Türkan Seda TAN, Kübra KORKMAZ, İrem DİNÇER, Cemre Gül TEKİN, Hüseyin NAZMAN

Sol Atriyal Uzunlamasına Deformasyon Değerinin Kardiyak Amiloidoz Hastalarında Prognoz Üzerine Etkisinin Değerlendirilmesi

Amaç: İmmünoglobulin hafif zincir amiloidoz (AL) en sık görülen amiloidozdur. Kardiyak tutulum varlığı kötü sağkalımla ilişkili olup prognostik sınıflamada biyobelirteçler kullanılmaktadır. Ekokardiyografik parametreler tanıda kullanılmakla birlikte hastalık ciddiyetini belirlemede kabul gören bir görüntüleme parametresi bulunmamaktadır. Biz çalışmamızda sol atriyal uzunlamasına deformasyon ölçümünün kardiyak AL amiloidoz hastalarında sağkalımı öngörmedeki yerini araştırmak istedik. Gereç ve Yöntem: Çalışmamıza kardiyak amiloidoz saptanan 37 hasta dahil edildi. Hastaların klinik ve demografik verilerine ve transtorasik ekokardiyografi (TTE) görüntülerine arşiv kayıtlarından ulaşıldı. Hastalar sağkalımlarına göre iki gruba ayrıldı. Bulgular: Çalışmaya dahil edilen hastaların ortalama yaşı 63,9±10,8 yıldı. İki grubun demografik özellikleri ile laboratuvar verileri NT-proBNP değeri dışında benzer bulundu. NT-proBNP değeri ölüm olan grupta istatistiksel olarak anlamlı derecede artmış saptandı (p=0,02). TTE parametrelerinden sol atriyal volüm indeksi değeri ölüm olan grupta belirgin olarak artmış (p=0,02), pik atriyal uzunlamasına deformasyon (PALS) değeri ise ölüm olan grupta belirgin olarak azalmış olarak saptandı (p=0,004). Cox regresyon analizinde PALS’nin NT-proBNP değerinden bağımsız, sağkalımın en önemli ön gördürücüsü olduğu saptandı [risk oranı (%95 güven aralığı): 0,92 (0,84-0,98); p=0,01]. Sonuç: Çalışmamızda PALS’nin kardiyak AL amiloidoz hastalarında sağkalımla belirgin olarak ilişkili olduğunu saptadık. PALS’nin kardiyak AL amiloidoz hastalarında prognoza etkisinin daha büyük, prospektif çalışmalarla değerlendirilmesi gerektiğini düşünmekteyiz.

Evaluation of Prognostic Impact of Left Atrial Longitudinal Strain on Patients with Cardiac Amyloidosis

Objectives: Immunoglobulin light chain cardiac amyloidosis (AL) is the most common systemic amyloidosis. Cardiac involvement associated with poor prognosis and biomarkers are used to determine the prognostic stages. Although echocardiographic parameters are used for the diagnosis of cardiac involvement, they are not established as the markers of disease severity. We sought to investigate whether left atrial longitudinal strain parameter predicts survival in patients with cardiac AL amyloidosis. Materials and Methods: Thirty-seven patients with cardiac amyloidosis were included into our study. Clinical and demographic data and transthoracic echocardiographic views were obtained from medical records. All patients were divided into two groups according to their survival. Results: The mean age of the study population was 63.9±10.8 years. Demographic characteristics and laboratory parameters except NT-proBNP were similar between two groups. NT-proBNP value was found as statistically higher in death caused group (p=0.02). In TTE parameters, the left atrial volume index was higher (p=0.02) however, peak atrial longitudinal strain (PALS) was reduced in death caused group (p=0.004). In Cox proportional regression method, PALS was found the most important predictor of survival after adjustment for NT-proBNP [hazard ratio (95% confidence interval): 0.92 (0.84-0.98); p=0.01]. Conclusion: In our study we determined that PALS was highly associated with survival in patients with cardiac AL amyloidosis. We thought that larger, prospective studies are necessary to evaluate the prognostic impact of PALS in patients with cardiac AL amyloidosis.

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