Bilge YIlMAZ KARA, Benan MüSEllİM, Gül ÖNGEN

Pulmoner Arteriyel Hipertansiyon Olgularının Özellikleri, Tedavi Yanıtına ve Sağkalıma Etkili Faktörlerin Değerlendirilmesi

Amaç:Çalışmamızda pulmoner arteriyel hipertansiyon (PAH) tanılı hastaların klinik özelliklerinin, teda-vi etkinliğinin ve sağkalıma etkili faktörlerin saptanmasını amaçladık.Yöntem: 2005-2012 yılları arasında PAH tanısıyla takip edilen hastaların verileri retrospektif olarak incelendi. Hastalar; bağ dokusu hastalığına bağlı PAH (BDH), idiyopatik PAH (İPAH) ve doğumsal kalp hastalığına bağlı PAH (DKH) olarak gruplandırıldı.On sekiz yaşından küçük olanlar, ESC/ERS kılavuzu klinik sınıflamasına göre grup 1 dışındakiler ve malignitesi olanlar çalışmadan dışlandı. Demografik veriler, tedavide kullanılan ilaçlar, başlangıç ve takiplerdeki Dünya Sağlık Örgütü Fonksiyonel Sınıflaması (DSÖ-FS), 6 dakika yürüme mesafesi (6DYM) ve hemodinamik parametreleri hasta dosyalarından elde edildi. Takipte primer sonlanım noktası ölüm olarak belirlendi. Bulgular:Çalışmaya toplam 43 hasta (K/E:35/8, medyan (çeyrekler arası genişlik) yaş: 57 (16) yıl) alındı. Etyolojide 22 hastada BDH, 15 hastada İPAH, 6 hastada KKH saptandı. Medyan (çeyrekler arası genişlik) 23 (25,7) ay takip süresinde 20 (%46,5) hasta öldü. PAH etyoloji grupları arasında mortalite açısından fark görülmedi. Sağkalım süreleri başlangıç tedavisi olarak bosentan alanlarda inhaler iloprost alanlara göre daha uzun (ortalama 53,9±6,4 ay’a karşılık 18,8±4,9 ay, p= 0,001), başlangıç DSÖ-FS evresi ileri olanlarda ise daha kısa saptandı (DSÖ-FS II ve IV için sırasıyla ortalama 40,7±4,3 ay’a karşı-lık 13,4±4 ay, p

Characteristics of Pulmonary Arterial Hypertension Patients and Evaluation of Factors Affecting Treatment Response and Survival

Method:The data of patients followed up with PAH between 2005 and 2012 were reviewed retros-pectively. The patients were grouped as PAH associated with connective tissue disease (CTD), idiopat-hic PAH (IPAH) and PAH due to congenital heart disease (CHD). Patients younger than 18 years of age, patients who belong to other pulmonary hypertension groups other than group 1, and those with malignancy were excluded. Demographic data, drugs used in treatment, functional class according to World Health Organization Functional Classification (WHO-FS), 6-minute walking distance (6MWD) and hemodynamic parameters at baseline and follow-up were obtained from the patient files. The primary endpoint of the follow-up was determined as death.Results:Totally 43 PAH patients (F/M:35/8, median (IQR: Interquartile range): 57 (16) years) were enrolled. In the etiology 22 patients had CTD, 15 patients had IPAH, and 6 patients had CHD. During the median (IQR) follow-up period of 23 (25,7) months, 20 (46.5%) patients died. The mortality rates of etiological groups were similar. Compared to iloprost, bosentan group had lower stage of WHO-FC (p

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