Caregiver Burden in Patients with Pulmonary Hypertension

2020 ◽  
pp. 105477382097731
Author(s):  
Derya Kocakaya ◽  
Özge Keniş-Coşkun ◽  
Betül Şentürk-Saraç ◽  
Bedrettin Yıldızeli ◽  
Bülent Mutlu ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Caregiver Burden ◽  
Significant Negative Correlation ◽  
Functional Class ◽  
World Health ◽  
Clinical Parameters ◽  
Systolic Pulmonary Arterial Pressure ◽  
Burden Scale ◽  
Pulmonary Arterial ◽  
Health Organization

Both quality of life (QoL) and caregiver burden are essential constructs in patients with pulmonary hypertension (PH) however; their relationship has never been investigated before. The aim of this study was to evaluate if there was any relationship between patients’ QoL and caregiver burden. Patients with PH and their caregivers were included. Patients’ age, sex, World Health Organization (WHO) functional class, systolic pulmonary arterial pressure (sPAP), and six-minute walk distances (6MWD) were recorded. Patients’ QoL was assessed using emPHasis-10 and caregiver burden with the Zarit Caregiver Burden Scale. 72 patient-caregiver dyads were included. Caregiver burden was significantly correlated with the QoL( r = 0.39 p < .003), but was not correlated with other clinical parameters. Patients’ QoL showed significant negative correlation with the 6MWD( r = −0.46 p < .005). There is a moderate correlation between QoL and caregiver burden. Clinical parameters influence QoL, but they do not affect caregiver burden.

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 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 Barriers and Solutions to Developing Future Therapies for Pulmonary Hypertension

2018 ◽  
Vol 17 (4) ◽  
pp. 159-165
Author(s):  
Brian Graham ◽  
Peter Fernandes ◽  
Sue Gu
Keyword(s):  
Clinical Trials ◽  
Pulmonary Hypertension ◽  
Phase 1 ◽  
Unmet Need ◽  
Orphan Drugs ◽  
World Health ◽  
Vasodilator Therapy ◽  
Scarce Resources ◽  
Pulmonary Arterial ◽  
Health Organization

Pulmonary hypertension (PH) and its subset, pulmonary arterial hypertension (PAH), are rare diseases with a significant unmet need. Between the 1980s and 2010s, the 5-year survival rate for PAH after diagnosis improved from 34% to 65%,12 but remains unacceptably low. Since the introduction of vasodilator therapy, 34 important advances have been made in the understanding of the disease pathophysiology and development of targeted therapies. There are now 14 US Food and Drug Administration (FDA)-approved therapies that target 3 distinct pathways that contribute to PAH, and additional therapeutic targets are currently under investigation in phase 1, 2, and 3 clinical trials.5 However, there have been major challenges in PH medication development to date, including: 1) only one medication approved for pediatric PAH; 2) focusing on vasodilator therapy rather than targeting the underlying pathogenesis of the disease; 3) no medications approved for PH World Health Organization (WHO) Groups 2, 3, and 5; and 4) several recent high-profile clinical failures after promising preclinical studies.The focus and goal of the PH research community should be directed at identifying new options and solutions for patients. The field must ensure that the approaches used for clinical trials to develop orphan drugs maximize the scarce resources available for recruiting subjects, and are directed toward making safe and effective therapies available in a timely manner. Therefore, there is a critical need to coordinate and harmonize innovative approaches within the field, including strengthening translational research to deliver promising candidates and optimize the designs, endpoints, and biomarkers to conduct safe and efficient clinical trials.

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 Liver transplantation in a patient with hereditary haemorrhagic telangiectasia and pulmonary hypertension

2019 ◽  
Vol 9 (4) ◽  
pp. 204589401989667
Author(s):  
Kentaro Ejiri ◽  
Satoshi Akagi ◽  
Kazufumi Nakamura ◽  
Naofumi Amioka ◽  
Keishi Ichikawa ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Pulmonary Hypertension ◽  
World Health Organization ◽  
Arteriovenous Malformations ◽  
Functional Class ◽  
Arteriovenous Shunt ◽  
World Health ◽  
Right Atrial ◽  
Hereditary Haemorrhagic Telangiectasia ◽  
Health Organization

Hereditary haemorrhagic telangiectasia or Rendu-Osler-Weber syndrome is a systemic vascular disease with autosomal dominant inheritance, mucocutaneous telangiectasia, and repeated nasal bleeding due to vascular abnormalities. Hereditary haemorrhagic telangiectasia may occasionally lead to complications, including arteriovenous malformations and pulmonary hypertension. We present a case of a 52-year-old female patient with hereditary haemorrhagic telangiectasia who was referred to our hospital for treatment of pulmonary hypertension. She had been diagnosed with hereditary haemorrhagic telangiectasia during adolescence and was being followed up. Six months prior to presentation, she had undergone coil embolization for pulmonary haemorrhage due to pulmonary arteriovenous malformations. She was in World Health Organization functional class IV, with a mean of pulmonary arterial pressure of 38 mmHg, a pulmonary capillary wedge pressure of 10 mmHg, and a right atrial pressure of 22 mmHg. A contrast-enhanced computed tomography angiography showed large arteriovenous malformations in the liver. Right heart catheterization revealed an increase in oxygen saturation in the inferior vena cava between the supra- and infra-hepatic veins, low pulmonary vascular resistance, and high right atrial pressure. Hence, she was diagnosed with hereditary haemorrhagic telangiectasia with pulmonary hypertension due to major arteriovenous shunt resulting from arteriovenous malformations in the liver. Therefore, we considered liver transplantation as an essential treatment option. She underwent cadaveric liver transplantation after a year resulting in dramatic haemodynamic improvement to World Health Organization functional class I. Liver transplantation is a promising treatment in patients with hereditary haemorrhagic telangiectasia and pulmonary hypertension resulting from arteriovenous shunt caused by arteriovenous malformations in the liver.

scholarly journals An Open-Label, Multicentre Pilot Study of Bosentan in Pulmonary Arterial Hypertension Related to Congenital Heart Disease

2006 ◽  
Vol 13 (8) ◽  
pp. 415-420 ◽  
Author(s):  
Reda Ibrahim ◽  
John T Granton ◽  
Sanjay Mehta
Keyword(s):  
Oxygen Saturation ◽  
Short Form ◽  
Functional Class ◽  
World Health ◽  
Short Form Health Survey ◽  
Form Health ◽  
Efficacious Treatment ◽  
Pulmonary Arterial ◽  
Health Organization ◽  
Short Form Health

BACKGROUND: Bosentan has been shown to be a safe and efficacious treatment for idiopathic pulmonary arterial hypertension (PAH) and PAH associated with connective tissue disease. However, there are limited studies examining the benefits of bosentan in PAH associated with congenital heart disease (CHD).OBJECTIVE: The aim of the present pilot study was to explore the safety and efficacy of bosentan in patients with PAH associated with CHD.PATIENTS AND METHODS: In the present study, 11 patients with PAH associated with CHD were enrolled to receive bosentan for a minimum of 16 weeks (62.5 mg twice a day for four weeks; thereafter 125 mg twice a day). Safety was assessed by monitoring adverse events, oxygen saturation, systemic blood pressure, pulse, complete blood count and liver function tests. Efficacy was assessed by the World Health Organization functional class, 6 min walk test (6-MWT), modified Borg dyspnea index, echocardiography and the 36-item short form health survey.RESULTS: Ten patients completed the 16-week treatment period (one patient withdrew). Bosentan was not associated with a deterioration in resting oxygen saturation (83.0±4.6% at week 16 versus 81.9±6.1% at baseline; P=0.402), or a deterioration in post-6-MWT oxygen saturation (70.1±10.9% at week 16 versus 68.7±15.1% at baseline; P=0.747). Two patients experienced three serious adverse events. The distance walked in 6 min improved significantly by 28 m (P=0.005) at week 16 compared with baseline, and the modified Borg dyspnea index also improved at week 16 compared with baseline (P=0.050). The World Health Organization functional class improved from class III to class II for five of 10 patients (50%). Patients’ self-rated quality of life (36-item short form health survey) demonstrated a non-significant improvement in each of the eight domains. Obtaining reliable echocardiographic measurements was difficult. Most echocardiographic parameters were only measurable on few patients, and none were measured on all patients, questioning the usefulness of echocardiography as a measuring tool for patients with complex CHD.CONCLUSION: Bosentan was not associated with worsening of resting oxygen saturation or exercise systemic oxygen saturation, suggesting its potential as a safe treatment option for patients with PAH associated with CHD. Improved 6-MWT and the modified Borg dyspnea index also suggested the possibility of bosentan as an efficacious treatment option for these patients. The results of the present study provide evidence for the need and feasibility of a large randomized, placebo-controlled clinical trial.

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.

Pulmonary Hypertension - A Distinct Perspective

2021 ◽  
Vol 8 (26) ◽  
pp. 2349-2362
Author(s):  
Ankit Kumar Sahu ◽  
Sudeep Kumar
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Pressure ◽  
Primary Treatment ◽  
World Health ◽  
Mean Pulmonary Arterial Pressure ◽  
Pulmonary Vasoconstriction ◽  
Pulmonary Arterial ◽  
Health Organization ◽  
Pulmonary Vascular Remodelling

In this review, we have discussed pulmonary hypertension including definition, classification, pathophysiology, epidemiology, diagnosis and treatment of each class of PH as per latest available data and guidelines. Pulmonary hypertension is defined as an increase in mean pulmonary arterial pressure of ≥ 20 mmHg at rest. The clinical classification of pulmonary hypertension (as per world health organization) includes five different classes constructed on the basis of clinical causes of pulmonary hypertension. The main pathophysiology involved in the development of pulmonary hypertension of all types is prolonged pulmonary vasoconstriction caused by multiple factors which ultimately leads to pulmonary vascular remodelling (structural as well as functional). The primary treatment of pulmonary hypertension comprises of correction/control of underlying cause of pulmonary hypertension. The prevalence of pulmonary hypertension is increasing in developed as well as developing countries and thus its awareness is important for timely diagnosis and management of this chronic condition. The main aim of our review is to provide all the necessary information regarding pulmonary hypertension in one document. KEYWORDS Pulmonary Hypertension, Chronic Thromboembolism, Left Heart Disease, Lung Disease, Pulmonary Arterial Hypertension

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