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 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 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 Epidemiology and Management of Chronic Thromboembolic Pulmonary Hypertension in Greece. Real-World Data from the Hellenic Pulmonary Hypertension Registry (HOPE)

2021 ◽  
Vol 10 (19) ◽  
pp. 4547
Author(s):  
Eftychia Demerouti ◽  
Panagiotis Karyofyllis ◽  
Vassilios Voudris ◽  
Maria Boutsikou ◽  
George Anastasiadis ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Functional Class ◽  
World Health ◽  
Real World Data ◽  
Significant Difference ◽  
Thromboembolic Pulmonary Hypertension ◽  
History Of ◽  
Pulmonary Arterial ◽  
Health Organization

Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is a rare disease with poor prognosis if left untreated, characterized by pulmonary vascular bed obstruction due to unresolving thromboembolic material. The Hellenic pulmonary hypertension registry (HOPE) was launched in Greece in early 2015 and enrolls patients from all pulmonary hypertension subgroups in Greece. In total, 98 patients with CTEPH were enrolled from January 2015 until November 2019. Of these patients, 55.1% represented incident population, 50% were classified in the World Health Organization functional class II and 49% had a history of acute pulmonary embolism. The median values of pulmonary vascular resistance (PVR) and cardiac index were 7.4 (4.8) WU and 2.4 (1.0) L/min/m2, respectively, the mean diffusing capacity for carbon monoxide was 74.8 ± 20.6%, the median 6-minute walk distance was 347 (220) meters and the median value of N Terminal-pro brain natriuretic peptide was 506.0 (1450.0) pg/mL. In total, 60.2% of the patients were under pulmonary arterial hypertension-targeted therapy at the time of enrolment; specifically, riociguat was received by 35.7% of the patients and combination therapy was the preferred strategy for 16% of the patients. In total, 74 patients were evaluated for pulmonary endarterectomy (PEA), 34 (45.9%) were assessed as operable but only 23 of those (31.1%) finally underwent PEA. The remaining 40 patients were ineligible for PEA according to the operability assessment and 13 (17.6%) of them underwent balloon pulmonary angioplasty. The age of the non-operable patients was significantly higher than the operable patients (p < 0.001), while there was no significant difference with regard to the history of coagulopathies between the operable and non-operable patients (p = 0.33).

scholarly journals Dasatinib-Induced Pulmonary Arterial Hypertension Treated with Upfront Combination Therapy

2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
Makoto Nishimori ◽  
Tomoyuki Honjo ◽  
Kenji Kaihotsu ◽  
Naohiko Sone ◽  
Sachiko Yoshikawa ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Combination Therapy ◽  
Arterial Hypertension ◽  
Rare Complication ◽  
Functional Class ◽  
World Health ◽  
First Line Therapy ◽  
Mean Pulmonary Arterial Pressure ◽  
Pulmonary Arterial ◽  
Health Organization

Pulmonary arterial hypertension (PAH) is a rare complication of dasatinib that was approved as a first-line therapy for chronic myelocytic leukemia (CML). A 24-year-old man presenting dyspnea at rest and leg edema was admitted to our hospital. He had been diagnosed with CML and prescribed dasatinib for 4 years. Chest X-ray showed significant bilateral pleural effusion and heart enlargement. Echocardiography revealed interventricular septal compression and elevated peak tricuspid regurgitation pressure gradient of 66.7 mmHg indicating severe pulmonary hypertension. After the other specific diseases to provoke PAH were excluded, he was diagnosed with dasatinib-induced PAH. Despite discontinuation of dasatinib and intravenous administration of diuretic for two weeks, World Health Organization (WHO) functional class was still II and mean pulmonary arterial pressure (PAP) was high at 37 mmHg. Therefore, we administered sildenafil and bosentan together as an upfront combination therapy three weeks after dasatinib discontinuation. Six months later, his symptoms improved to WHO functional class I and mean PAP was decreased to 31 mmHg. Although PAH is a rare complication of dasatinib, symptomatic patients prescribed with dasatinib should have an echocardiogram for PAH screening. Moreover, the upfront combination therapy would be a useful option for symptomatic patients after discontinuation of dasatinib.

scholarly journals Severe pulmonary hypertension in lung disease: phenotypes and response to treatment

2015 ◽  
Vol 46 (5) ◽  
pp. 1378-1389 ◽  
Author(s):  
Melanie J. Brewis ◽  
Alistair C. Church ◽  
Martin K. Johnson ◽  
Andrew J. Peacock
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lung Disease ◽  
Functional Class ◽  
World Health ◽  
Response To Treatment ◽  
Walking Distance ◽  
Vasodilator Therapy ◽  
Pulmonary Arterial

Pulmonary hypertension (PH) due to lung disease (World Health Organization (WHO) group 3) is common, but severe PH, arbitrarily defined as mean pulmonary artery pressure ≥35 mmHg is reported in only a small proportion. Whether these should be treated as patients in WHO group 1 (i.e.pulmonary arterial hypertension) with PH-targeted therapies is unknown.We compared the phenotypic characteristics and outcomes of 118 incident patients with severe PH and lung disease with 74 idiopathic pulmonary arterial hypertension (IPAH) patients, all treated with pulmonary vasodilators.Lung disease patients were older, more hypoxaemic, and had lower gas transfer, worse New York Heart Association functional class and lower 6-min walking distance (6MWD) than IPAH patients. Poorer survival in those with lung disease was driven by the interstitial lung disease (ILD) cohort.In contrast to IPAH, where significant improvements in 6MWD and N-terminal pro-brain natruiretic peptide (NT-proBNP) occurred, PH therapy in severe PH lung disease did not lead to improvement in 6MWD or functional class, but neither was deterioration seen. NT-proBNP decreased from 2200 to 1596 pg·mL−1(p=0.015). Response varied by lung disease phenotype, with poorer outcomes in patients with ILD and emphysema with preserved forced expiratory volume in 1 s. Further study is required to investigate whether vasodilator therapy may delay disease progression in severe PH with lung disease.

Risk assessment and treatment goals in patients presenting with pulmonary arterial hypertension

ESC CardioMed ◽  
2018 ◽  
pp. 2560-2562
Author(s):  
Marius M. Hoeper
Keyword(s):  
Risk Assessment ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Ventricular ◽  
Right Ventricular Function ◽  
Functional Class ◽  
Low Risk ◽  
World Health ◽  
Pulmonary Arterial ◽  
Health Organization

Risk stratification in patients with pulmonary arterial hypertension is based on a multidimensional approach that utilizes clinical features, World Health Organization functional class, exercise capacity, biomarkers, and evaluation of right ventricular function by imaging and/or invasive measurements. Risk assessment at the time of diagnosis determines the initial treatment strategy, while risk assessment during follow-up is performed to identify disease progression and the need for treatment modifications. A low-risk category is characterized by non-progressive disease, absence of signs of right heart failure and syncope, World Health Organization functional class I or II with normal or near normal exercise capacity, normal or near normal plasma levels of B-type natriuretic peptide, and well-preserved right ventricular function as determined by echocardiography, cardiovascular magnetic resonance imaging, or right heart catheterization. Achieving and maintaining a low-risk status is a major objective in the treatment of patients with pulmonary arterial hypertension.

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