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Published on: August 8, 2022
Late Gadolinium Enhancement in Patients With Hypertrophic Cardiomyopathy and Preserved Systolic Function
Amgad Mentias1, Pejman Raeisi-Giglou1, Nicholas G Smedira1
1Hypertrophic Cardiomyopathy Center, Heart and Vascular Institute, Cleveland Clinic, Cleveland, Ohio.
Insights
Late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) provides significant prognostic value in hypertrophic cardiomyopathy (HCM) patients. This finding aids in risk stratification for sudden cardiac death (SCD) and appropriate implantable cardioverter-defibrillator discharge.
Area of Science:
- Cardiology
- Medical Imaging
- Genetics
Background:
- Hypertrophic cardiomyopathy (HCM) frequently exhibits late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) imaging.
- LGE indicates myocardial scarring and fibrosis, which are implicated in adverse outcomes.
Purpose of the Study:
- To evaluate the additional prognostic information provided by LGE in patients diagnosed with HCM.
- To assess the association of LGE with adverse cardiovascular events in different HCM subgroups.
Main Methods:
- A cohort of 1,423 low- to intermediate-risk adult HCM patients with preserved ejection fraction were analyzed.
- Transthoracic echocardiography (TTE) and CMR with LGE quantification were performed.
- The primary endpoint was a composite of sudden cardiac death (SCD) and appropriate implantable cardioverter-defibrillator (ICD) discharge over a 5-year period.
Main Results:
- Fifty percent of patients showed LGE on CMR, with a median of 8.4%.
- LGE ≥15% was associated with an increased risk of composite events.
- In obstructive HCM, ≥15% LGE increased event rates, while myectomy reduced them.
- LGE improved risk prediction models, demonstrating incremental prognostic utility.
Conclusions:
- In low-/intermediate-risk HCM patients, LGE provides significant incremental prognostic value for adverse cardiovascular events.
- LGE assessment is crucial for risk stratification in obstructive, nonobstructive, and post-myectomy HCM subgroups.
- These findings support the integration of LGE into clinical risk assessment protocols for HCM.
Background:
A high proportion of patients with hypertrophic cardiomyopathy (HCM) have evidence of late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR).
Objectives:
This study sought to assess the incremental prognostic utility of LGE in patients with HCM.
Methods:
We studied 1,423 consecutive low-/intermediate-risk patients with HCM (age ≥18 years) with preserved left ventricular (LV) ejection fraction (mean age 66 ± 14 years, 60% men) who underwent transthoracic echocardiography (TTE) (including dimensions and LV outflow tract gradients) and CMR (including LGE as a % of LV mass) at our center between January 2008 and December 2015. The primary composite endpoint was sudden cardiac death (SCD) and appropriate implantable cardioverter-defibrillator discharge. The percent 5-year SCD risk score was calculated.
Results:
The mean 5-year SCD risk score was 2.3 ± 2.0. Mean maximal LV outflow tract gradient (TTE) was 70 ± 55 mm Hg (median 74 mm Hg [interquartile range (IQR): 10 to 67 mm Hg]); indexed LV mass and LGE (both on CMR) were 91 ± 10 g/m2 and 8.4 ± 12% (IQR: 0% to 19%); 50% had LGE on CMR. Of these, 458 were nonobstructive and 965 were obstructive (of which 686 were underwent myectomy). At 4.7 ± 2.0 years of follow-up, 60 (4%) met the composite endpoint. On quadratic spline analysis, LGE ≥15% was associated with increased risk of composite events. In the obstructive subgroup, on competing risk regression analysis, ≥15% LGE (subhazard ratio: 3.04 [95% confidence interval: 1.48 to 6.10]) was associated with a higher rate and myectomy (subhazard ratio: 0.44 [95% confidence interval: 0.20 to 0.76]) was associated with a lower rate of composite endpoints (both p < 0.01). Similarly, sequential addition of LGE ≥15% and myectomy to % 5-year SCD risk score improved the log likelihood ratios from -227.85 to -219.14 (chi-square 17) and to -215.14 (chi-square 8; both p < 0.01). Association of %LGE with composite events was similar even in myectomy and nonobstructive subgroups.
Conclusions:
In low-/intermediate-risk adult patients with HCM (obstructive, myectomy, and nonobstructive subgroups) with preserved systolic function, %LGE was significantly associated with a higher rate of composite endpoint, providing incremental prognostic utility.
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