Characterization of hypertrophic cardiomyopathy according to global, regional, and multi-layer longitudinal strain analysis, and prediction of sudden cardiac death

2018 ◽  
Vol 34 (7) ◽  
pp. 1091-1098 ◽  
Author(s):  
Marie-Philippe Vergé ◽  
Hubert Cochet ◽  
Amélie Reynaud ◽  
Lucas Morlon ◽  
Jérôme Peyrou ◽  
...  
Author(s):  
Hyun-Jung Lee ◽  
Hyung-Kwan Kim ◽  
Sang Chol Lee ◽  
Jihoon Kim ◽  
Jun-Bean Park ◽  
...  

Abstract Aims We investigated the prognostic role of left ventricular global longitudinal strain (LV-GLS) and its incremental value to established risk models for predicting sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathy (HCM). Methods and results LV-GLS was measured with vendor-independent software at a core laboratory in a cohort of 835 patients with HCM (aged 56.3 ± 12.2 years) followed-up for a median of 6.4 years. The primary endpoint was SCD events, including appropriate defibrillator therapy, within 5 years after the initial evaluation. The secondary endpoint was a composite of SCD events, heart failure admission, heart transplantation, and all-cause mortality. Twenty (2.4%) and 85 (10.2%) patients experienced the primary and secondary endpoints, respectively. Lower absolute LV-GLS quartiles, especially those worse than the median (−15.0%), were associated with progressively higher SCD event rates (P = 0.004). LV-GLS was associated with an increased risk for the primary endpoint, independent of the LV ejection fraction, apical aneurysm, and 2014 European Society of Cardiology (ESC) risk score [adjusted hazard ratio (aHR) 1.14, 95% confidence interval (CI) 1.02–1.28] or 2011 American College of Cardiology/American Heart Association (ACC/AHA) risk factors (aHR 1.18, 95% CI 1.05–1.32). LV-GLS was also associated with a higher risk for the composite secondary endpoint (aHR 1.06, 95% CI 1.01–1.12). The addition of LV-GLS enhanced the performance of the ESC risk score (C-statistic 0.756 vs. 0.842, P = 0.007) and the 2011 ACC/AHA risk factor strategy (C-statistic 0.743 vs. 0.814, P = 0.007) for predicting SCD. Conclusion LV-GLS is an important prognosticator in patients with HCM and provides additional information to established risk stratification strategies for predicting SCD.


2021 ◽  
Vol 22 (Supplement_2) ◽  
Author(s):  
P Martinez Vives ◽  
A Cecconi ◽  
A Vera ◽  
T Nogales-Romo ◽  
B Lopez-Melgar ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Hypertrophic cardiomyopathy (HCM) is a relatively prevalent condition associated with arrhythmic events and sudden cardiac death. Several tools are currently available to identify which HCM patients are at risk of developing these events. Purpose We aimed to evaluate the association of Tissue Tracking strain analysis by cardiac magnetic resonance (CMR) and the development of arrhythmic events in patients with HCM. Methods We prospectively analyzed 136 consecutive patients with HCM diagnosis (established according to current clinical practice guidelines) from January 2006 to October 2017. Every routine 24 hours ECG-monitoring test was registered and looked for sustained or non-sustained ventricular tachycardia (any VT). CMR studies were performed following our predefined CMR protocol for HCM with 1.5T magnets. Cine images were obtained with standard, retrospectively gated, steady-state free-precession (SSFP) sequences in 2, 3 and 4 chambers views and in 10–15 contiguous short-axis slices covering the ventricles from the base to the apex, with breath holding. The strain evaluation was performed by a commercially available Tissue Tracking analysis software, manually defining the endocardial border in short axis, 4, 3 and 2 chambers views and, after verifying adequate identification of the different structures, running the strain analysis (Figure 1, displaying myocardium identification by the strain analysis software). Results Mean follow-up was 49 ± 45 months. Mean age was 61 ± 15 years old (p for the comparation between the group with arrhythmic and the group without arrhythmic events 0.212) and 31% of patients were women (p 0.420). Mean ejection fraction was 69 ± 9.21% (p 0.223) and mean HCM-SCD (hypertrophic cardiomyopathy sudden cardiac death) risk score was 2.20 ± 1.34 (p <0.001). Median percentage of total myocardium showing late gadolinium enhancement (LGE) was 0.61 (interquartile range 2.9; p 0.170). Mean global radial strain (GRS) was 26.23 ± 8.78% (p <0.001). 21 VT episodes were recorded during follow-up. GRS showed an area under de ROC curve of 0.75 predicting VT during follow-up, selecting the value of 27% as the best sensitivity/specificity cutoff point. Statistically significant differences were not found when analyzing global circumferential strain (GCS) and global longitudinal strain (GLS) as VT predictors after adjusting for possible confusion factors (GRS, GCS and GLS distributions depicted in Figure 2). A binary GRS ≥27%/<27% variable was included in a logistic regression model adjusted by age, percent of total myocardium mass showing LGE and HCM-SCD risk score. Significantly more arrhythmic events were found to occur in patients with a GRS <27% (OR 7.33; 95% confidence interval 1.07 – 50.41; p 0.043) after adjusting by age, percent of total myocardium mass showing LGE, and HCM-SCD risk score Conclusions A GRS value of <27% on CMR appears to be a good predictor of worse arrhythmic prognosis in patients with HCM.


Heart ◽  
2020 ◽  
pp. heartjnl-2020-317701
Author(s):  
Guixin Wu ◽  
Jie Liu ◽  
Shuiyun Wang ◽  
Shiqin Yu ◽  
Ce Zhang ◽  
...  

ObjectiveElevated levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) are associated with heart failure-related death in hypertrophic cardiomyopathy (HCM), but the relationship between NT-proBNP level and sudden cardiac death (SCD) in HCM remains undefined.MethodsThe study prospectively enrolled 977 unrelated patients with HCM with available NT-proBNP results who were prospectively enrolled and followed for 3.0±2.1 years. The Harrell’s C-statistic under the receiver operating characteristic curve was calculated to evaluate discrimination performance. A combination model was constructed by adding NT-proBNP tertiles to the HCM Risk-SCD model. The correlation between log NT-proBNP level and cardiac fibrosis as measured by late gadolinium enhancement (LGE) or Masson’s staining was analysed.ResultsDuring follow-up, 29 patients had SCD. Increased log NT-proBNP levels were associated with an increased risk of SCD events (adjusted HR 22.27, 95% CI 10.93 to 65.63, p<0.001). The C-statistic of NT-proBNP in predicting SCD events was 0.80 (p<0.001). The combined model significantly improved the predictive efficiency of the HCM Risk-SCD model from 0.72 to 0.81 (p<0.05), with a relative integrated discrimination improvement of 0.002 (p<0.001) and net reclassification improvement of 0.67 (p<0.001). Furthermore, log NT-proBNP levels were significantly correlated with cardiac fibrosis as detected either by LGE (r=0.257, p<0.001) or by Masson’s trichrome staining in the myocardium (r=0.198, p<0.05).ConclusionNT-proBNP is an independent predictor of SCD in patients with HCM and may help with risk stratification of this disease.


2013 ◽  
Vol 34 (suppl 1) ◽  
pp. P2954-P2954
Author(s):  
P. Debonnaire ◽  
S. Katsanos ◽  
E. Joyce ◽  
O. V. W. Van Den Brinck ◽  
D. E. Atsma ◽  
...  

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Lars Grosse-Wortmann ◽  
Laurine van der Wal ◽  
Aswathy Vaikom House ◽  
Lee Benson ◽  
Raymond Chan

Introduction: Cardiovascular magnetic resonance (CMR) with late gadolinium enhancement (LGE) has been shown to be an independent predictor of sudden cardiac death (SCD) in adults with hypertrophic cardiomyopathy (HCM). The clinical significance of LGE in pediatric HCM patients is unknown. Hypothesis: LGE improves the SCD risk prediction in children with HCM. Methods: We retrospectively analyzed the CMR images and reviewed the outcomes pediatric HCM patients. Results: Amongst the 720 patients from 30 centers, 73% were male, with a mean age of 14.2±4.8 years. During a mean follow up of 2.6±2.7 years (range 0-14.8 years), 34 experienced an episode of SCD or equivalent. LGE (Figure 1A) was present in 34%, with a mean burden of 14±21g, or 2.5±8.2g/m2 (6.2±7.7% of LV myocardium). The presence of ≥1 adult traditional risk factor (family history of SCD, syncope, LV thickness >30mm, non-sustained ventricular tachycardia on Holter) was associated with an increased risk of SCD (HR=4.6, p<0.0001). The HCM Risk-Kids score predicted SCD (p=0.002). The presence of LGE was strongly associated with an increased risk (HR=3.8, p=0.0003), even after adjusting for traditional risk factors (HR adj =3.2, p=0.003) or the HCM Risk-Kids score (HR adj =3.5, p=0.003). Furthermore, the burden of LGE was associated with increased risk (HR=2.1/10% LGE, p<0.0001). LGE burden remained independently associated with an increased risk for SCD after adjusting for traditional risk factors (HRadj=1.5/10% LGE, p=0.04) or HCM Risk-Kids (HRadj=1.9/10% LGE, p=0.0018, Figure 1B). The addition of LGE burden improved the predictive model using traditional risk markers (C statistic 0.67 vs 0.77, p=0.003) and HCM Risk-Kids (C statistic 0.68 vs 0.74, p=0.045). Conclusions: Quantitative LGE is an independent risk factor for SCD in pediatric patients with HCM and improves the performance of traditional risk markers and the HCM Risk-Kids Score for SCD risk stratification in this population.


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