PH experience in an expert university hospital

Abstract

Objective:Pulmonary artery hypertension (PAH) is characterized by remodeling of the small pulmonary arteries, leading to a progressive increase in pulmonary vascular resistance and right ventricular failure. In this study, we aimed to share our 10 years of experience dealing with pulmonary hypertension (PH) and provide information in real-life settings in terms of demographics, clinical course, PH subgroup distribution, and treatment patterns in patients with PAH in a tertiary center.Methods:In this retrospective, single-center, observational study, we screened the patients who applied to PH outpatient clinic of İstanbul University Institute of Cardiology due to the suspicion of PAH between 2008 and 2017. While group 1, 4, and 5 PH patients were included, group 2 and 3 PH patients were excluded from the study.Results:Our study group comprised 162 patients (115 females, 71%). The female: male ratio was 2.4. The mean age was 52±16 years. Most (86.4%) of the patients were in group 1 PH (PAH). The rest (13.6%, n=22) of the patients were in group 4 PH (chronic thromboembolic PH). In group 1 PH, 45.7% of patients (n=64) were classified as having idiopathic PAH (IPAH) after excluding the alternative diagnosis using PH diagnostic algorithm. The remaining 54.3% of group 1 PH patients (n=76) had various diseases that caused PAH, which is called associated PAH (APAH); APAH group included PAH associated with congenital heart diseases (n=70), connective tissue disorders (scleroderma, n=4) and portal hypertension (n=2).Conclusion:Our data provides important information in real-life settings in terms of demographics, clinical course, PH subgroup distribution, and treatment patterns in patients with PAH in a reference tertiary center in Turkey.

Prescription patterns of ambrisentan in some cities of Colombia

“… 13 , 14 , 15 , 17 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 Arterial hypertension was the most prevalent comorbidity in this report, which is consistent with the literature. 17 , 20 , 26 , 27 , 28 , 29 , 30 , 31 Pulmonary arterial hypertension (Group 1) was the most prevalent, in line with other studies with real‐world evidence, 20 , 22 , 23 , 25 and most of the patients had WHO Functional Class I; this differs from other reports in which the patients presented a more deteriorated functional class, 15 , 20 , 22 , 23 , 25 , 29 but the proportion of cases with home oxygen use was similar (33.3% vs. 21.6–30.2%). 24 , 28 …”

“…The median age of the patients in this study was similar to that found in some studies in Asia (47.0–50.8 years) 20 , 21 and Europe (49.6–52.0 years), 22 , 23 , 24 but in other reports from Europe (61.0–61.6 years), 13 , 14 , 25 North America (62.7–68.1 years) 17 , 26 , 27 , 28 and Oceania (60.0 years), 29 patients were older, 13 , 14 , 17 , 25 , 26 , 27 , 28 , 29 and there was a predominance of women (82.1% vs. 61.0–79.0%). 13 , 14 , 15 , 17 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 Arterial hypertension was the most prevalent comorbidity in this report, which is consistent with the literature. 17 , 20 , 26 , 27 , 28 , 29 , 30 , 31 Pulmonary arterial hypertension (Group 1) was the most prevalent, in line with other studies with real‐world evidence, 20 , 22 , 23 , 25 and most of the patients had WHO Functional Class I; this diffe...…”

A focus on riociguat in the treatment of pulmonary arterial hypertension

“…In Europe, the incidence and prevalence of PAH have been estimated to 5-10/million/y and 15-60/million. 16 Given that PAH often progresses to WHO-FC II or III before diagnosis, many cases may not be registered, and the prevalence might be higher than reported. Riociguat is a promising new agent in a novel therapeutic class called sGC stimulators, which affect the NO pathway to stimulate vasodilation.…”

“…In Europe, the incidence and prevalence of PAH have been estimated to 5‐10/million/y and 15‐60/million . Given that PAH often progresses to WHO‐FC II or III before diagnosis, many cases may not be registered, and the prevalence might be higher than reported.…”

Complement alternative pathway activation associated with pulmonary hypertension in lupus nephritis patients

“…3 It is characterized by remodeling of the small pulmonary arteries, leading to a progressive increase in pulmonary vascular resistance and right ventricular failure. 4 Right heart catheterization studies have not been conducted in SLE patients and the prevalence of PH mainly depends on echocardiography-based cohorts. 5,6 The mechanisms involved in the pathogenesis of PH are complex.…”