Pulmonary Arterial Hypertension (PAH), a fatal orphan disease, is defined as increased mean Pulmonary Arterial Pressure (mPAP) above 25 mm/Hg, Pulmonary Capillary Wedge Pressure (PCWP) below 15 mm/Hg, and pulmonary vascular Resistance over 3 Wood Unit (WU). Although Pulmonary Hypertension (PH) is a relatively prevalent disease, the idiopathic form (iPAH) is a rare disease with a prevalence of 1 to 3 in million population.
The Iranian Registry Program was introduced in 2009 by using its website, www.ipah.ir, for delivery of a standard and national diagnostic protocol to PH patients and also gather patient data in order to provide effective and updated management services to all patients. In this program, the patient data was collected based on guidelines of European Respiratory Society (ERS), European Society of Cardiology (ESC), International Society of Heart and Lung Transplantation (ISHLT), and American College of Cardiology Foundation (ACCF). Moreover, follow up of all patients was possible through the website.
353 PH patients (225 female, 125 male) referred to Masih Daneshvari Hospital Tehran, Iran between 2009-19 and were registered. Among them 182 patients were characterized as iPAH (n=121 female, n= 61 male). The average age of PH patients was 48.6±16 years for suffering PH and 48.2±15 years for primary idiopathic form. Sparing idiopathic subtype, the aetiology of most PAH patients was Eisen-menger syndrome (43 patients, 12.2%), collagen vascular (22 patients, 6.2%), and chronic thromboembolic pulmonary hypertension (95 patients, 26.9%). Evaluation of functional class based on New York Heart Associated Functional Class (NYHA) showed 15 patients with FC=I (4.6%), 177 patients with FC=II (50.7%), 142 patients with FC=III (40.7%) and 14 patients with FC=IV (4%). Treatment strategy was Tadafil in 209 patients, Bosentan in 230 and Ilomedin in 65. Due to the economic sanctions in the country, we had limited access to standard medications based on guidelines.
Diagnosis, management and follow up of PH and certainly iPAH patients under Iranian PAH Registry System (website www.ipah.ir) during 10 years, leads to organized patient care system and management and also provides invaluable data.
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