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Pulmoner arteriyel hipertansiyon: tanı ve tedavisi

Pulmonary arterial hypertension: diagnosis and treatment

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Abstract (2. Language): 
Pulmonary arterial hypertension (PAH) is a rare disease of unknown etiology. Signs and symptoms of pulmonary arterial hypertension are often subtle and nonspecific. The diagnosis should be suspected in patients with increasing dyspnea on exertion and a known cause of pulmonary hypertension. Two-dimensional echocardiography with Doppler flow studies is the most useful imaging modality in patients with suspected pulmonary hypertension. If pulmonary hypertension is present, further evaluation may include assessment of oxygenation, pulmonary function testing, high-resolution computed tomography of the chest, ventilation-perfusion lung scanning and cardiac catheterization. Treatment with standart or combination therapy improve exercise capacity, quality of life, hemodynamics and long-term survival in patients with PAH. New agents are also under examination with affirmative results. Lung transplantation remains an option for selected patients with pulmonary arterial hypertension that does not respond to medical management.
Abstract (Original Language): 
Pulmoner arteriyel hipertansiyon (PAH), altta yatan, sebebi bilinmeyen nadir bir hastalıktır. Pulmoner arteriyel hipertansiyon bulguları belirgin olmadığından sessiz seyreder. Ancak eforla artan dispne şikayeti olan olgularda şüphe edildiğinde ve nedeni bilinen PH olgularında tanı konulabilmektedir. PH şüphe edilen olgularda, iki boyutlu Doppler ekokardiyografi en yararlı tanı yöntemlerinden biridir. PH varlığında ise oksijenizasyon, akciğer fonksiyon testleri, yüksek rezolüsyonlu bilgisayarlı tomografi, ventilasyon-perfüzyon sintigrafisi ve kardiyak kateterizasyon gibi ileri tetkiklerin yapılması gerekir. Standart veya kombine tedavilerle egzersiz kapasitesinde, yaşam kalitesinde, hemodinamide ve yaşam süresinde iyileşme saptanır. Yeni tedavi yöntemleri ve ajanlarla PAH tedavisinde yüz güldürücü sonuçlar elde edilmektedir. Akciğer transplantasyonu, tıbbi tedaviye cevap vermeyen pulmoner arteriyel hipertansiyonu olan seçilmiş hastalarda bir umut olarak durmaktadır.
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