Deposition of amyloid proteins in the epicardial coronary arteries of 58 patients with primary systemic amyloidosis.
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.
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ABSTRACT: The heart is involved in more than one third of patients with primary (AL) amyloidosis at diagnosis and it is by far the most common cause of death. Rhythm and conduction abnormalities generally represent the terminal event. The aims of this study were to determine the spectrum of Holter abnormalities found in AL amyloidosis and to assess their prognostic significance, particularly in relation to sudden death. Fifty-one patients with AL amyloidosis were included, and all of them had a complete history, physical examination, two-dimensional echocardiography, and 24-hour Holter monitoring. Fifty-five percent of these patients had echographic signs of heart involvement and 23% had heart failure. Complex ventricular arrhythmias were found in 57% of patients, couplets in 29%, and nonsustained ventricular tachycardia in 18%. Overall median survival was 23.4 months. Congestive heart failure, echocardiographic abnormalities, and Holter abnormalities adversely affected survival. The multivariate analysis demonstrated that interventricular septum thickness and couplets were independent predictors of survival. The presence of couplets correlated with sudden death. Holter monitoring may contribute to assessing the prognosis of patients with AL amyloidosis.Pacing and Clinical Electrophysiology 09/2001; 24(8 Pt 1):1228-33. · 1.75 Impact Factor
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ABSTRACT: The purpose of this study was to determine the prevalence of clinical syndromes and pathologic changes of myocardial ischemia due to obstructive intramural coronary amyloidosis among patients with primary amyloidosis and cardiac involvement. Medical records and pathologic specimens were reviewed from 96 patients with primary amyloidosis and cardiac involvement at autopsy or after cardiac transplantation during a 20-year period. Medical records were reviewed for patient demographic and clinical characteristics, including evidence for syndromes of myocardial ischemia. Pathologic specimens were examined for obstructive intramural coronary amyloidosis and microscopic changes of myocardial ischemia. Obstructive intramural coronary amyloidosis was present in 63 of 96 patients (66%). Microscopic changes of myocardial ischemia were more common in patients with obstructive intramural coronary amyloidosis (86%) than in those without (52%) (P <.001). In the 76 patients without coexistent severe epicardial coronary atherosclerosis, changes of myocardial ischemia were more common in those with obstructive intramural coronary amyloidosis (83%) than in those without (45%) (P <.001). In patients who had tissue available for review, none had obstruction of epicardial coronary arteries from amyloid. Syndromes of myocardial ischemia affected 16 patients (25%) with obstructive intramural coronary amyloidosis but only 2 patients (6%) without (P=.027). For 11% of the patients with obstructive intramural coronary amyloidosis, a syndrome of myocardial ischemia consisting of acute myocardial infarction or angina pectoris was the first manifestation of primary amyloidosis. Most patients with primary systemic amyloidosis and cardiac involvement have obstructive intramural coronary amyloidosis and associated microscopic changes of myocardial ischemia. Syndromes of myocardial ischemia may occur in these patients.The American journal of medicine 11/2005; 118(11):1287. · 4.47 Impact Factor
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ABSTRACT: The heart is subject to involvement by primary (AL), senile (AS), and familial (AF) forms of amyloidosis, but the frequency, severity, and therapy of amyloid-related cardiac symptoms differ depending on the type of amyloidosis present. Endomycardial biopsy is a safe and reliable procedure for diagnosing cardiac amyloidosis, and immunohistochemical staining of routinely processed biopsy specimens can be performed to classify the type of amyloid present. However, whether or not the type can be determined from the histologic extent and pattern of amyloid deposition is unclear. Endomyocardial biopsy specimens from 100 patients with cardiac amyloidosis (74 AL, 22 AS, 4 AF) were examined, and the histologic extent and pattern of amyloid deposition were correlated with the amyloid immunophenotype. No difference in the extent of amyloid deposition was identified among the three types. Interstitial nodules of amyloid were more common in AS (82%) than in AL (50%, p = 0.0129), whereas vascular involvement was more frequently observed in AL (88%) than in AS (26%, p < 0.0001). Endocardial and interstitial pericellular deposition occurred with similar frequencies in both groups. Although statistically significant differences existed in the patterns of amyloid deposition, they did not allow reliable distinction between the different types in individual cases. Consequently, in older patients without serum or urinary light chains, immunohistochemical staining is recommended to distinguish AL from AS types of amyloid in cardiac biopsy tissues.Cardiovascular Pathology. 01/1995;