General

Echocardiography remains the first line imaging modality to assess LVH. In most cases, LVH may be attributable to hypertension, aortic stenosis, or obesity. Not considered in this chapter: Mitochondrial myopathy and mucopolysaccharidosis.

Athlete’s Heart

History No symptoms High level sporting activity ECG Resting bradycardia AV block I High voltage QRS Early repolarisation Anatomy/Function Increased chamber dimensions and LV wall thickness Symmetric LVH Endurance athletes: eccentric LVH Strength-trained athletes: concentric LVH Combination athletes: overlapping features of endurance and strength-trained athletes Max. wall thickness ≤14 mm Black athletes show greater increases in LV wall thickness Normal systolic function Normal mechanical function (strain) Normal diastolic function LGE pattern No LGE or non-specific pattern Follow up LVH↓ after deconditioning

Hypertensive heart disease

History Long-lasting hypertension ECG High voltage QRS LV strain pattern Anatomy/Function Symmetric concentric LVH: Max. wall thickness <15 mm (Caucasian),<15-20 mm (Black) Basal septum hypertrophy Common in elderly and hypertensive patients LVOT obstruction uncommon Normal systolic function – in late stages systolic dysfunction can develop Abnormal mechanical function (strain) Diastolic dysfunction LGE pattern Occasionally patchy LGE pattern or focal (mid-wall) LGE of RV insertion points (hinge points) Follow up LVH↓ under OMT for hypertension

Athlete’s Heart versus HCM

Data feature	Athlete's Heart	HCM
Increased wall thickness	Yes	Yes
Atrial remodeling	Yes	Yes
Systolic function	Normal	Normal
Mechanical function (strain)	Normal	Abnormal
Diastolic physiology	Normal	Abnormal

Aortic stenosis

History Severe aortic stenosis ECG High voltage QRS LV strain pattern Anatomy/Function Symmetric concentric LVH: Degree of hypertrophy associated with the degree of stenosis LVOT obstruction uncommon Normal systolic function LV dilation and heart failure in decompensated state Abnormal mechanical function (strain) Diastolic dysfunction LGE pattern Non-specific LGE pattern: patchy, diffuse or septal mid-wall LGE T1 mapping Native T1↑ ECV↑ Follow-Up LVH↓ and fibrosis↓ after aortic valve replacement

Hypertrophic CMP

History Positive familiy history (50% sarcomeric protein gene mutation) No typical symptoms; in case of LVOT obstruction Palpitations (SV/V arrhythmias) ECG High voltage QRS Narrow Q waves in the lateral and inferior leads Repolarisation abnormalities (“giant inverted T waves” in apical HCM) WPW associated Anatomy/Function LVED wall thickness ≥15 mm (Caucasian), ≥20 mm (Black) Asymmetric HCM: Sigmoidal HCM HCM vs. HOCM (SAM / MR) Apical HCM (Yamaguchi) Apical obliteration of LV cavity Mid-ventricular HCM Apical pouch with LGE Symmetric concentric HCM Possible RV involvement Wall motion abnormalities and abnormal mechanical function (strain↓) of hypertrophied segments Diastolic dysfunction LGE pattern Patchy mid-wall LGE of RV hinge points LGE usually localized to region of hypertrophy; transmural LGE with wall thinning or LGE without hypertrophy are also possible Extent of LGE correlates with risk of SCD T1 mapping Native T1↑, post-contrast T1↓ T1 mapping may detect diffuse fibrosis that is not detected by LGE (subclinical/early stage of HCM) ECV↑ Follow-Up SCD/ICD risk calculator (ESC 2014)

Cardiac amyloidosis

History Multiorgan disease (cardiac involvement associated with poor prognosis) Heart failure ECG Heart block Absence of high voltage QRS (despite LVH) Anatomy/Function Global LV (and RV) hypertrophy Restrictive CMP Small ventricles with preserved systolic function, but reduced longitudinal contraction Severe diastolic dysfunction Severe diastolic dysfunction Enlarged atria Pericardial and pleural effusion Thickened interatrial septal walls (in 20% of cases) LGE pattern Difficulty in nulling myocardial signal Predominantly global subendocardial distribution (non-coronary pattern) Other patterns such as patchy subendocardial or transmural enhancement can also occur On Look-Locker images the blood pool and myocardium cross zero (appear black) at similar timepoints Base-apex LGE gradient Cardiac involvement without any LGE is rare For DD AL vs. ATTR see amyloidosis chapter T1 mapping Native T1↑ Amyloid burden ECV↑↑ (> 0.40) T2 mapping Edema Bright on T2w imaging Follow-Up CMR guided endomyocardial biopsy Chemotherapy in AL amyloidosis Novel TTR-specific treatment (Phase III) in ATTR

Anderson-Fabry disease

History Multi-system lysosomal storage disease X-linked (men > women) Palpitations (SV/V arrhythmias) Acroparesthesia and anhydrosis are common ECG Young age: Short PR (no delta wave) Older age: AV block Prolonged, high voltage QRS Anatomy/Function Concentric LVH Patterns of hypertrophy often indistinguishable from HCM LVH associated with progressive myocardial fibrosis LGE pattern Mid-wall or subepicardial LGE of the inferolateral wall of mid to basal LV (in 50% of cases) T1 mapping Native T1↓ of septum (< 940ms) ECV↓ Inferolateral LGE: pseudonormalisation native T1 normal/↑ Follow-Up Evaluate genetic testing Enzyme replacement therapy (ERT) for α-galactosidase LVH ↓ after ERT

References

Yilmaz A, Sechtem U. Diagnostic approach and differential diagnosis in patients with hypertrophied left ventricles. Heart 2014;100:662-671 Noureldin R, Liu S, Nacif M, et al. The diagnosis of hypertrophic cardiomyopathy by cardiovascular magnetic resonance. JCMR 2012;14:17 Puntmann VO, Voigt T, Chen Z, et al. Native T1 mapping in differentiation of normal myocardium from diffuse disease in hypertrophic and dilated cardiomyopathy. J Am Coll Cardiol Img 2013;6:475-84 Fontana M, Banypersad SM, Treibel TA, et al. Differential myocyte responses in patients with cardiac transthyretin amyloidosis and light-chain amyloidosis. Radiology 2015;21:141744 Chan RH, Maron BJ, Olivotto I, et al. Prognostic value of quantitative contrast-enhanced cardiovascular magnetic resonance for the evaluation of sudden death risk in patients with hypertrophic cardiomyopathy. Circulation 2014;130:484-495 Hoigné P, Attenhofer Jost CH, Duru F, et al. Simple criteria for differentiation of Fabry disease from amyloid heart disease and other causes of left ventricular hypertrophy. Int J Cardiol. 2006 Aug 28;111(3):413-22. Paterick TE, Jan MF, Paterick ZR, et al. Cardiac evaluation of collegiate student athletes: a medical and legal perspective. Am J Med. 2012 Aug;125(8):742-52 Sado DM, White SK, Piechnik SK et al. Identification and assessment of Anderson-Fabry disease by cardiovascular magnetic resonance noncontrast myocardial T1 mapping. Circ Cardiovasc Imaging. 2013 May 1;6(3):392-8.