Article

Deposition of amyloid proteins in the epicardial coronary arteries of 58 patients with primary systemic amyloidosis

Department of Internal Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.
Cardiovascular Pathology (Impact Factor: 2.34). 03/2007; 16(2):75-8. DOI: 10.1016/j.carpath.2006.09.011
Source: PubMed

ABSTRACT We sought to determine the distribution and the effect of amyloid on epicardial coronary arteries in patients with primary cardiac amyloidosis.
We reviewed pathologic specimens taken after autopsy or cardiac transplantation from 58 patients with primary cardiac amyloidosis. Patients were seen from 1981 to 2000. Multiple sections of epicardial coronary arteries (left anterior descending artery, left circumflex artery, and right coronary artery) were examined to determine the degree of amyloid deposition in the intima, media, adventitia, and vasa vasorum (vasa vasorum are nutrient arteries for the coronary arteries themselves).
In 56 of 58 patients (97%), amyloid was present in epicardial coronary arteries. Amyloid was identified in all artery layers (intima, media, and adventitia), and more patients had amyloid in the adventitia. However, amyloid did not cause intraluminal obstruction of epicardial coronary arteries in any patient. The vasa vasorum had considerable deposits and, in many patients, were obstructed by amyloid. Patients with obstruction of the vasa vasorum were significantly more likely to have obstructive intramural coronary amyloidosis than patients without vasa vasorum obstruction (P=.002).
The epicardial coronary arteries of patients with primary cardiac amyloidosis had extensive amyloid deposition. This deposition, however, did not lead to obstruction of epicardial coronary arteries and therefore did not contribute to ischemic syndromes observed in these patients. Obstruction of the vasa vasorum was associated with obstructive intramural coronary amyloidosis.

Download full-text

Full-text

Available from: William D Edwards, Jul 06, 2015
0 Followers
 · 
72 Views
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Cardiac involvement occurs frequently in primary amyloidosis and is associated with heart failure hospitalizations and poor survival. The initial presentation of the disease may be misleading, resulting in under-diagnosis of cardiac amyloidosis and late initiation of treatment. We present a case of cardiac amyloidosis initially misdiagnosed as hypertrophic cardiomyopathy and we discuss the key findings of the disease along with the latest evidence regarding the management and prognosis of cardiac amyloidosis.
    Hellenic journal of cardiology: HJC = Hellēnikē kardiologikē epitheōrēsē 51(6):552-7. · 0.79 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: The literature is unclear as to the frequency and degree of vascular involvement among the various types of cardiac amyloidosis, specifically the senile type. We analyzed the intramyocardial vascular involvement, both qualitatively and quantitatively, in 18 patients with autopsy-proven cardiac amyloidosis [11 with primary amyloidosis (AL) and 7 with senile systemic amyloidosis (SSA)]. AL patients (10/11) showed focally transmural vascular involvement by amyloid with thickening of the wall and impingement on the lumens. In SSA patients (6/7), the amyloid deposition was concentrated largely in the adventitia and external media. Histomorphometric analysis revealed that all patients with AL, SSA, and heart failure without amyloidosis had greater luminal areas than normal controls. In addition, the median of the ratio of subendocardial to subepicardial vascular luminal areas was 0.59 in AL patients, 1.17 in SSA patients, 0.94 in heart failure without amyloidosis patients, and 0.89 in normal controls (P=.034). This study shows that the degree of vascular involvement and wall thickening with subsequent impingement on the lumen is much greater in AL than in SSA. All patients with heart failure, with and without amyloidosis, displayed greater luminal areas than normal controls. Moreover, a twofold dilatation of the vessels in the subepicardium compared to those in the subendocardium was noted only in patients with AL. This may be due to transmural vascular involvement by amyloid in these patients, which concomitantly decreases the transmural ventricular perfusion pressure, leading to compensatory expansion of the subepicardial vessels.
    Cardiovascular Pathology 03/2008; 17(2):65-71. DOI:10.1016/j.carpath.2007.05.008 · 2.34 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: At least 12 distinct forms of amyloidosis are known to involve the heart or great vessels. Patient treatment regimens require proper subtyping of amyloid deposits in small diagnostic cardiac specimens. A growing lack of confidence in immunohistochemical staining for subtyping amyloid has arisen primarily as a result of studies utilizing immunoperoxidase staining of formalin-fixed paraffin-embedded tissue. Immunofluorescence staining on fresh frozen tissue is generally considered superior to immunoperoxidase staining for subtyping amyloid; however, this technique has not previously been reported in a series of cardiac specimens. Amyloid deposits were subtyped in 17 cardiac specimens and 23 renal specimens using an immunofluorescence panel. Amyloid deposits were successfully subtyped as AL, AH, or AA amyloid by immunofluorescence in 82% of cardiac specimens and 87% of renal specimens. In all cases, the amyloid classification was in good agreement with available clinical and laboratory assessments. A cross-study analysis of 163 cases of AL amyloidosis reveals probable systemic misdiagnosis of cardiac AL amyloidosis by the immunoperoxidase technique, but not by the immunofluorescence technique. Amyloid deposits can be reliably subtyped in small diagnostic cardiac specimens using immunofluorescence. The practical aspects of implementing an immunofluorescence approach are compared with those of other approaches for subtyping amyloid in the clinical setting.
    Cardiovascular pathology: the official journal of the Society for Cardiovascular Pathology 08/2008; 18(4):205-16. DOI:10.1016/j.carpath.2008.05.004 · 2.34 Impact Factor
Show more