Grup 1; Pulmoner Arteriyel Hipertansiyon Alt Tipler
Özet
Pulmoner arteriyel hipertansiyon (PAH), prekapiller pulmoner hipertansiyonun en iyi tanımlanmış ve en ayrıntılı araştırılmış alt grubudur. Sağ kalp kateterizasyonunda ortalama pulmoner arter basıncının >20 mmHg, pulmoner arter kama basıncının ≤15 mmHg ve pulmoner vasküler direncin >2 Wood Ünitesi olması ile tanımlanır. Güncel klinik sınıflamada Grup 1 PAH; idiyopatik, herediter, ilaç ve toksinlerle ilişkili, bağ dokusu hastalıkları, HIV enfeksiyonu, portal hipertansiyon, konjenital kalp hastalıkları ve şistozomiyazis ile ilişkili PAH, ayrıca pulmoner veno-oklüzif hastalık/pulmoner kapiller hemanjiyomatozis (PVOD/PCH) özellikleri gösteren PAH ve yenidoğanın persistan pulmoner hipertansiyonu gibi alt grupları içermektedir. Bu bölümde Grup 1 PAH'ın güncel sınıflaması, epidemiyolojisi, genetik özellikleri, patofizyolojik mekanizmaları ve klinik alt tipleri, son uluslararası kılavuzlar ve literatür doğrultusunda özetlenmiştir. Ayrıca herediter PAH'da genetik testlerin önemi, ilaç ilişkili PAH'ın güncel sınıflaması ve ilişkili PAH alt gruplarının klinik özellikleri ele alınmıştır. Grup 1 PAH'ın doğru sınıflandırılması, uygun tedavi stratejisinin belirlenmesi, prognozun öngörülmesi ve kişiselleştirilmiş hasta yönetimi açısından temel öneme sahiptir.
Pulmonary arterial hypertension (PAH) is the best-defined and most extensively studied form of precapillary pulmonary hypertension. It is hemodynamically characterized by a mean pulmonary arterial pressure >20 mmHg, pulmonary arterial wedge pressure ≤15 mmHg, and pulmonary vascular resistance >2 Wood units measured by right heart catheterization. According to the current clinical classification, Group 1 PAH includes idiopathic PAH, heritable PAH, drug- and toxin-associated PAH, PAH associated with connective tissue diseases, HIV infection, portal hypertension, congenital heart disease, schistosomiasis, PAH with pulmonary veno-occlusive disease/pulmonary capillary hemangiomatosis (PVOD/PCH) features, and persistent pulmonary hypertension of the newborn. This chapter reviews the current classification, epidemiology, genetic background, pathophysiological mechanisms, and clinical characteristics of Group 1 PAH based on recent international guidelines and contemporary evidence. Special emphasis is placed on genetic testing in heritable PAH, the updated classification of drug-induced PAH, and the clinical features of associated PAH subgroups. Accurate classification of Group 1 PAH is essential for appropriate therapeutic decision-making, prognostic assessment, and individualized patient management.
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