Ciddi pulmoner arteryal hipertansiyonlu geniş ventriküler septal defektli olgularının cerrahi sonuçları ve uzun dönem takibi: tek merkez deneyimi

Yıl 2023, Cilt: 48 Sayı: 3, 1131 - 1138, 30.09.2023

Ali Orgun , Niyazi Tokel , İlkay Erdoğan , Birgül Varan , Murat Özkan , Mehmet Sait Aşlamacı

https://doi.org/10.17826/cumj.1335119

Öz

Amaç: Bu retrospektif çalışmada, şiddetli pulmoner arteryal hipertansiyonlu (PAH) geniş ventriküler septal defekt (VSD) nedeniyle ameliyat edilen pediatrik hastalar, klinik özellikleri ve uzun dönem sonuçları açısından ayrıntılı olarak değerlendirilmiş ve güncel ameliyat kriterleri ile yeniden yorumlanmıştır.
Gereç ve Yöntem: 1994-2005 yılları arasında PAH’lı geniş VSD nedeni ile ameliyat edilen ve takibi yapılan 24 çocuk hasta çalışmaya dahil edildi. Hastalar pulmoner vasküler resiztans indexine(PVRI) göre iki gruba ayrıldı ve karşılaştırıldı (grup 1:PVRI <6 WU.m² olan olgular, grup 2: PVRI ≥ 6 WU.m² olan olgular). PVRI 6-8 WU.m² olan olgular gri bölgede kabul edildi ve detaylı olarak değerlendirildi.
Bulgular: Tanı yaşı 5 ay ile 17 yıl (medyan: 5 yıl) arasındaydı. Ortanca PVRI 5 WU.m² olup 3 -18,4 WU.m² arasında değişmekteydi. PVRI' ye göre 13 hasta grup 1, 11 hasta ise grup 2 olarak kabul edildi. Grup 1'de grup 2'ye göre istatistiksel olarak anlamlı olarak mPAP, PVR/SVR ve son PAP değerleri düşük bulundu. Ameliyat sonrası ortanca takip süresi 6 yıl (1 ay-18 yıl) olup takipte 15 hastada (%62,5) PAH görüldü. Bu 15 hastanın 7'sinde şiddetli PAH gözlenmiş olup bu 7 hastanın tamamı grup 2'deydi (dördünde PVRI ≥8WU.m² ve üçünde PVRI 6-8 WU.m²).
Sonuç: Şiddetli PAH gelişmiş geniş VSD'si olan hastalarda, ameliyat için uygun adayları daha doğru bir şekilde belirlemek için ameliyattan önce klinik ve özellikle hemodinamik durumları dikkatlice değerlendirilmelidir.

Anahtar Kelimeler

Doğuştan kalp hastalığı, pulmoner arteriyal hipertansiyon, ventriküler septal defekt

Surgical outcomes and long-term follow-up of patients with large ventricular septal defects with severe pulmonary arterial hypertension: single-center experience

Öz

Purpose: In this retrospective study, pediatric patients who underwent surgery for large ventricular septal defect (VSD) with severe pulmonary arterial hypertension (PAH) were evaluated in detail in terms of clinical characteristics, and long-term results, and reinterpreted with the current surgical criteria.
Materials and Methods: Twenty-four patients who underwent surgery for large VSD due to PAH between 1994 and 2005 were included in the study. The patients were divided into two groups according to their pulmonary vascular resistance index (PVRI) and compared (group 1 with PVRI <6 WU.m² and group 2 with PVRI ≥ 6 WU.m²). The patients with PVRI 6-8 WU.m² were accepted as being in the gray zone and were evaluated in detail.
Results: Age at diagnosis ranged from 5 months to 17 years (median: 5 years). PVRI ranged between 3 and 18.4 WU.m² (median PVRI was 5 WU.m².) According to the PVRI, 13 patients were accepted as group 1, and 11 patients were accepted as group 2. mPAP, PVR/SVR, and last PAP values were found statistically significantly lower in group 1 than in group 2. The median postoperative follow-up time was 6 years (range, 1 month-18 years). PAH was seen in 15/24 (62.5%) patients. Severe PAH was observed in seven of these 15 patients and all were in group 2 (four had PVRI ≥8WU.m² and three had PVRI 6-8 WU.m²).
Conclusion: Patients with large VSD with severe PAH should be carefully evaluated before surgery to more accurately identify suitable candidates.

Anahtar Kelimeler

Congenital heart disease, Pulmonary arterial hypertension, Ventricular septal defect

Kaynakça

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