Recent Updates in Both the Diagnosis and Treatment Options that Impact the Clinical Treatment of Pulmonary Arterial Hypertension

2010 ◽  
Vol 2 ◽  
pp. CMT.S4192
Author(s):  
Charles D. Burger
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Treatment Plan ◽  
Treatment Options ◽  
Pharmaceutical Formulations ◽  
World Health ◽  
New Agents ◽  
Pulmonary Arterial ◽  
New Treatment ◽  
Health Organization

The evaluation and management of pulmonary arterial hypertension (PAH) is a rapidly evolving area of subspecialty medicine requiring regular clinical updates. Most notably are changes in the World Health Organization diagnostic scheme whereby the clinician categorizes the correct type of pulmonary hypertension in order direct the most specific evaluation and treatment plan. In addition, there have been several changes in both the FDA-approved pharmaceutical formulations and new agents for the treatment of PAH. This review will provide an update in these areas and more importantly, guidance to the clinician on the most appropriate utilization of these new treatment options.

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.

scholarly journals Epidemiology and initial management of pulmonary arterial hypertension: real-world data from the Hellenic pulmOnary hyPertension rEgistry (HOPE)

2019 ◽  
Vol 9 (3) ◽  
pp. 204589401987715 ◽  
Author(s):  
Alexandra Arvanitaki ◽  
Maria Boutsikou ◽  
Anastasia Anthi ◽  
Sotiria Apostolopoulou ◽  
Aikaterini Avgeropoulou ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Functional Class ◽  
World Health ◽  
Real World Data ◽  
Treatment Data ◽  
Risk Patients ◽  
Pulmonary Arterial ◽  
Health Organization

Pulmonary arterial hypertension (PAH) is a heterogenous clinical entity with poor prognosis, despite recent major pharmacological advances. To increase awareness about the pathophysiology, epidemiology, and management of the disease, large national registries are required. The Hellenic pulmOnary hyPertension rEgistry (HOPE) was launched in early 2015 and enrolls patients from all pulmonary hypertension subgroups in Greece. Baseline epidemiologic, diagnostic, and initial treatment data of consecutive patients with PAH are presented in this article. In total, 231 patients with PAH were enrolled from January 2015 until April 2018. At baseline, about half of patients with PAH were in World Health Organization functional class II. The majority of patients with PAH (56.7%) were at intermediate 1-year mortality risk, while more than one-third were low-risk patients, according to an abbreviated risk stratification score. Half of patients with PAH were on monotherapy, 38.9% received combination therapy, while prostanoids were used only in 12.1% of patients. In conclusion, baseline data of the Greek PAH population share common characteristics, but also have some differences with other registries, the most prominent being a better functional capacity. This may reflect earlier diagnosis of PAH that in conjunction with the increased proportion of patients with atypical PAH could partially explain the preference for monotherapy and the limited use of prostanoids in Greece. Nevertheless, early, advanced specific therapy is strongly recommended.

scholarly journals Respiratory rate modulation improves symptoms in patients with pulmonary hypertension

2021 ◽  
Vol 9 ◽  
pp. 205031212110539
Author(s):  
Barbro Kjellström ◽  
Bodil Ivarsson ◽  
Lise-Lotte Landenfelt Gestré ◽  
Henrik Ryftenius ◽  
Magnus Nisell
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Respiratory Rate ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
World Health ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
Respiratory Modulation ◽  
Health Organization

Background: Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension are chronic diseases with a severe symptom burden. Common symptoms are dyspnoea at light activity and general fatigue that limits daily activities. Respiratory modulation by device-guided breathing decreased symptoms in patients with heart failure. The aim of this pilot study was to investigate if respiratory modulation could improve symptoms of dyspnoea in patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension. Method: Adult patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension with symptoms of dyspnoea at rest or light activity performed home-based respiratory modulation by device-guided breathing 20 min a day for 3 months. Patients were on stable disease-specific treatment ⩾3 months and willing to undergo all study procedures. Dyspnoea score, World Health Organization class, physical status, N-terminal pro b-type natriuretic peptide, quality of life, respiratory rate and 6-min walk distance were assessed before and after 3 months with respiratory modulation. Results: Nine patients with pulmonary arterial hypertension and five with chronic thromboembolic pulmonary hypertension completed the study protocol. Mean age was 71 ± 14 years, and 11 were women. After 3 months of respiratory modulation, dyspnoea score (−0.6, p = 0.014), respiratory rate at rest (−3 breaths/min, p = 0.013), World Health Organization class (−0.3, p = 0.040), quality of life (EuroQol Visual Analogue Scale +5 points, not significant) and decreased N-terminal pro b-type natriuretic peptide (−163 ng/L, p = 0.043) had improved. The fatigue and respiratory rate after the 6-min walk decreased while the 6-min walk distance remained unchanged. Conclusion: Patients with pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension that used device-guided breathing for 3 months improved symptoms of dyspnoea and lowered the respiratory rate at rest and after exercise.

scholarly journals Rates of hospitalization associated with the use of aldosterone receptor antagonists in patients with pulmonary arterial hypertension

2019 ◽  
Vol 9 (3) ◽  
pp. 204589401986842
Author(s):  
Morgan E. Corkish ◽  
Lauren T. Devine ◽  
Megan M. Clarke ◽  
Brian P. Murray ◽  
Lisa J. Rose-Jones
Keyword(s):  
Heart Failure ◽  
Retrospective Study ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
World Health ◽  
Aldosterone Receptor ◽  
Pulmonary Arterial ◽  
Retrospective Study Design ◽  
Health Organization ◽  
Group 1

We show that spironolactone use was associated with an increased rate of all-cause hospitalizations, but no difference in hospitalizations for heart failure or pulmonary arterial hypertension, in patients with World Health Organization Group 1 pulmonary arterial hypertension. A possible reason for this finding is confounding from retrospective study design.

scholarly journals New Oral Drugs for Pulmonary Arterial Hypertension: Macitentan, Riociguat, and Treprostinil

2014 ◽  
Vol 13 (2) ◽  
pp. 68-75 ◽  
Author(s):  
Marco A. Caccamo ◽  
Tim Lahm
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Soluble Guanylate Cyclase ◽  
Clinical Trial Design ◽  
The United States ◽  
World Health ◽  
Oral Drugs ◽  
Prostacyclin Analogue ◽  
Pulmonary Arterial ◽  
Health Organization

In 2013, the US Food and Drug Administration (FDA) approved 3 new oral drugs for the treatment of pulmonary arterial hypertension (PAH; World Health Organization [WHO] Group 1 pulmonary hypertension [PH]). These include the endothelin receptor antagonist macitentan, the soluble guanylate cyclase stimulator riociguat, and the prostacyclin analogue treprostinil. In addition, riociguat was approved for the treatment of patients with inoperable or postsurgery recurrent or persistent chronic thromboembolic PH (CTEPH; WHO Group 4 PH). The approval of these drugs has several important clinical implications: first, in a disease where many of the currently available treatments are complicated by significant side effects and/or complex administration regimens, the availability of new oral drugs clearly represents a valuable addition to the armamentarium. Second, the macitentan study was the first long-term, event-driven trial to be published in the PAH field, making the results more robust and paving the way for improved clinical trial design in the future. Third, riociguat is the first FDA-approved medical treatment regimen for selected CTEPH patients, thus providing a critical treatment option for patients with inoperable or recurrent/persistent CTEPH. Lastly, the approval of oral treprostinil made this drug the first oral prostacyclin analogue to be available in the United States. In this article, the authors will discuss the mechanisms of action of macitentan, riociguat, and oral treprostinil; review the landmark trials that led to the FDA approval of these drugs, and discuss their clinical use.

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