Differentiation HCMP / Amyloidosis / athletes heart / hypertension is possible — pattern recognition
Rules to remember for normal vs pathological
A certain basal to apical gradient is visible in all forms of myocardial wall thickening & even in healthy individuals
Basal/apical gradient — Laplace-Law— basal more wall stress (larger radius) + apical more matrix formation of the myocytes ➜ less wall stress
LA Strain as a differentiation for myocardial wall thickening ➜ storage disease/restrictive CMP with the lowest values ➜ hypertensive heart disease with an LASr >20%, amyloidosis <20% ➜ in HCMP, a reduction of all 3 LA Strain phases is possible
PSI mean values ➜ HCMP (6%), Amyloidosis (7%), around 2% in healthy individuals
H(O)CMP
HCM GLS mean value of -11,7% vs. Hypertensive heart disease "low-normal" -17,8%
Hypertrophied segments in HCM around -12%, also non-hypertrophied show reduction
Mortality increases in Strain >-16%; with a GLS >-10% 10-year-mortality >75% (H(O)CMP)
GLS more positive than -10% ➜ increased probability for ventricular arrhythmias in H(O)CMP
H(O)CMP — all morphological LV forms are possible
Strain reduction in HCMP, like LGE in MRI (detection of end-stage fibrosis)
Subclinical & early fibrosis detection (no LGE in MRI)
LA Strain values improve with Mavacamten (LASr (baseline 20,5%) 2,8% in 56 weeks, LASct (baseline -8,7%) -1,2%) ➜ reverse remodelling of the LA
Significant improvement of the GLS in HOCMP in reversible myosin inhibitors (especially in the areas with the most significant reduction)
LASr as a predictor in HCM for ≤6METS (cut-off ≤8% — Limitation 3D LA Strain)
Apical HCMP with a reduction in the hypertrophied segments & potentially "Blueberry on top" ➜ "early" time to peak Strain sometimes visible in the apical hypertrophied segments
Sun 2019; Alfonso 2012; Liu 2017; Saio et al., PLOS ONE 2022; Altersberger, Genger 2023
Cut-offs to remember
Amyloid heart disease
Gradient & apical sparing in Amyloid heart disease in all forms visible (AL, ATTR); Amyloid heart disease ➜ Cherry on Top = apical sparing
All strain parameters have prognostic information for amyloid heart disease (LV, RV, LA, RA Strain). LASr is the strongest parameter. Thicker walls ➜ worse Strain (GLS -14,8% with IVS 16mm vs. GLS -8% with IVS 18mm ➜ FWS -20,7% vs. -13,4% ➜ LASr 23,4% vs. 9,1%; RASr 22,9% vs. 8,1%)
In LASr 13,4% survival of 25% in 4a ➜ the worse the strain, the more survival is reduced
Relative apical sparing (RAS) can be calculated in the 17-segment model; RAS ≥1 = (Mean value 5 apical segments / (Mean value 6 basal + 6 midventricular segments)
Early forms ➜ often basal to apical gradient with GLS >-15% ➜ use LA Strain as well (LASr <20% in restrictive CMP before restrictive filling)
Stabilisation in TTR stabilizers therapy of GLS -10,6% vs -11,3% in 1 year follow-up in SR ➜ LASr improvement was significant 10,5% vs 11,9% in 1 year follow-up; reduction over time in all parameters in AFIB; untreated GLS reduction is -1% per year; -10,4% associated as cut-off for poor outcomes
RV GLS >-17% as risk marker (2,8 fold risk of CD death)
Morbus Anderson-Fabry
Strain reduction in ≥1 Segment also without myocardial wall thickening; inferolateral (basal, medial) and lateral strain reduction
GLS reduction ➜ progress
Improvement of LASr ➜ successful therapy (GLS does not change)
Hypertension (CATEGORY like Fabry, Amyoid heart disease)
Borderline normal Strain with -17,8% ➜ Tip: GLS -16%, think about HFpEF, in a relevant basal/apical gradient, think about amyloid heart disease
Others
Diffusely reduced strain in mitochondrial disease
Endurance athletes
IVS >11mm & GLS ≥-18% 79% specificity for pathological LVH in normal EF ➜ Tip: in athletes perform handgrip or similar (easy) stresstest in unclear and borderline GLS (quick improvement during exercise); be aware of structural heart disease in low or low-normal GLS
HCM
Mean GLS -11,7%, prognosis (and arrhythmias) worse ➜ >-16%; severely reduced prognosis in GLS >-10% = Tip: also segments without LVH can be diseased (subclinically) and show a reduced Strain ➜ no LGE in MRI (beginning fibrosis), strain otherwise marker for LGE in MRI
Amyloid heart disease
LASr <20%, HTN >20%, lowest values in restrictive CMPs; relative apical sparing ≥1 or GLS / EF >4,1 amyloid heart disease possible ➜ Tip: in early stages and in unclear strain reduction (with clear basal to apical gradient) think about it (GLS >-16%, IVS 11-12mm, LASr reduction with relatively preserved GLS)
