Kronik Tromboembolik Pulmoner Hipertansiyon Tedavisinde Güncel Yaklaşımlar
Özet
Kronik tromboembolik pulmoner hipertansiyon (KTEPH), akut pulmoner emboli sonrası organize tromboembolik materyalin pulmoner arterlerde kalıcı obstrüksiyona yol açması ve buna eşlik eden küçük damar hastalığı sonucu gelişen, tedavi edilebilir bir pulmoner hipertansiyon alt grubudur. Tedavi edilmediğinde ilerleyici sağ ventrikül yetmezliği, egzersiz kapasitesinde azalma ve mortalite ile ilişkilidir. Günümüzde tedavi yaklaşımı, deneyimli merkezlerde multidisipliner KTEPH ekibi tarafından gerçekleştirilen bireyselleştirilmiş değerlendirmeye dayanmaktadır. Pulmoner endarterektomi, cerrahi olarak ulaşılabilir lezyonlara sahip uygun hastalarda kür sağlayabilen altın standart tedavi yöntemidir. Cerrahiye uygun olmayan olgularda veya cerrahi sonrası rezidüel ya da rekürren pulmoner hipertansiyon gelişen hastalarda balon pulmoner anjiyoplasti önemli bir girişimsel seçenektir. Medikal tedavi ise inoperabl hastalarda, rezidüel pulmoner hipertansiyonda veya girişimsel tedavilerle kombine edildiğinde semptom kontrolü, egzersiz kapasitesi ve hemodinamik parametrelerde anlamlı iyileşme sağlar. Çözünür guanilat siklaz uyarıcısı riociguat, bu alanda etkinliği en güçlü kanıtlarla desteklenen tedavi seçeneğidir. Bu bölümde kronik tromboembolik pulmoner hipertansiyonun güncel tedavi yaklaşımı; pulmoner endarterektomi, balon pulmoner anjiyoplasti ve medikal tedavi seçenekleri güncel kanıtlar ve uluslararası kılavuzlar doğrultusunda klinik uygulamaya yönelik bir bakış açısıyla ele alınmıştır.
Chronic thromboembolic pulmonary hypertension (CTEPH) is a treatable subgroup of pulmonary hypertension that develops when organized thromboembolic material causes persistent obstruction of the pulmonary arteries following acute pulmonary embolism, accompanied by concomitant small-vessel disease. If left untreated, it is associated with progressive right ventricular failure, reduced exercise capacity, and increased mortality. Contemporary management is based on an individualized assessment carried out by a multidisciplinary CTEPH team at experienced centers. Pulmonary endarterectomy is the gold-standard treatment and may be curative in eligible patients with surgically accessible lesions. In patients who are not suitable for surgery, or who develop residual or recurrent pulmonary hypertension after surgery, balloon pulmonary angioplasty has emerged as an important interventional option. Medical therapy provides significant improvements in symptom control, exercise capacity, and hemodynamic parameters, particularly in inoperable patients, in residual pulmonary hypertension, or when combined with interventional treatments. Riociguat, a soluble guanylate cyclase stimulator, is the treatment option supported by the strongest evidence of efficacy. In addition, agents targeting the prostacyclin pathway and other pulmonary arterial hypertension therapies may be used in selected patient groups. This chapter summarizes the current treatment approach to chronic thromboembolic pulmonary hypertension—pulmonary endarterectomy, balloon pulmonary angioplasty, and medical treatment options—in light of current evidence and international guidelines, from a perspective oriented toward clinical practice.
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