How do hypertrophic cardiomyopathy mutations affect myocardial function in carriers with normal wall thickness? Assessment with cardiovascular magnetic resonance

Background Administration of gadolinium-contrast can cause problems in cardiac amyloidosis (CA) patients with impaired renal function. Purpose To compare patterns of cardiovascular magnetic resonance tissue tracking (CMR-TT) for CA and hypertrophic cardiomyopathy (HCM) and to assess the feasibility of CMR-TT to distinguish between these diseases without administration of gadolinium-contrast. Material and Methods Included were 54 patients with biopsy-proven CA, 40 patients with HCM, and 30 healthy people. We calculated strain ratio of apex to base (SRAB) in the left ventricle (LV) using radial (R), circumferential (C), and longitudinal (L) strain from CMR-TT. The LV ejection fraction (LVEF) and the ratio of septal to posterior wall at basal level were also calculated. Late gadolinium enhancement (LGE) image analysis was performed for differential diagnosis. Area under the receiver operating characteristic curve (AUC) comparisons were used. Results All SRAB values were significantly different between CA and HCM (all P < 0.001). AUC values for parameters were 0.806 for LVEF, 0.815 for ratio of wall thickness, 0.944 for the LGE pattern, 0.898 for SRABR, 0.880 for SRABC, and 0.805 for SRABL. AUCs for the LGE pattern were significantly higher than for LVEF, ratio of wall thickness and SRABL (all P < 0.008). No significant differences were seen between AUCs for the LGE pattern, SRABR, and SRABC (all P > 0.109). Conclusion SRABR and SRABC were reliable parameters for distinguishing between CA and HCM.

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Patients who do not fulfill criteria for hypertrophic cardiomyopathy but have unexplained giant T-wave inversion: a cardiovascular magnetic resonance mid-term follow-up study

Journal of Cardiovascular Magnetic Resonance ◽

10.1186/s12968-020-00700-5 ◽

2021 ◽

Vol 23 (1) ◽

Author(s):

Shuang Li ◽

Jian He ◽

Jing Xu ◽

Baiyan Zhuang ◽

Bailing Wu ◽

...

Keyword(s):

Cardiovascular Magnetic Resonance ◽

Hypertrophic Cardiomyopathy ◽

Magnetic Resonance ◽

Wall Thickness ◽

Receiver Operating Curve ◽

T Wave ◽

Wave Inversion ◽

The Mean ◽

Over Time

Abstract Background Patients who have unexplained giant T-wave inversions but do not meet criteria for hypertrophic cardiomyopathy (HCM) (left ventricular (LV) wall thickness < 1.5 cm) demonstrate LV apical morphological features that differ from healthy subjects. Currently, it remains unknown how the abnormal LV apical morphology in this patient population changes over time. The purpose of this study was to investigate LV morphological and functional changes in these patients using a mid-term cardiovascular magnetic resonance (CMR) exam. Methods Seventy-one patients with unexplained giant T-wave inversion who did not fulfill HCM criteria were studied. The mean interval time of the follow-up CMR was 24.4 ± 8.3 months. The LV wall thickness was measured in each LV segment according to the American Heart Association 17-segmented model. The apical angle (ApA) was also measured. A receiver operating curve (ROC) was used to identify the predictive values of the CMR variables. Results Of 71 patients, 16 (22.5%) progressed to typical apical HCM, while 55 (77.5%) did not progress to HCM criteria. The mean apical wall thickness was significantly different between the two groups at both baseline and follow-up, with the apical HCM group having greater wall thickness at both time points (all p < 0.001). There was a significant difference between the two groups in the change of ApA (− 1.5 ± 2.7°/yr vs. − 0.7 ± 2.0°/yr, p < 0.001) over time. The combination of mean apical wall thickness and ApA proved to be the best predictor for fulfilling criteria for apical HCM with a threshold value of 8.1 mm and 90° (sensitivity 93.8%, specificity 85.5%). Conclusions CMR metrics identify predictors for progression to HCM in patients with unexplained giant T-wave inversion.

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