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Journals / Çukurova Anestezi ve Cerrahi Bilimler Dergisi / 2026 / Cilt: 9 Sayı: 3

Hemodynamic Reclassification Under the 2022 Definition in Connective Tissue Disease-Associated Pulmonary Arterial Hypertension: A Single-Center Cohort Study

Bağ Dokusu Hastalıklarına Bağlı Pulmoner Arteriyel Hipertansiyonda 2022 Tanımı Kapsamında Hemodinamik Yeniden Sınıflandırma: Tek Merkezli Kohort Çalışması

Burak Okyar(SAĞLIK BİLİMLERİ ÜNİVERSİTESİ, ADANA ŞEHİR SAĞLIK UYGULAMA VE ARAŞTIRMA MERKEZİ, DAHİLİ TIP BİLİMLERİ BÖLÜMÜ)0000-0002-9028-9930
Mert Evlıce(SAĞLIK BİLİMLERİ ÜNİVERSİTESİ, ADANA ŞEHİR SAĞLIK UYGULAMA VE ARAŞTIRMA MERKEZİ)0000-0003-4733-6348
Emre Paçacı(Osmaniye City Training and Research Hospital, Department of Cardiology)0000-0001-6256-3771
Bekir Akmaz(SAĞLIK BİLİMLERİ ÜNİVERSİTESİ, ADANA ŞEHİR SAĞLIK UYGULAMA VE ARAŞTIRMA MERKEZİ)0009-0001-4888-623X
İlhan Kaplan(SAĞLIK BİLİMLERİ ÜNİVERSİTESİ, ADANA ŞEHİR SAĞLIK UYGULAMA VE ARAŞTIRMA MERKEZİ)0009-0004-3756-3699
Halil Coşkun(SAĞLIK BİLİMLERİ ÜNİVERSİTESİ, ADANA ŞEHİR SAĞLIK UYGULAMA VE ARAŞTIRMA MERKEZİ)0009-0007-4450-2183
Mükremin Coşkun(SAĞLIK BİLİMLERİ ÜNİVERSİTESİ, ADANA ŞEHİR SAĞLIK UYGULAMA VE ARAŞTIRMA MERKEZİ)0000-0003-2061-8337
Alper Yıldırım(SAĞLIK BİLİMLERİ ÜNİVERSİTESİ, ADANA ŞEHİR SAĞLIK UYGULAMA VE ARAŞTIRMA MERKEZİ)0000-0003-3160-458X
Emine Duygu Ersözlü(SAĞLIK BİLİMLERİ ÜNİVERSİTESİ, ADANA ŞEHİR SAĞLIK UYGULAMA VE ARAŞTIRMA MERKEZİ)0000-0001-6172-7762
İbrahim Halil Kurt(SAĞLIK BİLİMLERİ ÜNİVERSİTESİ, ADANA ŞEHİR SAĞLIK UYGULAMA VE ARAŞTIRMA MERKEZİ)0000-0001-5007-2246
Pages
1003–1020
Publication Date
2026-09-30
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AI Summary & Key Takeaways

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⚡ TL;DR(Too Long; Didn't Read)

Results: Overall, 22 patients (81.5%) met the previous definition, while 5 (18.5%) were classified as 2022-only early CTD-PAH.

📌 3 Key Takeaways

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Objective & Hypothesis:Introduction/Objectives: The 2022 pulmonary hypertension guidelines lowered the hemodynamic thresholds for pulmonary hypertension and pulmonary arterial hypertension, potentially expanding the spectrum of connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH).
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Key Findings & Data:To evaluate the reclassification impact of the 2022 hemodynamic definition in a catheterization-based cohort of clinically adjudicated CTD-associated Group 1 PAH.
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Conclusion & Impact:Results: Overall, 22 patients (81.5%) met the previous definition, while 5 (18.5%) were classified as 2022-only early CTD-PAH.
🔬Design:Araştırma Makalesi
📍Scope:2022 Verileri
⚙️Variables:Pulmoner Hipertansiyon · Bağ Doku Hastalıkları · Hemodinami

Abstract

Introduction/Objectives: The 2022 pulmonary hypertension guidelines lowered the hemodynamic thresholds for pulmonary hypertension and pulmonary arterial hypertension, potentially expanding the spectrum of connective tissue disease-associated pulmonary arterial hypertension (CTD-PAH). The profile of patients identified only by the updated definition remains incompletely characterized. To evaluate the reclassification impact of the 2022 hemodynamic definition in a catheterization-based cohort of clinically adjudicated CTD-associated Group 1 PAH. Methods: We retrospectively analyzed 27 patients with CTD-associated Group 1 PAH. Patients fulfilling previous mPAP and PVR thresholds were classified as established PAH, whereas those fulfilling the 2022 classification but not the previous definition were classified as 2022-only early CTD-PAH. Clinical, pulmonary function, echocardiographic, and hemodynamic variables were compared. Exploratory high burden was defined as ≥4 positive components among PVR ≥5 WU, mPAP ≥35 mmHg, right atrial mean pressure ≥8 mmHg, cardiac index <2.5 L/min/m², NT-proBNP/ProBNP ≥300, and TAPSE <18 mm. Results: Overall, 22 patients (81.5%) met the previous definition, while 5 (18.5%) were classified as 2022-only early CTD-PAH. Compared with established PAH, the 2022-only group had lower mPAP, sPAP, dPAP, PVR, and TPG, with more preserved FVC. ILD was absent in this group. No 2022-only patient had a high-burden phenotype, compared with 45.5% of established PAH patients. Conclusion: In this CTD-associated Group 1 PAH cohort, the 2022 definition reclassified nearly one-fifth of patients as 2022-only early CTD-PAH. These patients demonstrated a milder hemodynamic profile, preserved pulmonary function, absence of ILD, and no high-burden phenotype, supporting the clinical relevance of early hemodynamic recognition in CTD-PAH. Keywords: Hypertension, Pulmonary; Connective Tissue Diseases; Hemodynamics.

Özet

Giriş/Amaç: 2022 pulmoner hipertansiyon kılavuzları, pulmoner hipertansiyon ve pulmoner arteriyel hipertansiyon için hemodinamik eşikleri düşürerek bağ dokusu hastalığı ilişkili pulmoner arteriyel hipertansiyonun (CTD-PAH) tanısal kapsamını genişletmiştir. Ancak yalnızca güncel tanımla saptanan hastaların klinik ve hemodinamik profili yeterince tanımlanmamıştır. Bu çalışmada, sağ kalp kateterizasyonu ile değerlendirilmiş ve klinik olarak CTD-associated Group 1 PAH kabul edilmiş bir kohortta 2022 hemodinamik tanımının yeniden sınıflandırma etkisinin değerlendirilmesi amaçlandı. Yöntem: Retrospektif olarak 27 CTD-associated Group 1 PAH hastası analiz edildi. Önceki mPAP ve PVR eşiklerini karşılayan hastalar established PAH; 2022 sınıflamasını karşılayıp önceki tanımı karşılamayan hastalar ise 2022-only early CTD-PAH olarak sınıflandırıldı. Klinik, solunum fonksiyon, ekokardiyografik ve hemodinamik değişkenler karşılaştırıldı. Keşifsel high-burden fenotip, PVR ≥5 WU, mPAP ≥35 mmHg, sağ atriyum ortalama basıncı ≥8 mmHg, kardiyak indeks <2.5 L/dk/m², NT-proBNP/ProBNP ≥300 ve TAPSE <18 mm bileşenlerinden en az dördünün varlığı olarak tanımlandı. Bulgular: Toplam 22 hasta (%81.5) önceki tanımı karşılarken, 5 hasta (%18.5) 2022-only early CTD-PAH olarak sınıflandırıldı. Established PAH ile karşılaştırıldığında, 2022-only grupta mPAP, sPAP, dPAP, PVR ve TPG daha düşük, FVC daha korunmuştu. Bu grupta ILD ve high-burden fenotip saptanmadı; established PAH grubunda high-burden oranı %45.5 idi. Sonuç: 2022 tanımı, CTD-associated Group 1 PAH kohortunun yaklaşık beşte birini 2022-only early CTD-PAH olarak yeniden sınıflandırdı. Bu hastalar daha hafif hemodinamik profil, korunmuş solunum fonksiyonu, ILD yokluğu ve high-burden fenotipin bulunmamasıyla ayrıştı. Anahtar Kelimeler: Pulmoner Hipertansiyon, Bağ Doku Hastalıkları, Hemodinami

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Cite This Article

Okyar, B., Evlıce, M., Paçacı, E., Akmaz, B., Kaplan, İ., Coşkun, H., Coşkun, M., Yıldırım, A., Ersözlü, E. D., Kurt, İ. H. (2026). Hemodynamic Reclassification Under the 2022 Definition in Connective Tissue Disease-Associated Pulmonary Arterial Hypertension: A Single-Center Cohort Study. Çukurova Anestezi ve Cerrahi Bilimler Dergisi, Cilt: 9 Sayı: 3, 1003–1020. https://doi.org/10.36516/jocass.2004738

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