Grup 3; Akciğer Hastalıkları ve Hipoksiye Bağlı PH
Özet
Pulmoner hipertansiyon, kronik akciğer hastalıkları ve hipoksi ile ilişkili solunum bozukluklarının önemli komplikasyonlarından biri olup egzersiz kapasitesinde azalma, yaşam kalitesinde bozulma ve mortalite artışı ile ilişkilidir. Hastalığın gelişiminde kronik alveoler hipoksiye bağlı pulmoner vazokonstriksiyon, pulmoner vasküler yeniden yapılanma, endotel disfonksiyonu ve inflamasyon temel rol oynamaktadır. Kronik obstrüktif akciğer hastalığında pulmoner hipertansiyon genellikle hafif seyretmekle birlikte bazı hastalarda belirgin pulmoner vasküler tutulum gelişebilmektedir. İnterstisyel akciğer hastalıkları ve özellikle kombine pulmoner fibrozis amfizem sendromunda ise pulmoner hipertansiyon daha sık görülmekte ve prognozu olumsuz etkilemektedir. Obstrüktif uyku apne sendromu ve hipoventilasyon sendromlarında tekrarlayan hipoksemi, alveoler hipoventilasyon ve kardiyovasküler etkilenme pulmoner vasküler yükün artmasına katkıda bulunmaktadır. Tedavinin temelini altta yatan akciğer hastalığının, hipokseminin ve eşlik eden solunum bozukluklarının optimal yönetimi oluşturmaktadır. Bu bölümde akciğer hastalıkları ve hipoksiye bağlı pulmoner hipertansiyonun patofizyolojik mekanizmaları, başlıca klinik tablolarla ilişkisi, güncel tanısal yaklaşımı ve tedavi prensipleri gözden geçirilmiştir.
Pulmonary hypertension is one of the major complications of chronic lung diseases and hypoxia-related respiratory disorders, and is associated with reduced exercise capacity, impaired quality of life, and increased mortality. Pulmonary vasoconstriction due to chronic alveolar hypoxia, pulmonary vascular remodeling, endothelial dysfunction, and inflammation play central roles in the development of the disease. In chronic obstructive pulmonary disease, pulmonary hypertension generally follows a mild course, although marked pulmonary vascular involvement may develop in some patients. In interstitial lung diseases—and particularly in combined pulmonary fibrosis and emphysema syndrome—pulmonary hypertension is more common and adversely affects prognosis. In obstructive sleep apnea syndrome and hypoventilation syndromes, recurrent hypoxemia, alveolar hypoventilation, and cardiovascular involvement contribute to the increased pulmonary vascular burden. The mainstay of treatment is optimal management of the underlying lung disease, hypoxemia, and accompanying respiratory disorders. This chapter reviews the pathophysiological mechanisms of pulmonary hypertension due to lung diseases and hypoxia, its association with the main clinical conditions, and current diagnostic approaches and treatment principles.
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