Table 1 Open in new tab Multimodality...

Imaging modality

Typical findings

Echocardiography

Concentric LVH
LVOTO or mid-cavity obstruction
Abnormal LV contractile reserve on exertion
Hypertrophy of papillary muscle
LV diastolic dysfunction
Left atrial dilatation
Posterolateral reduced GLS
Hypokinesis of the inferolateral LV wall
RV hypertrophy
Mild valvular thickening with regurgitation
Aortic root dilatation

Cardiac magnetic resonance

Concentric LVH
Hypertrophy of papillary muscles
Increased myocardial trabeculation
Preserved LV ejection fraction.
LGE in the basal and mid-inferolateral wall
Progression towards extensive LGE (in the advanced stages of the disease)
Low myocardial T1 values
Increased myocardial T2 values in LGE area
Pseudo-normalization of myocardial T1 signal in LGE area
Normal extracellular volume (sometimes increased in LGE area)
Right ventricular hypertrophy

Nuclear imaging

Perfusion defects observed on SPECT scans in specific areas
global reduction in coronary flow reserve obtained by PET (coronary microvascular dysfunction as early sign)
Focal ¹⁸F-FDG uptake
Reduced MIBG uptake

Table 1

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Multimodality imaging key findings in AFD

Imaging modality	Typical findings
Echocardiography	Concentric LVH LVOTO or mid-cavity obstruction Abnormal LV contractile reserve on exertion Hypertrophy of papillary muscle LV diastolic dysfunction Left atrial dilatation Posterolateral reduced GLS Hypokinesis of the inferolateral LV wall RV hypertrophy Mild valvular thickening with regurgitation Aortic root dilatation
Cardiac magnetic resonance	Concentric LVH Hypertrophy of papillary muscles Increased myocardial trabeculation Preserved LV ejection fraction. LGE in the basal and mid-inferolateral wall Progression towards extensive LGE (in the advanced stages of the disease) Low myocardial T1 values Increased myocardial T2 values in LGE area Pseudo-normalization of myocardial T1 signal in LGE area Normal extracellular volume (sometimes increased in LGE area) Right ventricular hypertrophy
Nuclear imaging	Perfusion defects observed on SPECT scans in specific areas global reduction in coronary flow reserve obtained by PET (coronary microvascular dysfunction as early sign) Focal ¹⁸F-FDG uptake Reduced MIBG uptake

Imaging modality	Typical findings
Echocardiography	Concentric LVH LVOTO or mid-cavity obstruction Abnormal LV contractile reserve on exertion Hypertrophy of papillary muscle LV diastolic dysfunction Left atrial dilatation Posterolateral reduced GLS Hypokinesis of the inferolateral LV wall RV hypertrophy Mild valvular thickening with regurgitation Aortic root dilatation
Cardiac magnetic resonance	Concentric LVH Hypertrophy of papillary muscles Increased myocardial trabeculation Preserved LV ejection fraction. LGE in the basal and mid-inferolateral wall Progression towards extensive LGE (in the advanced stages of the disease) Low myocardial T1 values Increased myocardial T2 values in LGE area Pseudo-normalization of myocardial T1 signal in LGE area Normal extracellular volume (sometimes increased in LGE area) Right ventricular hypertrophy
Nuclear imaging	Perfusion defects observed on SPECT scans in specific areas global reduction in coronary flow reserve obtained by PET (coronary microvascular dysfunction as early sign) Focal ¹⁸F-FDG uptake Reduced MIBG uptake

¹⁸F-FDG, 18F-fluorodeoxyglucose; LGE, late gadolinium enhancement; GLS, global longitudinal strain; LV, let ventricular; LVOTO, left ventricular outflow tract obstruction; PET, positron emission tomography; RV, right ventricle; SPECT, single-photon emission computed tomography.

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