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Vasculature involvement of AL amyloid or amyloid fibrils has been commonly reported in tissue biopsy. However, the prognostic role of vasculature involvement has not been studied.
Tissue biopsies of patients with biopsy-proven AL amyloidosis were reviewed between 2006 to 2011. Tissue and vasculature involvement were recorded from Congo red staining and/or electronic microscopic detection of amyloid fibrils in tissue and/or capillaries. Amyloid deposition in vasculature was defined as Congo red staining of small vessels and fibrils deposition in capillaries under electronic microscope. EKG and echocardiography at the time of diagnosis were reviewed. NCCN Guidelines Version 1.2012 was used for reassessing cardiac involvement for all patients. Death was censored till November 30, 2011.
28 patients with biopsy data available were included for the analysis. Mean age was 64.8 ± 9.9 years. All patients had at least one organ biopsy with tissue Congo Red staining positive. 18 patients with positive amyloid deposition in vasculature (V positive), 14 (77.8%) had cardiac involvement based on NCCN criteria; 10 patients with negative amyloid deposition (V negative), 4 (40%) had cardiac involvement (P<0.01). LV wall thickness and LV filling pressure (E/e') were significantly increased in V positive group comparing with V negative group (15 mm ± 0.07 vs 1.1 mm ± 0.07; 21.6 ±1.7 vs 11 ±1.7 respectively, both P = 0.002). 7 cases in V positive group had pericardial effusion comparing 0 case in V negative group (P = 0.03). 12 cases with low voltage from ECG in V positive group comparing with 3 cases in V negative group (P = 0.03). LV ejection fraction, left atrium size, pulmonary pressure, troponin I and NT-ProBNP levels were not significantly different between two groups. Mortality rate in V positive group (10/18, 55.6%) was significantly higher comparing with V negative group (2/10, 20%) (P = 0.04)
The patients with positive amyloid deposition in vasculature had significantly worse outcome compared with the patients with negative amyloid deposition. This is likely contributed to more and severe cardiac involvement.
Poster Sessions, Expo North
Saturday, March 09, 2013, 3:45 p.m.-4:30 p.m.
Session Title: What Is Unfolding in Cardiac Amyloidosis Research?
Abstract Category: 23. Pericardial/Myocardial Disease
Presentation Number: 1163-151
- 2013 American College of Cardiology Foundation