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Impact of cardiac magnetic resonance on the diagnosis and management of patients with cardiomyopathies

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05. Sept. 2024

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Figure 1

Volumetric assessment of left ventricular (LV) and right ventricular (RV) function in dedicated software. It involves contouring the LV endocardium (in red), epicardium (in green), and RV endocardium (in purple) in a short-axis stack at end-diastole (ED) and end-systole (ES).
Volumetric assessment of left ventricular (LV) and right ventricular (RV) function in dedicated software. It involves contouring the LV endocardium (in red), epicardium (in green), and RV endocardium (in purple) in a short-axis stack at end-diastole (ED) and end-systole (ES).

Figure 2

Cardiac magnetic resonance (CMR) exam of a 65-year-old female addressed for hypertrophic cardiomyopathy assessment, the contrast-enhanced CMR identifies changes susceptible to cardiac amyloidosis. (A) The diastolic phase of a balanced steady-state free precession (bSSFP) cine sequence of the short axis shows predominant septal hypertrophy; the basal antero-septum is 18mm thick, while the other walls are 12mm. (B) LGE acquired in the short axis shows difficult nulling of the myocardium, with areas of transmural hyperenhancement without respecting a coronary artery distribution. (C) T1 mapping imaging identified elevated T1=1100 ms and very high extracellular volume (ECV=48%). (D) T2 mapping imaging revealed diffuse edema, T2 =52-58 ms.
Cardiac magnetic resonance (CMR) exam of a 65-year-old female addressed for hypertrophic cardiomyopathy assessment, the contrast-enhanced CMR identifies changes susceptible to cardiac amyloidosis. (A) The diastolic phase of a balanced steady-state free precession (bSSFP) cine sequence of the short axis shows predominant septal hypertrophy; the basal antero-septum is 18mm thick, while the other walls are 12mm. (B) LGE acquired in the short axis shows difficult nulling of the myocardium, with areas of transmural hyperenhancement without respecting a coronary artery distribution. (C) T1 mapping imaging identified elevated T1=1100 ms and very high extracellular volume (ECV=48%). (D) T2 mapping imaging revealed diffuse edema, T2 =52-58 ms.

Figure 3

Significant clinical impact definition. ICD- implantable cardioverter-defibrillator, EP-electrophysiology, PCI-percutaneous coronary intervention, CABG-coronary artery bypass grafting, CT- computer tomography
Significant clinical impact definition. ICD- implantable cardioverter-defibrillator, EP-electrophysiology, PCI-percutaneous coronary intervention, CABG-coronary artery bypass grafting, CT- computer tomography

Figure 4

The change of diagnosis after CMR examination. DCM- dilated cardiomyopathy, NDLVC non-dilated left ventricular cardiomyopathy, HCM- hypertrophic cardiomyopathy, LVH- left ventricular hypertrophy, ARVC- arrhythmogenic right ventricular cardiomyopathy
The change of diagnosis after CMR examination. DCM- dilated cardiomyopathy, NDLVC non-dilated left ventricular cardiomyopathy, HCM- hypertrophic cardiomyopathy, LVH- left ventricular hypertrophy, ARVC- arrhythmogenic right ventricular cardiomyopathy

Figure 5

(A) Contrast-enhanced cardiovascular magnetic resonance diastolic frame of cine image in 4 chamber view shows a noncompact aspect of the myocardium. (B) The early gadolinium enhancement image in the same four-chamber view highlights apical thrombi at the left ventricle (LV) and right ventricle (RV) levels, indicated by yellow arrows
(A) Contrast-enhanced cardiovascular magnetic resonance diastolic frame of cine image in 4 chamber view shows a noncompact aspect of the myocardium. (B) The early gadolinium enhancement image in the same four-chamber view highlights apical thrombi at the left ventricle (LV) and right ventricle (RV) levels, indicated by yellow arrows

Comparison of CMR’s significant clinical impact in current studies

Study Study period Study patient n Change in diagnostic (%) Change in management (%)
Bruder O et al. [28] 2007-2009 euro CMR registry (German pilot) 11, 040 16.4% 61.8%
Bruder O et al. [20] 2006-2012 SCMR registry 27,301 8.7 % 61 %
Roifman et al. [19] 2013-2019 Patients with HF indications from the SCMR registry 3,837 49% -
Abbasi et al. [21] 2013 Patient with LVEF < 50%. 150 52% 65%
Lin et al. [3] 2004-2017 Patients Undergoing Cardiac Transplantation 338 23 (7%)
Kangala et al. [22] 2017 Patient with HFpEF 154 27%
Witek et al. [29] 2008-2017 Patient with HF of unknown etiology 243 38. 7% 16.9%
Onciul et al. [30] 2018-2020 Patient referred for stress CMR 120 15.85%

CMR criteria for the majority of cardiomyopathy diagnoses

Indication Structural Features (cine-SSFP) LGE Native T1 ECV T2
DCM

-LV dilatation and disfunction

- diffuse regional wall motion abnormalities

- +/_RV dilatation and disfunction

- typical mid-wall pattern -slightly diffuse increased -slightly increased - slightly increased, especially in inflammatory etiologies
NDLVC

-no LV dilatation with LV disfunction

-normal LV systolic function with + LGE

-nonischemic pattern

-normal

-slightly increased

-normal

-slightly increased

-can be increased in inflammatory etiologies
Myocarditis

-none

-regional wall motion abnormalities

-global LV dysfunction

-pericardial effusion

-patchy subepicardial pattern -increased in the oedema zone -increased in the oedema zone -regional or global increased
HCM

-hypertrophy (≥15 mm LV wall thickness): symmetrical, asymmetric, apical

- other anomalies: LV crypts, papillar hypertrophy, SAM

-mid-wall, usually in the areas where the wall is the thickest

-insertion points of the septum to the RV level

-increased -increased

-normal

-slightly increased in the burnout type

ARVC

-RV dilatation, dysfunction,

-akinetic, dyskinetic aneurysms at the RV level, often affecting the RV triangle: sub tricuspid area, RV ejection tract, and RV apex

-LV systolic dysfunction, regional wall motion abnormality

- transmural RV region(s) (inlet, outlet, and apex)

- subepicardial or mid-wall at LV lateral/inferolateral, septum, or both

-subepicardial circumferential infiltration in those with predominantly LV damage

-decreased in fat infiltration

-increased in scar region

-non-specific - can be increased at the level of LGE in acute phases
Cardiac amyloidosis

-concentric hypertrophy

-IAS, LA wall thickening

- LV dysfunction

-pericardial effusion

-diffuse subendocardial

-nulling effect of the myocardium

-dark blood aspect

-diffuse increased -very increased

-non-specific pattern

- can be increased in those with AL form

Cardiac sarcoidosis

-global systolic dysfunction

-regional or global wall motion abnormalities,

-bi-ventricular dilatation or hypertrophy.

-slight increase in left ventricular mass secondary to granulomatous expansion

-mid-wall or sub-epicardial focal fibrosis,

-transmural or subendocardial, but without a correlation to a coronary territory

-papillary muscles, RV-free wall, and the atria can also be involved.

-increased in the scar zone - increased diffuse -increased in areas with granulomas
LV non-compaction/hypertrabeculation

-noncompacted-to-compacted myocardium ratio of 2.3:1

-trabeculated mass > 20%

- LV disfunction/dilatation

-non-specific pattern

-most frecvent subepicardial

-slightly increase -increased diffuse -normal
Anderson-Fabry disease -concentric important LV hypertrophy

-mid-wall inferolateral

-mid-wall septal

-decreased

- pseudonormal

-decreased -slightly increased, basal anteroseptal
Neuromuscular dystrophy

-normal

-LV dysfunction

-regional wall motion defects

-focal fibrosis predominantly at the level of the lateral and inferolateral walls -increased diffuse -increased diffuse -hypersignal at the LGE level

CMR parameters_ Predictor of significant clinical impact-univariable analysis

Predictor N Event N OR (95% CI)1 p-value
LVEF 272 180 0.97 (0.95 to 0.99) 0.002
LVEDVi 272 180 1.01 (1.00 to 1.01) 0.067
LVESVi 272 180 1.01 (1.00 to 1.02) 0.012
LVSVi 272 180 0.98 (0.96 to 1.00) 0.026
RVEF 265 175 0.97 (0.95 to 0.99) 0.002
LGE 177 113 1.84 (1.11 to 3.07) 0.019
Native septal T1 243 157 1.00 (1.00 to 1.01) 0.507
T2 254 167 0.99 (0.93 to 1.01) 0.387

Demographic Parameters_ Predictor of significant clinical impact - univariable analysis

Predictor N Event N OR (95% CI)1 p-value
Age 272 180 1.00 (0.99 to 1.02) 0.733
Gender (M) 179 121 1.20 (0.71 to 2.03) 0.492
BSA 272 180 1.22 (0.53 to 2.96) 0.643
Coronary angiography 57 41 1.39 (0.74 to 2.71) 0.312
Hypertension 151 102 1.16 (0.70 to 1.93) 0.559
Hypercholesterolemia 140 95 1.18 (0.71 to 1.96) 0.517
Alcohol 12 10 2.66 (0.68 to 17.6) 0.212
Tabacco use 40 28 1.24 (0.61 to 2.64) 0.568
NYHA class
1 29 19
2 176 110 0.88 (0.37 to 1.96) 0.755
3 & 4 36 29 2.18 (0.72 to 6.97) 0.175

CMR parameters

Cardiac dimensions and function
LVEDV, ml 224 (±100)
LVEDVi, ml/m2 113(± 47)
LVESV, ml 129 (±92)
LVESVi, ml/m2 64 (±43)
LVSV 92 (±58)
LVEF, % 46 (±15)
LV mass i, g/m2 68(±27)
RVEDV, ml 168 (±58)
RVEDVi, ml/m2 81 (±29)
RVESV, ml 89 (±27)
RVESVi, ml/m2 43 (±26)
RVEF% 55 (±11)
Tissue characterization
Oedema n, (%) 15, (4,2%)
Native T1 (ms) 1030(±50)
T2 (ms) 49 (±19)
Characterization of focal scars
Scar present n, (%) 177 (65%)
Non-ischaemic scar n, (%) 164 (92%)
Ischaemic scar n, (%) 13 (7.3%)
Number of scars (%)

· 1 scar

· 2 scars

· 3 scars

· >3 scars

· 91 (51.4%)

· 38 (21.4%)

· 14 (7.9%)

· 34 (19.2%)

Baseline characteristics

Baseline characteristics
Age, years 49 (±14)
Sex (male), % 65%
Body surface area, m2 1.99 (±0.2)
Family history of cardiomyopathy 4%
NYHA Class
I 21%
II 65%
III 13%
IV 1%
Coronary artery risk factors
Hypertension, % 55%
Diabetes mellitus, % 3%
Hypercholesterolemia, % 51%
Smoking % 15%
Alcohol consumption, % 4.5%
Comorbidities
History of myocardial infarction, % 0.5%
Aortocoronary bypass operation, % 0%
PCI, % 0.5%
CKD, % 5%
Electrocardiogram
Sinus Rhythm, % 93%
Atrial Fibrillation, % 7%
Complete LBBB, % 12%
Frequent PVC, % 7%
Complications 0.5%
Cardiac tests before CMR
Echocardiography 100%
Coronary angiogram CT 1%
Coronary angiography 20%
SPECT 1%
Treadmill test 2%
The primary indication for CMR
DCM/ NDLVC 45%
HCM/ LVH 18%
Cardiac amyloidosis 5.2%
ARVC 4 %
Myocarditis 3.6%
Cardiac sarcoidosis 1%
LV non-compaction/hypertrabeculation 2%
Anderson-Fabry disease 2%
Tachycardia-induced cardiomyopathy 1%
Chemotherapy induces cardiomyopathy 0.5%
Neuromuscular cardiomyopathy 0.5%
Arrhythmia/SND substrate 2.5%
Ischemic cardiomyopathy 1%
Other cardiomyopathy 10%

CMR parameters_ Multiple univariate binominal logistic regression

Predictor N SCI N OR (95% CI) 1 p-value VIF1
LVESVi 272 180 1.01 (1.005 to 1.02) 0.021 1.0
LGE 177 113 1.72 (1.03 to 2.89) 0.038
Sprache:
Englisch
Zeitrahmen der Veröffentlichung:
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Fachgebiete der Zeitschrift:
Medizin, Klinische Medizin, Allgemeinmedizin, Innere Medizin, Kardiologie, Chirurgie, Herzchirurgie, Kinder- und Jugendmedizin, Kinderkardiologie