Grup 5; Multipl/Belirsiz Mekanizmalara Bağlı Pulmoner Hipertansiyon
Özet
Grup 5 Pulmoner Hipertansiyon (PH); hematolojik, sistemik, metabolik ve kronik böbrek yetmezliği gibi net olarak sınıflandırılamayan, patofizyolojisi belirsiz ve multifaktöriyel mekanizmalara dayalı heterojen bir hastalık grubunu kapsar. Bu grupta, altta yatan primer patolojilere bağlı olarak pre-kapiler, post-kapiler veya kombine vasküler tutulumlar bir arada görülebilir. Tedavide konvansiyonel pulmoner hipertansiyon (PH) ilaçlarının rutin kullanımı önerilmemekte olup, temel klinik strateji doğrudan altta yatan primer hastalığın yönetilmesine (enzim replasmanı, diyaliz optimizasyonu veya mekanik müdahaleler) dayanmaktadır.
Group 5 Pulmonary Hypertension (PH) encompasses a heterogeneous group of disorders with unclear pathophysiology and multifactorial mechanisms that cannot be distinctly classified, including hematologic, systemic, metabolic, and chronic kidney diseases. In this group, pre-capillary, post-capillary, or combined vascular involvement may coexist depending on the underlying primary pathologies. The routine use of conventional pulmonary hypertension (PH) medications is not recommended in this cohort; instead, the primary clinical strategy relies directly on managing the underlying primary disease through targeted interventions such as enzyme replacement therapy, dialysis optimization, or mechanical procedures.
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