Markers of Prognosis in Hypertrophic Cardiomyopathy

Quick Takes

  • In a network meta-analysis of 112 studies that examined markers of prognosis in HCM, late gadolinium enhancement (LGE) was the single best marker of adverse events.
  • A prediction score comprised of LGE and imaging markers of diastolic function was superior in predicting adverse events compared to traditional models used currently in practice for HCM patients.
  • Cardiac MRI plays a key role in prognostication in HCM patients.

Study Questions:

What is the prognostic impact of demographic, clinical, biochemical, and imaging findings in patients with hypertrophic cardiomyopathy (HCM)?


This was a systematic review of studies exploring prognosticators in HCM patients. The authors performed a network meta-analysis of studies that examined the association between endpoints and baseline characteristics classified as: 1) genotype, 2) biomarkers, 3) imaging characteristics, and 4) clinical parameters. The primary endpoint of interest was a composite of all-cause death or ventricular arrhythmic events (ventricular tachycardia [VT]/ventricular fibrillation/sudden cardiac death [SCD]/appropriate implantable cardioverter-defibrillator shocks) with heart failure (HF) hospitalization or cardiac transplantation.


Of 4,329 studies, 112 were included in the analysis. Age was not predictive of adverse events. Male sex was associated with a reduced risk for the primary endpoint. Higher B-type natriuretic peptide (BNP) or N-terminal pro–BNP (NT-proBNP), positive genotype, atrial fibrillation, and all variables included in the HCM Risk SCD score were associated with the composite endpoint. Apical aneurysm, left ventricular (LV) mass indexed for body surface area, LV ejection fraction <50%, presence of late gadolinium enhancement (LGE), and impaired global longitudinal strain correlated with the composite endpoint. Diastolic markers (E/E’ and left atrial [LA] dimensions) were also associated with the composite endpoint.

In direct comparisons in the meta-analysis, when evaluated individually, LGE followed by New York Heart Association class >II and nonsustained VT had the highest predictive value for the composite endpoint. A composite of imaging characteristics that included cardiac function and tissue characterization of scarring and diastolic function was superior in predicting adverse clinical events in HCM patients compared to traditional models.


In a network meta-analysis of all studies examining prognostic ability of patient characteristics, LGE was the strongest predictor individually for adverse events in HCM patients. A composite endpoint including cardiac function and tissue characterization was superior in predicting adverse clinical events compared to traditional models.


Management of HCM centers on both symptomatic management and risk stratification for SCD. At present, risk scores largely incorporate conventional clinical and limited imaging variables largely obtained from an echocardiogram. In this large meta-analysis, compared to several demographic, biomarker, and clinical parameters, imaging characteristics had the best predictive ability for a composite of all-cause mortality/ventricular arrhythmia/HF hospitalization or cardiac transplant. Of all variables assessed, LGE individually had the best predictive ability. A composite model accounting for LGE, diastolic function, and LA and LV dimension was superior in predicting adverse clinical events in HCM patients as opposed to conventional models. This study highlights the role of imaging, especially cardiac magnetic resonance imaging (MRI) as a powerful prognostic tool in HCM patients. It also highlights the need for revised risk scores in HCM patients that incorporate imaging-based tissue characterization and predict endpoints other than arrhythmic events such as HF or need for transplant.

Clinical Topics: Arrhythmias and Clinical EP, Cardiac Surgery, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Aortic Surgery, Cardiac Surgery and Arrhythmias, Cardiac Surgery and Heart Failure, Acute Heart Failure, Heart Failure and Cardiac Biomarkers, Heart Transplant, Interventions and Imaging, Interventions and Structural Heart Disease, Interventions and Vascular Medicine, Magnetic Resonance Imaging

Keywords: Aneurysm, Arrhythmias, Cardiac, Atrial Fibrillation, Cardiomyopathy, Hypertrophic, Contrast Media, Death, Sudden, Cardiac, Defibrillators, Implantable, Diagnostic Imaging, Gadolinium, Heart Failure, Heart Transplantation, Magnetic Resonance Imaging, Natriuretic Peptide, Brain, Prognosis, Risk Assessment, Risk Factors, Tachycardia, Ventricular, Ventricular Fibrillation

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