Pulmoner Hipertansiyonun Tarihsel Gelişimi ve Güncel Tanımı
Özet
Pulmoner hipertansiyon, istirahatte ölçülen ortalama pulmoner arter basıncının patolojik düzeyde yükselmesiyle tanımlanan, ilerleyici ve yaşamı tehdit eden hemodinamik bir bozukluktur. Bu bölüm, hastalığın ondokuzuncu yüzyıl sonundaki ilk otopsi temelli tanımlarından günümüzün moleküler düzeyde hedeflenebilen tablosuna uzanan bir asırlık gelişimini ele almaktadır. Von Romberg ve Brenner’in patolojik gözlemleri, Euler-Liljestrand’ın hipoksik pulmoner vazokonstrüksiyon keşfi ve Forssmann, Cournand ile Richards’ın geliştirdiği sağ kalp kateterizasyonu, hastalığın yaşayan hastada nesnel olarak tanımlanmasını olanaklı kılan dönüm noktalarıdır. Dresdale’in “primer pulmoner hipertansiyon” kavramı, asetilkolinle yürütülen erken vazoreaktivite çalışmaları ve aminoreks ile fenfluramin salgınları, hastalığın klinik ve epidemiyolojik anlayışını biçimlendiren başlıca aşamalardır. Bölüm ayrıca Dünya Sağlık Örgütü toplantıları (Cenevre 1973), Evian (1998) ve Venedik (2003) sempozyumlarıyla evrilen uluslararası sınıflandırmayı; ortalama pulmoner arter basıncı eşiğinin 25 mmHg’den 20 mmHg’ye indirildiği ve pulmoner vasküler direncin tanıma dâhil edildiği güncel ESC/ERS 2022 hemodinamik tanımını; pre-kapiller ile post-kapiller ayrımını ve tedavinin destekleyici yaklaşımdan üç temel yolağı (prostasiklin, endotelin ve nitrik oksit) hedefleyen çağdaş kombinasyon tedavilerine evrimini özetlemektedir.
Pulmonary hypertension is a progressive and life-threatening hemodynamic disorder defined by a pathological elevation of the mean pulmonary arterial pressure measured at rest. This chapter examines the disease's century-long development, from its first autopsy-based descriptions in the late nineteenth century to today's clinical picture, which can be targeted at the molecular level. The pathological observations of von Romberg and Brenner, Euler and Liljestrand's discovery of hypoxic pulmonary vasoconstriction, and the right heart catheterization developed by Forssmann, Cournand, and Richards represent the turning points that made it possible to objectively define the disease in the living patient. Dresdale's concept of "primary pulmonary hypertension," the early vasoreactivity studies conducted with acetylcholine, and the aminorex and fenfluramine epidemics are among the principal stages that shaped the clinical and epidemiological understanding of the disease. The chapter also summarizes the international classification that evolved through the World Health Organization meetings (Geneva 1973) and the Evian (1998) and Venice (2003) symposia; the current ESC/ERS 2022 hemodynamic definition, in which the mean pulmonary arterial pressure threshold was lowered from 25 mmHg to 20 mmHg and pulmonary vascular resistance was incorporated into the definition; the distinction between pre-capillary and post-capillary forms; and the evolution of treatment from a supportive approach to contemporary combination therapies targeting the three principal pathways (prostacyclin, endothelin, and nitric oxide).
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