Improving Survival in Patients with Pulmonary Arterial Hypertension: Focus on Intravenous Epoprostenol

Demerouti, Eftychia; Karyofyllis, Panagiotis; Manginas, Athanassios; Anthi, Anastasia; Karatasakis, George; Athanassopoulos, George; Voudris, Vasilios

doi:10.1007/s40256-018-00319-z

Cited by 10 publications

(18 citation statements)

References 55 publications

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“…1,2 However, the satisfactory functional status (WHO I/II) of the majority of patients in our cohort, in contrast to other European registries, may partially explain the low percentage of prostanoid use in this population, albeit a more aggressive approach could also be considered. 26 Additionally, the intravenous route of administration along with the known side effects might have contributed to the low percentage of use of parenteral prostanoids as monotherapy or combination therapy in our cohort. A limitation of this study is the relatively small sample size which does not yet permit the application of more sophisticated statistical methods that would provide reliable prognostic information.…”

Section: Discussionmentioning

confidence: 96%

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

Arvanitaki

Boutsikou²,

Anthi

et al. 2019

Pulm. circ.

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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.

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Section: Discussionmentioning

confidence: 96%

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

Arvanitaki

Boutsikou²,

Anthi

et al. 2019

Pulm. circ.

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“…In PAH patients the prostacyclin analogue intravenous epoprostenol has been found to significantly increase transplant free survival [10,14,15]. Intravenous epoprostenol is reserved for patients with WHO class IV symptoms, where it is used in combination with one or both of an endothelin receptor antagonist and a phospho-di-esterase 5 inhibitor [16,17].…”

Section: Discussionmentioning

confidence: 99%

Surgical Management of Giant Pulmonary Artery Aneurysm in Patients with Severe Pulmonary Arterial Hypertension

Gajera

Doi

Niewodowski

et al. 2021

Preprint

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Patients with pulmonary arterial hypertension (PAH) may develop large pulmonary artery aneurysms (PAA) which may be complicated by rupture, dissection or intravascular thrombus formation. These patients were traditionally considered for heart-lung transplantation but more recently, there have been reports of successful lung transplantation with reconstruction of the pulmonary artery (PA). We present two patients who underwent successful transplantation for PAH with giant PAA. One patient had end stage PAH and right pulmonary artery atresia complicated by a giant main PAA. This patient underwent bilateral sequential lung transplantation with concurrent pulmonary artery reconstruction. Another patient had end stage PAH with giant PAA on a background of D-transposition of the great arteries who had a Mustard repair at 9 months of age. This patient underwent heart-lung transplantation. Both heart-lung transplantation and lung transplantation with reconstruction of the pulmonary should be considered as a treatment option for patients with PAH with PAA.

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“…The etiology of the disease is complex and multifactorial, and it preferentially affects women, exerting substantial impact on their quality of life, i.e., reduced functional ability, greater oxygen requirements, and an increased risk of mortality [2]. Despite the advances in the knowledge of PAH, there is a need to improve its prevention and treatment [2,3]. Several non-invasive predictors are used to evaluate the prognosis of pulmonary hypertension (PH), i.e., New York Heart Association (NYHA) functional class, reduced 6-min walk test (6MWT), diffusing capacity for carbon monoxide (DLCO), and B-type natriuretic peptide (BNP) and the N-terminal fragment of pro-BNP (NT-pro-BNP) levels [4].…”

Section: Introductionmentioning

confidence: 99%

Total, Bioavailable, and Free Vitamin D Levels and Their Prognostic Value in Pulmonary Arterial Hypertension

Callejo

Mondéjar-Parreño

Esquivel-Ruiz

et al. 2020

JCM

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Introduction: Epidemiological studies suggest a relationship between vitamin D deficiency and cardiovascular and respiratory diseases. However, whether total, bioavailable, and/or free vitamin D levels have a prognostic role in pulmonary arterial hypertension (PAH) is unknown. We aimed to determine total, bioavailable, and free 25-hydroxy-vitamin D (25(OH)vitD) plasma levels and their prognostic value in PAH patients. Methods: In total, 67 samples of plasma from Spanish patients with idiopathic, heritable, or drug-induced PAH were obtained from the Spanish PH Biobank and compared to a cohort of 100 healthy subjects. Clinical parameters were obtained from the Spanish Registry of PAH (REHAP). Results: Seventy percent of PAH patients had severe vitamin D deficiency (total 25(OH)vitD < 10 ng/mL) and secondary hyperparathyroidism. PAH patients with total 25(OH)vitD plasma above the median of this cohort (7.17 ng/mL) had better functional class and higher 6-min walking distance and TAPSE (tricuspid annular plane systolic excursion). The main outcome measure of survival was significantly increased in these patients (age-adjusted hazard ratio: 5.40 (95% confidence interval: 2.88 to 10.12)). Vitamin D-binding protein (DBP) and albumin plasma levels were downregulated in PAH. Bioavailable 25(OH)vitD was decreased in PAH patients compared to the control cohort. Lower levels of bioavailable 25(OH)vitD (<0.91 ng/mL) were associated with more advanced functional class, lower exercise capacity, and higher risk of mortality. Free 25(OH)vitD did not change in PAH; however, lower free 25(OH)vitD (<1.53 pg/mL) values were also associated with high risk of mortality. Conclusions: Vitamin D deficiency is highly prevalent in PAH, and low levels of total 25(OH)vitD were associated with poor prognosis.

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Improving Survival in Patients with Pulmonary Arterial Hypertension: Focus on Intravenous Epoprostenol

Cited by 10 publications

References 55 publications

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

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

Surgical Management of Giant Pulmonary Artery Aneurysm in Patients with Severe Pulmonary Arterial Hypertension

Total, Bioavailable, and Free Vitamin D Levels and Their Prognostic Value in Pulmonary Arterial Hypertension

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