ASİYE KANBAY, Hakan BÜYÜKDOĞAN, F.Sema OYMAK, Ramazan DEMİR

Pulmoner hipertansiyon ve yeni sınıflama

Pulmoner hipertansiyon klinik sınıflamasının amacı tedavi yaklaşımında klinik prezentasyonda ve patofizyolojik mekanizmalarda benzerlikleri olan farklı hastalık bulgu-larını birlikte gruplamaktır. 1998 yılında 2. Dünya Sem-pozyumu sırasında tanımlanan Pulmoner Hipertansiyon klinik sınıflaması 2003 yılında 3. dünya Pulmoner Hiper-tansiyon Sempozyumu sırasında modifiye edilmiştir. 2008 yılında yapılan 4. Dünya Sempozyumu sırasında da önceki klinik sınıflamaların genel yapısının korunmasına karar verildi. Bu toplantı sırasında yapılan temel değişik-likler grup 1 hipertansiyon ile ilgili alt grupta yapılmıştır. Bu alt grup aile öyküsü ile birlikte olan pulmoner hiper-tansiyonu veya genetik mutasyonla birlikte idyopatik pulmoner hipertansiyonu olan hastaları kapsamaktadır (Bone morfojenik protein reseptör-2, aktivin reseptör like kinaz tip 1 ve endoglin). Yeni sınıflamada şiştozo-miazis ve kronik hemolitik anemi belirli hastalıkla ilişkili pulmoner arter hipertansiyon alt grubunda ayrı bir grup olarak tanımlanmıştır. Son olarak, pulmoner venooklusif hastalık ve pulmoner kapiller hemanjiyomatozis grup1’e çok yakın ancak farklı bir grup olarak tanımlanmıştır. Bu derlemede, yeni sınıflama özetlenecektir.

New classification of pulmonary hypertension

The purpose of a clinical classification of pulmonary hypertension is to group together different manifestations of disease sharing similarities in pathophysiologic mechanisms, clinical presentation, and therapeutic approaches which might facilitate clinical management. In 2003, during the 3rd World Symposium on Pulmonary Hypertension, the clinical classification of PH the 2nd World Symposium on Pulmonary Hypertension was modified. During the 4th World Symposium held in 2008, it was decided to maintain the general architecture and philosophy of the previous clinical classifications by board members. The last modifications principally concern Group 1, pulmonary arterial hypertension. This subgroup includes patients with pulmonary arterial hypertension with a family history or patients with idiopathic pulmonary arterial hypertension with mutations (e.g., bone morphogenetic protein receptor-2, activin receptor-like kinase type 1, and endoglin). In the last classification, schistosomiasis and chronic hemolytic anemia appear as divided entities in the subgroup of pulmonary arterial hypertension associated with identified diseases. Finally, it was also decided to place pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis in other group, distinct but very close to Group 1 (now called Group 1'). Thus, Group 1 of pulmonary arterial hypertension is now more homogeneous. The aim of this review is to summarize the last pulmonary hypertension classification shortly.

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