Objective: Pulmonary arterial hypertension (PAH) is a term used to define a variety of progressive conditions that have in common, increased pulmonary vascular resistance leading to right heart failure and death. There has been considerable decrease in mortality and morbidity with the advances in PAH treatment over the past decade. However, since there is no epidemiologic study in Turkey, the prevalence of PAH and its importance is not known yet. This study aimed to evaluate the diagnostic clinical experience of Ege University Medical School Cardiology Department with PAH patients.
Methods: We evaluated the diagnostic approach to patients referred to our department with the diagnosis of PAH since 2000 by retrospective analysis method.
Results: The diagnosis of pulmonary hypertension was definite in 70 patients (mean age 47±16 years, 61% women). Etiology from most prevalent to least was as following: congenital heart diseases (27%), chronic thromboembolic pulmonary hypertension (24%), connective tissue diseases-scleroderma (14%), idiopathic PAH (8%), diastolic dysfunction (3%), pulmonary disease (3%), pulmonary veno-occlusive disease (2%), hepatopulmonary hypertension (1%), and HIV-infection associated PAH (1%). At diagnosis, 68% of patients were in NYHA functional class-III or IV. Six-minute walk test was 263±127m. Mean pulmonary artery pressure was 65±20 mmHg. The prognostic marker pro-BNP (brain natriuretic peptid) level was 3208±4145 pg/ml.
Conclusion: Our practice shows that PAH is diagnosed late in the course of the disease in Turkey. This can be overcome with structured management in designated centers with multidisciplinary team-working in a shared care approach. There is also an urgent need for an epidemiological registry in order to determine the burden of PAH in Turkey and increase the awareness of doctors.