Grup 4; Kronik Tromboembolik Pulmoner Hipertansiyon
Özet
Kronik tromboembolik pulmoner hipertansiyon (KTEPH), akut pulmoner tromboemboli sonrası gelişen tedavi edilebilir bir hastalıktır ve PH sınıflandırmasında Grup 4’te yer almaktadır. Akut pulmoner emboli geçiren hastalarda KTEPH gelişme sıklığı %0,01-0,05 arasında olup, takip edilen hastalarda bu oran artabilir. KTEPH’li hastalarda damar yapısında değişiklikler ve mikrovaskülopatinin PH’nin devamında etkili olduğu düşünülmektedir. PH bulguları olan hastalarda, KTEPH açısından risk faktörleri, ekokardiyografi ve görüntüleme ile değerlendirilmelidir. Perfüzyon sintigrafisi ve sağ kalp kateterizasyonu temel tanı araçlarıdır; uyumsuz perfüzyon defekti ve radyolojik bulgular ile doğrulanır. Tedavi edilmezse ilerleyici olup, yüksek pulmoner arter basıncı geliştiğinde sağ ventrikül yetmezliği ve ölüm riski artar. Cerrahi, medikal ve balon anjiyoplasti gibi tedavi seçenekleri ile prognoz önemli ölçüde iyileşmiştir. Yüksek risk faktörleri (yüksek pulmoner vasküler direnç, yüksek sağ atriyum basıncı) ve fonksiyonel durum uzun dönem sağkalımı belirler. Tüm KTEPH hastaları, multidisipliner merkezlerde ömür boyu izlenmeli; antikoagülasyon, semptom ve fonksiyonel kapasite takibi yapılmalıdır. İzlemde, hemodinamik ve klinik parametreler dinamik olarak değerlendirilerek, tedavi ve cerrahi kararları güncellenmelidir.
Chronic thromboembolic pulmonary hypertension (CTEPH) is a treatable disease that develops after acute pulmonary thromboembolism and is classified as Group 4 in the pulmonary hypertension (PH) classification. The incidence of CTEPH after acute pulmonary embolism ranges from 0.01% to 0.05%, although this rate may be higher among patients who are followed over time. In patients with CTEPH, vascular remodeling and microvasculopathy are thought to contribute to the persistence of PH. Patients with signs of PH should be evaluated for CTEPH risk factors using echocardiography and imaging studies. Ventilation–perfusion scintigraphy and right heart catheterization are the main diagnostic tools; the diagnosis is confirmed by the presence of mismatched perfusion defects and characteristic radiological findings. If left untreated, the disease is progressive, and the development of elevated pulmonary artery pressure increases the risk of right ventricular failure and death. Treatment options such as surgery, medical therapy, and balloon pulmonary angioplasty have significantly improved the prognosis. High-risk factors (such as elevated pulmonary vascular resistance and increased right atrial pressure) and functional status are key determinants of long-term survival. All patients with CTEPH should be followed lifelong at multidisciplinary centers, with ongoing anticoagulation therapy and monitoring of symptoms and functional capacity. During follow-up, hemodynamic and clinical parameters should be assessed dynamically, and treatment and surgical decisions should be updated accordingly.
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