Obstructive intramural coronary amyloidosis and myocardial ischemia are common in primary amyloidosis

Michelle A Neben-Wittich1, Christopher M Wittich, Paul S Mueller

  • 1Department of Internal Medicine, Mayo Clinic, Rochester, Minn 55905, USA.

Insights

Most patients with cardiac amyloidosis have obstructive intramural coronary amyloidosis, leading to myocardial ischemia. This condition can manifest as heart attacks or angina, sometimes being the first sign of primary amyloidosis.

Area of Science:

  • Cardiology
  • Pathology
  • Amyloidosis Research

Background:

  • Primary systemic amyloidosis frequently involves the heart.
  • Cardiac involvement can lead to heart failure and arrhythmias.
  • Coronary artery pathology in cardiac amyloidosis is not fully understood.

Purpose of the Study:

  • To determine the prevalence of obstructive intramural coronary amyloidosis in patients with primary amyloidosis and cardiac involvement.
  • To investigate the association between obstructive intramural coronary amyloidosis and myocardial ischemia.
  • To identify clinical syndromes of myocardial ischemia in this patient population.

Main Methods:

  • Retrospective review of medical records and pathologic specimens from 96 patients with primary amyloidosis and cardiac involvement.
  • Analysis of autopsy or cardiac transplantation cases over a 20-year period.
  • Examination for obstructive intramural coronary amyloidosis and microscopic ischemic changes.

Main Results:

  • Obstructive intramural coronary amyloidosis was found in 66% of patients.
  • Microscopic myocardial ischemia was significantly more common in patients with obstructive intramural coronary amyloidosis (86%) versus without (52%).
  • Syndromes of myocardial ischemia occurred in 25% of patients with obstructive intramural coronary amyloidosis, sometimes as the initial presentation of primary amyloidosis.

Conclusions:

  • Obstructive intramural coronary amyloidosis is highly prevalent in patients with primary systemic amyloidosis and cardiac involvement.
  • Associated microscopic myocardial ischemia is common.
  • Clinical syndromes of myocardial ischemia are a recognized complication.
Abstract

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