Table 2 Open in new tab Clinical, ECG,...

Cardiomyopathy

Clinical evaluation

ECG

Echocardiographic features

Speckle tracking echocardiography

CMR

AFD

Extracardiac signs:

Neurological (acroparaesthesia, stroke-like events)
Renal (proteinuria, end-stage kidney failure)
Ocular (corneal dystrophy)
Cutaneous (angiokeratoma, hypohidrosis)
Gastrointestinal (abdominal pain, vomiting, diarrhoea)

ECG: short PR, conduction abnormalities

Severe concentric LVH
Preserved LVEF
Progressive increase in LV volumes
LV diastolic dysfunction

Reduced longitudinal strain in the basal posterior-lateral wall
Reduced free wall RV strain

LVH
LGE in the basal and mid-inferolateral
Low T1 values
Increased T2 values in LGE area
Normal ECV

Cardiac amyloidosis

Extracardiac signs:

Neurological (carpal tunnel syndrome, peripheral neuropathy, autonomic neuropathy, spinal cord stenosis)
Ocular (cataract, vitreous opacity)

Normal or reduced QRS voltages
Q waves (pseudoinfarction pattern)
AF or conduction abnormalities

Concentric LVH, often RV hypertrophy
Preserved LVEF and progressive reduction in LV volumes
Biventricular and atrial septal hypertrophy
Biatrial enlargement
Pericardial effusion
Early LV diastolic dysfunction

Relative apical sparing pattern
Early LA strain reduction
Reduced RV strain pattern in severe disease
Reduced GCW and GWE by LV myocardial work

LVH
Subendocardial diffuse or transmural LGE
Increased ECV (>40%)
High T1 and low T2 values

HCM

Family history of sudden cardiac death/HCM

Meets voltage criteria for LVH
Biatrial enlargement
ST-T abnormalities

Asymmetrical hypertrophy with maximum wall thickness > 15 mm
Possible apical LVH
LVEF usually normal or mildly impaired
Possible presence of LVOTO and/or SAM with secondary MR (posteriorly directed jet)

Reduced longitudinal strain and GCW

LVH
No specific LGE pattern
T1 and ECV values slightly increased in hypertrophic areas
T2 values increased if oedema

Table 2

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Clinical, ECG, and imaging features for differential diagnosis between AFD, cardiac amyloidosis, and sarcomeric hypertrophic cardiomyopathy (HCM)¹⁰

Cardiomyopathy	Clinical evaluation	ECG	Echocardiographic features	Speckle tracking echocardiography	CMR
AFD	Extracardiac signs: Neurological (acroparaesthesia, stroke-like events) Renal (proteinuria, end-stage kidney failure) Ocular (corneal dystrophy) Cutaneous (angiokeratoma, hypohidrosis) Gastrointestinal (abdominal pain, vomiting, diarrhoea)	ECG: short PR, conduction abnormalities	Severe concentric LVH Preserved LVEF Progressive increase in LV volumes LV diastolic dysfunction	Reduced longitudinal strain in the basal posterior-lateral wall Reduced free wall RV strain	LVH LGE in the basal and mid-inferolateral Low T1 values Increased T2 values in LGE area Normal ECV
Cardiac amyloidosis	Extracardiac signs: Neurological (carpal tunnel syndrome, peripheral neuropathy, autonomic neuropathy, spinal cord stenosis) Ocular (cataract, vitreous opacity)	Normal or reduced QRS voltages Q waves (pseudoinfarction pattern) AF or conduction abnormalities	Concentric LVH, often RV hypertrophy Preserved LVEF and progressive reduction in LV volumes Biventricular and atrial septal hypertrophy Biatrial enlargement Pericardial effusion Early LV diastolic dysfunction	Relative apical sparing pattern Early LA strain reduction Reduced RV strain pattern in severe disease Reduced GCW and GWE by LV myocardial work	LVH Subendocardial diffuse or transmural LGE Increased ECV (>40%) High T1 and low T2 values
HCM	Family history of sudden cardiac death/HCM	Meets voltage criteria for LVH Biatrial enlargement ST-T abnormalities	Asymmetrical hypertrophy with maximum wall thickness > 15 mm Possible apical LVH LVEF usually normal or mildly impaired Possible presence of LVOTO and/or SAM with secondary MR (posteriorly directed jet)	Reduced longitudinal strain and GCW	LVH No specific LGE pattern T1 and ECV values slightly increased in hypertrophic areas T2 values increased if oedema

Cardiomyopathy	Clinical evaluation	ECG	Echocardiographic features	Speckle tracking echocardiography	CMR
AFD	Extracardiac signs: Neurological (acroparaesthesia, stroke-like events) Renal (proteinuria, end-stage kidney failure) Ocular (corneal dystrophy) Cutaneous (angiokeratoma, hypohidrosis) Gastrointestinal (abdominal pain, vomiting, diarrhoea)	ECG: short PR, conduction abnormalities	Severe concentric LVH Preserved LVEF Progressive increase in LV volumes LV diastolic dysfunction	Reduced longitudinal strain in the basal posterior-lateral wall Reduced free wall RV strain	LVH LGE in the basal and mid-inferolateral Low T1 values Increased T2 values in LGE area Normal ECV
Cardiac amyloidosis	Extracardiac signs: Neurological (carpal tunnel syndrome, peripheral neuropathy, autonomic neuropathy, spinal cord stenosis) Ocular (cataract, vitreous opacity)	Normal or reduced QRS voltages Q waves (pseudoinfarction pattern) AF or conduction abnormalities	Concentric LVH, often RV hypertrophy Preserved LVEF and progressive reduction in LV volumes Biventricular and atrial septal hypertrophy Biatrial enlargement Pericardial effusion Early LV diastolic dysfunction	Relative apical sparing pattern Early LA strain reduction Reduced RV strain pattern in severe disease Reduced GCW and GWE by LV myocardial work	LVH Subendocardial diffuse or transmural LGE Increased ECV (>40%) High T1 and low T2 values
HCM	Family history of sudden cardiac death/HCM	Meets voltage criteria for LVH Biatrial enlargement ST-T abnormalities	Asymmetrical hypertrophy with maximum wall thickness > 15 mm Possible apical LVH LVEF usually normal or mildly impaired Possible presence of LVOTO and/or SAM with secondary MR (posteriorly directed jet)	Reduced longitudinal strain and GCW	LVH No specific LGE pattern T1 and ECV values slightly increased in hypertrophic areas T2 values increased if oedema

AF, atrial fibrillation; ECV, extracellular volume; GCW, global constructive work; GWE, global work efficiency; HCM, hypertrophic cardiomyopathy; LGE, late gadolinium enhancement; LV, left ventricular; LVEF, left ventricular ejection fraction; LVH, left ventricular hypertrophy; LVOTO, left ventricular outflow tract obstruction; MR, mitral regurgitation; RV, right ventricle; SAM, systolic anterior motion.

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