Incremental benefit of late gadolinium cardiac magnetic resonance imaging for risk stratification in patients with hypertrophic cardiomyopathy

Christina Doesch,Stefan O Schoenberg,Boris Rudic,Johannes Budjan,Martin Borggrefe,Tobias Becher,Arman Smakic,Ibrahim Akin,Ibrahim El-Battrawy,Theano Papavassiliu,Juergen Kuschyk,Erol Tülümen

doi:10.1038/s41598-017-06533-0

Christina Doesch, Stefan O Schoenberg + Show 10 more

Open Access

https://doi.org/10.1038/s41598-017-06533-0

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Abstract

Hypertrophic cardiomyopathy (HCM) has a low risk for sudden cardiac death (SCD). The ESC clinical risk prediction model estimates the risk of SCD using clinical and echocardiographical parameters without taking into account cardiac magnetic resonance (CMR) parameters. Therefore, we compared the CMR characteristics of 149 patients with low, intermediate and high ESC risk scores. In these patients left and right ventricular ejection fraction and volumes were comparable. Patients with a high ESC risk score revealed a significantly higher extent of late gadolinium enhancement (LGE) compared to patients with intermediate or a low risk scores. During follow-up of 4 years an extent of LGE ≥20% identified patients at a higher risk for major adverse cardiac arrhythmic events in the low and intermediate ESC risk group whereas an extent of LGE <20% was associated with a low risk of major adverse cardiac arrhythmic events despite a high ESC risk score ≥6%. Hence, we hypothesize that the extent of fibrosis might be an additional risk marker.

Highlights

Hypertrophic cardiomyopathy (HCM) is a complex genetic heart disease[1,2,3,4]
Our study showed that HCM patients with a high risk of sudden cardiac death (SCD) according to the ESC clinical risk prediction model revealed the highest extent of late gadolinium enhancement (LGE) % compared to patients with HCM and lower ESC risk scores
During the follow-up an extent LGE ≥20% identified patients at a higher risk for major adverse cardiac arrhythmic events in the low and intermediate ESC risk group; whereas an extent LGE

Summary

Introduction

Hypertrophic cardiomyopathy (HCM) is a complex genetic heart disease[1,2,3,4]. the overall risk for sudden cardiac death (SCD) is relatively low, some patients with HCM die suddenly from fatal arrhythmic events[1, 5, 6]. The recently published ESC clinical risk prediction model[14] uses clinical and echocardiographic determined parameters such as age, family history of SCD, maximal left ventricular wall thickness, left atrial diameter, left ventricular outflow tract gradient, previous unexplained syncope and the occurrence of non-sustained ventricular tachycardia to estimate the probability of sudden cardiac death (SCD) at 5 years. This risk prediction model is just based on a multicenter, retrospective longitudinal cohort study[15] since prospective randomized studies are missing. Scores of SCD according to CMR characteristics in order to identify additional imaging risk factors that might help to refine risk stratification

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