‘Here comes the story of the Hurricane’: a case report of AL cardiac amyloidosis and myocardial bridging

Abstract

Background Cardiac amyloidosis (CA) is a rapidly progressive infiltrative cardiomyopathy, whose role is emerging as a not-so-rare disorder leading to heart failure (HF). Myocardial bridge (MB) is the most common inborn coronary artery variant, and its clinical relevance is still matter of debate. The exceptional coexistence of these two conditions could accelerate disease progression and worsen the already compromised clinical conditions. Case summary We present the case of a 76-year-old female patient experiencing relapsing HF decompensation and presenting to our center with dyspnea at rest and severe peripheral congestion. Echocardiogram showed severe concentric hypertrophy, severe biventricular contractile dysfunction and third degree diastolic disfunction. Coronary angiography excluded epicardial atherosclerotic disease, though displaying a long intramyocardial course of left anterior descending artery (LAD). Physiological invasive test was achieved in terms of instantaneous wave-free ratio (iFR), both at baseline and after inotropic and chronotropic stimuli, and attested hemodynamic significance. Concurrently, the diagnostic flowchart for cardiac amyloidosis was accomplished, by means of both invasive (periumbilical fat biopsy, bone marrow aspiration) and non-invasive tests (99mTc-diphosphonate scintigraphy, serum-urine immunofixation) that confirmed the suspect of primary amyloidosis. Acute heart failure therapy was personalized according to the singularity of the case, avoiding both nitrates and β-blockers, then first cycle of chemotherapy was started. Discussion Our clinical case shows a unique interaction between infiltrative cardiomyopathy and coronary artery abnormality. Amyloidosis can contribute to the ischemic burden of the MB and this may, in turn, abbreviate the path to heart failure decompensation.

Primary Systemic Amyloidosis: A Case Report

“…It is characterised by infiltrative cardiomyopathy which ultimately results in heart failure. 2 , 8 A case of relapsing decompensated heart failure with severe concentric hypertrophy and diastolic dysfunction was reported which is similar to our case where our patient has concentric left ventricular hypertrophy, and diastolic dysfunction with a reduced ejection fraction of 20-25%. 8 …”

Heart Failure and Cardiomyopathies: CT and MR from Basics to Advanced Imaging

“…T1 and ECV can show a marked increase in T1 values and expansion of extracellular volume. LGE, moreover, can show the absence of myocardial nulling and diffuse subendocardial enhancement [ 262 ]. Finally, T2 mapping shows a significant prognostic implication in amyloidosis, resulting as increased, especially in non-treated AL phenotypes [ 263 ].…”

Comprehensive functional and anatomic assessment of myocardial bridging: Unlocking the Gordian Knot

“…Although this anomaly is present at birth, the onset of symptoms usually does not occur before the third decade of age. Several factors, such as concomitant coronary artery disease (CAD), tachycardia, and the rise in left ventricular (LV) pressures, usually associated with aging, diastolic dysfunction and LV hypertrophy, may worsen the supply-demand mismatch imposed by MB and unmask or exacerbate the hemodynamic impact of MB ( 8 , 9 ).…”