◽  
Delphine Yung ◽  
Mary P. Mullen ◽  
Edward C. Kirkpatrick ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Pediatric Patients ◽  
Cardiopulmonary Exercise Testing ◽  
Functional Class ◽  
World Health ◽  
Walk Distance ◽  
Pulmonary Arterial ◽  
Health Organization

Treprostinil, a prostacyclin analogue, is approved for the treatment of pulmonary arterial hypertension (PAH) in adults. Transition from parenteral to oral treprostinil has been successfully accomplished in adults with PAH but not in children. In this multicenter study, pediatric patients treated with parenteral (Cohort 1) or inhaled (Cohort 2) treprostinil were transitioned to oral treprostinil. Prostacyclin-naïve individuals on background oral PAH therapy received oral treprostinil as add-on therapy (Cohort 3). Successful transition was oral treprostinil dose maintenance through week 24. Patients were monitored for adverse events (AEs), 6-min walk distance (6MWD), PAH symptoms, World Health Organization (WHO) Functional Class (FC), cardiac magnetic resonance imaging (cMRI), cardiopulmonary exercise testing (CPET), and quality of life through 24 weeks. A total of 32 patients were enrolled in the study; 23 (72%) were girls (mean age = 12.2 years). All patients were on background oral PAH therapy. Overall, patients (96.9%) maintained transition to oral treprostinil; one patient (Cohort 1) transitioned to oral treprostinil, then back to parenteral after experiencing syncope and WHO FC change from II to III. Cohorts 1, 2, and 3 received a final mean oral treprostinil dose of 5.6, 3.3, and 4.5 mg t.i.d., respectively. All cohorts had variable changes in 6MWD, cMRI, and CPET. Overall, 12 serious AEs were reported. All patients had drug-related AEs including headache (81%), diarrhea (69%), nausea (66%), vomiting (66%), and flushing (56%). Pediatric patients maintained transition to oral treprostinil with preservation of exercise capacity and WHO FC. Prostanoid-related AEs were most common and similar to those reported in adults.

Sign in / Sign up

Export Citation Format

Share Document