Cardiac amyloidosis presenting as recurrent acute coronary syndrome with unobstructed coronary arteries: Case report

Abstract

Amyloidosis is a systemic disorder characterized by the deposition of mis-folded protein molecules within various organs. Cardiac involvement may be the presenting feature of this condition or may be identified incidentally during investigation for amyloidosis affecting other organs. The presence and severity of cardiac involvement varies with the type of amyloidosis. Irrespective of the subtype, patients with cardiac amyloidosis usually present with symptoms of heart failure with echocardiography showing features of restrictive cardiomyopathy. The usual cardiac symptoms noted in patients with amyloidosis include dyspnea, peripheral edema, and palpitations secondary to arrhythmias.(1) Chest pain secondary to myocardial ischemia is an unusual presentation of cardiac amyloidosis, and is attributed to the deposition of protein molecules in the coronary microvasculature. We describe the case of a patient who presented with recurrent cardiac ischemia secondary to amyloidosis.

A Case Report of Cardiac Amyloidosis Presenting with Chest Discomfort

“…Amyloid deposition in the heart results in cardiac amyloidosis, clinically presents with rapidly progressive heart failure because of restrictive cardiomyopathy, and sometimes presents with typical angina due to amyloid deposition in arterial vessel wall causing progressive luminal narrowing and hence myocardial ischemia and impairment [7]. Anish et al described a case with cardiac amyloidosis presenting as recurrent acute coronary syndrome without coronary artery obstruction [8]. Diagnosis of amyloidosis relies on clinical awareness of the disease, clinical features, blood and tissue analysis, and positive findings on biopsy.…”

Medin amyloid and cardiovascular diseases: a review of the biology and the implications for the diagnosis and treatment of cardiac amyloidosis

“…The close correlation between medin deposition and hypertension may partly explain the role of medin in the pathogenesis of hypertensive cardiomyopathy and heart failure resulting from increasing cardiac afterload. As noted above, amyloid deposition in intramural coronary arteries may lead to a typical presentation of angina pectoris despite the normal coronary angiography presentation [1]. Indeed, it was demonstrated in a retrospective study that 66% of the 98 studied patients with immunoglobulin light chain (AL) amyloidosis presented intramural amyloid deposits, while 25% had symptoms suggestive of amyloidosis-related ischaemia [7].…”

“…One pathophysiological explanation for this presentation revolves around amyloid-deposition-related coronary changes. Specifically, an accumulation of amyloid in the coronary artery walls may lead to progressive narrowing of the arterial lumen, thereby causing myocardial ischaemia and cardiac dysfunction [1]. However, few studies have focused on the relationship between medin and coronary artery disease, despite the fact that medin is perhaps the most common amyloid protein that deposits in the aorta [2].…”

Impact of amyloidosis on the outcomes of patients with coronary artery disease: A nationwide analysis

“…8,9 However, a concrete link between cardiac amyloidosis and coronary artery disease (CAD), the leading cause of cardiovascular death in adults in the USA, has not been completely explored and elucidated in studies. [10][11][12] Cardiac amyloidosis and CAD may share a common ground in chest pain manifestation as the deposition of amyloid in the lumen of epicardial coronary arteries is analogous to atherosclerotic changes in CAD. [13][14][15] On the other hand, cardiac amyloidosis is also thought to accelerate preexisting CAD by precipitating endothelial dysfunction.…”