Cardiac amyloidosis‐A review of current literature for the practicing physician

Abstract

The amyloidoses are a family of diseases in which misfolded precursor proteins aggregate to form amyloid and deposit in body tissues. A very serious yet underrecognized form of this disease is cardiac amyloidosis, in which amyloid deposits into the extracellular space of the myocardium, resulting in thickening and stiffening of ventricular walls with resultant heart failure and conductive dysfunction. This review provides a discussion of the pathogenesis and clinical presentation of cardiac amyloidosis subtypes, as well as an up‐to‐date approach to diagnosis and treatment. Significant progress has been made in recent years regarding diagnosis and treatment of this condition, but prognosis remains heavily reliant on early detection of the disease. Two types of precursor protein are responsible for most cardiac amyloidosis cases: transthyretin amyloid, and immunoglobulin‐derived light chain amyloid. An early diagnosis of cardiac amyloidosis can allow for novel treatment modalities to be initiated with the potential to improve prognosis.

Cardiac Amyloidosis with Discordant QRS Voltage between Frontal and Precordial Leads

“…Moreover, global pattern of LGE is also shown to provide incremental prognostics over biomarker stage [ 17 ]. Finally, cMRI is useful in recognizing concentric and symmetric LV thickening which tends to be far more frequent in AL amyloidosis then in ATTR amyloidosis (68% vs. 18%) [ 18 , 19 ].…”

“…Moreover, global pattern of LGE is also shown to provide incremental prognostics over biomarker stage [17]. Finally, cMRI is useful in recognizing concentric and symmetric LV thickening which tends to be far more frequent in AL amyloidosis then in ATTR amyloidosis (68% vs. 18%) [18,19]. The voltage along the short axis of the heart is diminished by the phenomenon of voltage cancellation (yellow dotted arrows), while the voltage along the QRS axis in the horizontal plane (yellow solid arrow, Figure 4B), and long axis of the ventricle, such as the voltage in lead V3 used for calculation of Cornell criteria for LVH, is less affected by this phenomenon of cancellation (no opposing walls along this axis).…”

Clinical and prognostic implications of capillary density in patients with cardiac light chain amyloidosis

“…Cardiac amyloidosis is currently considered an underestimated cause of heart failure 1,2 . Recent studies reported that high prevalence of microvascular dysfunction in cardiac amyloidosis is one of pathophysiological mechanisms of heart failure in cardiac amyloidosis 3–5 .…”

“…Cardiac amyloidosis is currently considered an underestimated cause of heart failure. 1 , 2 Recent studies reported that high prevalence of microvascular dysfunction in cardiac amyloidosis is one of pathophysiological mechanisms of heart failure in cardiac amyloidosis. 3 , 4 , 5 Amyloid deposition in the vessel walls and extrinsic compression from perivascular and interstitial amyloid infiltration have impact on microvascular structure.…”

Cardiac Amyloidosis-Challenging Diagnosis and Unclear Clinical Picture

“…Amyloidosis is a disease entity in which proteins of abnormal structure gather in tissues [1]. The biochemical classification of amyloidosis includes the division into three, most common types: amyloidosis AL (Light chain), amyloidosis AA (Amyloid A) and amyloidosis ATTR (Transthyretin) [2].…”