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.