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Symptomatic ischemic heart disease resulting from obstructive intramural coronary amyloidosis
P S Mueller1, W D Edwards, M A Gertz
1Division of General Internal Medicine, Mayo Clinic and Mayo Foundation, Rochester, Minnesota 55905, USA.
Insights
Obstructive intramural coronary amyloidosis, a rare complication of systemic amyloidosis, often presents as symptomatic ischemic heart disease. Diagnosis is challenging, typically occurring post-mortem or after heart transplantation, highlighting a poor prognosis.
Area of Science:
- Cardiology
- Pathology
- Systemic Amyloidosis
Background:
- Systemic amyloidosis can manifest as obstructive intramural coronary amyloidosis, a rare but serious cardiac complication.
- This condition affects the coronary arteries, leading to myocardial injury and ischemic heart disease.
Purpose of the Study:
- To review the clinical characteristics of patients with obstructive intramural coronary amyloidosis.
- To understand the diagnostic challenges and prognosis associated with this rare cardiac condition.
Main Methods:
- Retrospective review of 11 patients diagnosed with intramural cardiac amyloidosis at Mayo Clinic between 1960 and 1999.
- Diagnosis was confirmed via autopsy or examination of explanted hearts.
Main Results:
- Eleven patients (8 men, 3 women) presented with symptomatic ischemic heart disease due to obstructive intramural coronary amyloidosis.
- Common symptoms included angina pectoris, unstable coronary syndromes, and congestive heart failure. Diagnostic tests like coronary angiography and endomyocardial biopsy were often inconclusive.
- Diagnosis was definitively established only at autopsy or after cardiac transplantation, with a mean time to death or transplantation of 18 months post-symptom onset.
Conclusions:
- Diagnosing obstructive intramural coronary amyloidosis before death or cardiac transplantation is exceedingly difficult.
- Despite a poor prognosis, accurate recognition is crucial for potential therapeutic interventions, such as chemotherapy or heart transplantation.
Purpose:
Obstructive intramural coronary amyloidosis is an unusual complication of systemic amyloidosis.
Subjects And Methods:
We review the characteristics of 11 patients seen at the Mayo Clinic (Rochester, Minnesota) from January 1, 1960, to June 1, 1999, with intramural cardiac amyloidosis diagnosed at autopsy or after examination of an explanted heart.
Results:
Symptomatic ischemic heart disease resulting from obstructive intramural coronary amyloidosis was found in 11 patients (8 men, 3 women). The mean (+/-SD) age at the diagnosis of primary amyloidosis was 62 +/- 12 years. All patients had angina pectoris; angina was the presenting symptom of primary amyloidosis in 6 patients. Unstable coronary syndromes occurred in 7 patients and congestive heart failure in 8. New electrocardiographic abnormalities after the development of angina were common and included ischemic changes, bundle branch block, and dysrhythmias. Low voltage was seen in only 2 patients. All 7 patients who underwent coronary angiography had normal or clinically insignificant findings. Endomyocardial biopsy was performed on 4 patients; amyloid was found in 3 patients, none of whom had obstructive intramural coronary amyloidosis. The diagnosis of obstructive intramural coronary amyloidosis with associated myocardial injury was established only at autopsy or after examination of the explanted heart after cardiac transplantation. The mean time to death or cardiac transplantation after symptoms of cardiac ischemia developed was 18 +/- 20 months.
Conclusions:
The diagnosis of ischemic heart disease resulting from obstructive intramural coronary amyloidosis is difficult to establish before death or cardiac transplantation. Although the condition has a poor prognosis, its accurate recognition may have therapeutic implications, because some patients may benefit from treatment, including systemic chemotherapy or cardiac transplantation.