Five-year prognostic significance of global longitudinal strain in individuals with a hypertrophic cardiomyopathy gene mutation without hypertrophic changes

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.

Download Full-text

Global Longitudinal Strain and Left Atrial Volume Index Provide Incremental Prognostic Value in Patients With Hypertrophic Cardiomyopathy

Circulation Cardiovascular Imaging ◽

10.1161/circimaging.116.005706 ◽

2017 ◽

Vol 10 (7) ◽

Cited By ~ 21

Author(s):

Yasmine L. Hiemstra ◽

Philippe Debonnaire, ◽

Marianne Bootsma ◽

Erik W. van Zwet, ◽

Victoria Delgado, ◽

...

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Left Atrial ◽

Prognostic Value ◽

Longitudinal Strain ◽

Global Longitudinal Strain ◽

Left Atrial Volume ◽

Volume Index ◽

Atrial Volume ◽

Left Atrial Volume Index ◽

Incremental Prognostic Value

Download Full-text

P1594 Relationship between left ventricular morphology and systolic performance and coronary microcirculatory dysfunction in hypertrophic cardiomyopathy

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jez319.1012 ◽

2020 ◽

Vol 21 (Supplement_1) ◽

Author(s):

S Aguiar Rosa ◽

L Branco ◽

A Galrinho ◽

A Fiarresga ◽

L Lopes ◽

...

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Myocardial Ischemia ◽

Coronary Flow ◽

Longitudinal Strain ◽

Global Longitudinal Strain ◽

Left Ventricular ◽

Coronary Flow Velocity ◽

Microcirculatory Dysfunction ◽

Maximal Wall Thickness ◽

Systolic Performance

Abstract Background Myocardial ischemia constitutes one of the most important pathophysiological features in hypertrophic cardiomyopathy (HCM). Chronic and recurrent myocardial ischemia leads to fibrosis, which may culminate in myocardial dysfunction. Objective To analyse the relationship between left ventricular (LV) morphology and systolic performance and coronary microcirculatory dysfunction in HCM. Methods The present study prospectively included HCM patients (P) who underwent transthoracic echocardiography. Left ventricular (LV) function was evaluated by ejection fraction (LVEF), global longitudinal strain (GLS) and tissue Doppler septal and lateral s’. The evaluation of coronary flow velocity reserve (CFVR) was performed in apical three chambers view for the left anterior descending (LAD) artery and in an apical three chambers view for the posterior descending (PD) artery. Diastolic coronary flow velocity was measured in basal conditions and in hyperemia, induced by adenosine perfusion (0.14 mg/kg/min intravenously, during 2 minutes). Absolute CFVR was calculated as the ratio of hyperemic to basal peak diastolic flow velocities; relative CFVR was calculated as the ratio between CFVR LAD and CFVR PD. Results 23 P were enrolled (57% male, mean age 57.9 ± 13.7 years). Asymmetric septal hypertrophy was verified in 70% of P, with maximal wall thickness of 21.6 ± 4.3mm. Obstructive HCM was documented in 35% of patients. CFV was successfully measured in the LAD in all patients, but only in 70% of patients in the PD due to technical issues related to poor acoustic window and anatomical constraints. 78% of P (n = 18) presented CFVR <2, denoting microcirculatory dysfunction. Relative CFVR (LAD CFVR/ PD CFVR) was ≥1 in 43% of P. P with maximal wall thickness (MWT)>20mm presented higher CFV PD at baseline (46.5 ± 17.4 vs 32.5 ± 12.6 cm/s; p = 0.072), lower CFVR PD (1.3 ± 0.3 vs 2.5 ± 0.8; p = 0.003) and greater regional difference of microcirculation (relative CFVR 1.4 ± 0.6 vs 0.8 ± 0.3; p = 0.048). At baseline conditions, CFV LAD was higher in obstructive HCM (44.0 ± 4.8 vs 35.3 ± 10.6 cm/s; p = 0.040). P with impairment in global longitudinal strain (GLS>-18%) had higher basal CFV LAD (40.1 ± 8.6 vs 30.0 ± 12.2 cm/s; p = 0.059) and PD (44.5 ± 15.2 vs 20.0 ± 5.0 cm/s; p = 0.015) but lower CFVR PD (1.5 ± 0.5 vs 2.8 ± 1.1; p = 0.039). The reduction in CFVR PD was also noted in P with time to peak longitudinal strain dispersion >90mseg (CFVR PD 1.2 ± 0.2vs1.9 ± 0.9;p = 0.012). Conclusion Higher CFV at baseline was noted in P with greater MWT, obstructive HCM and worse GLS. Coronary microcirculatory dysfunction was associated with the degree of LV hypertrophy and impairment in LV systolic performance.

Download Full-text

P1494Usefulness of longitudinal strain adjusted to regional thickness in hypertrophic cardiomyopathy

European Heart Journal ◽

10.1093/eurheartj/ehz748.0258 ◽

2019 ◽

Vol 40 (Supplement_1) ◽

Author(s):

N Mansencal ◽

S Utado ◽

M Hauguelf-Moreau ◽

S Mallet ◽

P Charron ◽

...

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Longitudinal Strain ◽

Global Longitudinal Strain ◽

Strain Analysis ◽

Region Of Interest ◽

Left Ventricular ◽

Control Group ◽

Myocardial Wall ◽

Regional Strain ◽

Standard Software

Abstract Background In hypertrophic cardiomyopathy (HCM), longitudinal strain analysis allows to early detect left ventricular (LV) contraction abnormalities despite preserved LV ejection fraction. In current software, the width of the region of interest (ROI) is the same over the entire myocardial wall, and might analyze partially LV hypertrophic segments. Purpose The aim of this study is to evaluate a novel software for strain analysis with an adjustable ROI according to each segment thickness. Methods We included 110 patients: 55 patients with HCM (HCM group) and 55 healthy subjects (age- and sex-matched control group). All patients underwent echocardiography using a Vivid 9 GE system and measurements were performed using EchoPAC software. Global longitudinal strain (GLS) and regional strain for each of the 17 segments was calculated with standard software (for 2 groups) and with software adjusted to the myocardial wall thickness (for HCM group). Results GLS was significantly decreased in the HCM group as compared to the control group (−15.1±4.8% versus −20.5±4.3%, p<0.0001). In HCM group, GLS (standard method versus adjusted to thickness) were not significantly different (p=0.34). Interestingly, regional strain adjusted to thickness was significantly lower than standard strain in hypertrophic segments, especially in basal inferoseptal segment (p=0.0002), median inferoseptal segment (p<0.001) and median anteroseptal segment (p=0.02). Strain adjusted to thickness was still significantly lower in the most hypertrophic segments (≥20 mm) (−3.7±3%, versus −5.9±4.4%, p=0.049 in the basal inferoseptal segment and −5.7±3.5% versus −8.3±4.5%, p=0.0007 in the median inferoseptal segment). Analysis of strain adjusted to thickness had a better feasibility (97.5% versus 99%, p=0.01). Conclusion Analysis of longitudinal strain adjusted to regional thickness is feasible in HCM and allows a better evaluation of myocardial deformation, especially in the most LV hypertrophic segments.

Download Full-text

Prognostic significance of longitudinal strain in dilated cardiomyopathy with recovered ejection fraction

Heart ◽

10.1136/heartjnl-2021-319504 ◽

2021 ◽

pp. heartjnl-2021-319504

Author(s):

Marco Merlo ◽

Marco Masè ◽

Andrew Perry ◽

Eluisa La Franca ◽

Elena Deych ◽

...

Keyword(s):

Ejection Fraction ◽

Dilated Cardiomyopathy ◽

Longitudinal Strain ◽

Global Longitudinal Strain ◽

Prognostic Significance ◽

Left Ventricular ◽

Left Ventricular Ejection ◽

Ventricular Ejection Fraction ◽

Risk Of Death

ObjectivePatients with non-ischaemic dilated cardiomyopathy (NICM) may experience a normalisation in left ventricular ejection fraction (LVEF). Although this correlates with improved prognosis, it does not correspond to a normalisation in the risk of death during follow-up. Currently, there are no tools to risk stratify this population. We tested the hypothesis that absolute global longitudinal strain (aGLS) is associated with mortality in patients with NICM and recovered ejection fraction (LVEF).MethodsWe designed a retrospective, international, longitudinal cohort study enrolling patients with NICM with LVEF <40% improved to the normal range (>50%). We studied the relationship between aGLS measured at the time of the first recording of a normalised LVEF and all-cause mortality during follow-up. We considered aGLS >18% as normal and aGLS ≥16% as of potential prognostic value.Results206 patients met inclusion criteria. Median age was 53.5 years (IQR 44.3–62.8) and 56.6% were males. LVEF at diagnosis was 32.0% (IQR 24.0–38.8). LVEF at the time of recovery was 55.0% (IQR 51.7–60.0). aGLS at the time of LVEF recovery was 13.6%±3.9%. 166 (80%) and 141 (68%) patients had aGLS ≤18% and <16%, respectively. During a follow-up of 5.5±2.8 years, 35 patients (17%) died. aGLS at the time of first recording of a recovered LVEF correlated with mortality during follow-up (HR 0.90, 95% CI 0.91 to 0.99, p=0.048 in adjusted Cox model). No deaths were observed in patients with normal aGLS (>18%). In unadjusted Kaplan-Meier survival analysis, aGLS <16% was associated with higher mortality during follow-up (31 deaths (22%) in patients with GLS <16% vs 4 deaths (6.2%) in patients with GLS ≥16%, HR 3.2, 95% CI 1.1 to 9, p=0.03).ConclusionsIn patients with NICM and normalised LVEF, an impaired aGLS at the time of LVEF recovery is frequent and associated with worse outcomes.

Download Full-text