Risk Stratification in Hypertrophic Cardiomyopathy

Abstract

Hypertrophic cardiomyopathy (HCM) is a hereditary primary myocardial disease that is most commonly due to mutations within genes encoding sarcomeric contractile proteins and is characterised by left ventricular hypertrophy in the absence of a cardiac or systemic cause. Although the overall prognosis is relatively good with an annual mortality rate <1 %, the propensity to potentially fatal ventricular arrhythmias is the most feared complication. The identification of patients at risk of arrhythmogenic sudden cardiac death (SCD) is an essential component in disease management. Aborted SCD and malignant ventricular arrhythmias are the most powerful risk factors for SCD and ICD implantation is recommended in such circumstances. The selection of patients who may benefit from ICD therapy for primary prevention purposes is more challenging. The heterogeneous nature of the disease and the variation in trigger factors provides an adequate explanation for the low predictive accuracy of most conventional risk factors in isolation. A new risk model for risk stratification proposed by the European Society of Cardiology HCM outcome group shows promise but requires validation in different cohorts. The ICD is the only effective therapy in preventing SCD for the disease with a relatively low adverse event rate, but most deaths occur in relatively young patients. However, it is also difficult to ignore the complications with the ICD, therefore, the strive to perfect risk stratification in HCM should continue to ensure that only the most high-risk patients receive an ICD.

Familial Hypertrophic Cardiomyopathy: Late Potentials and Other Prognostic Markers

“…Nevertheless, some evidence suggests it might be a useful marker for sudden death as it is correlated to an increased risk of fatal arrhythmias [17]. Currently, there are no official recommendations for the use of highresolution electrocardiography in the risk stratification of HCM patients [18]. Since these shreds of evidence are controversial, we aimed to investigate the association between late potentials and familial sudden death, syncope, and complex ventricular arrhythmias.…”

“…However, in HCM patients, the indications for the implant remain unclear. Primary and secondary sudden death prevention in HCM patients is supported by the European Society of Cardiology Guidelines, especially in patients with a familial history of HCM, unexplained syncope, severe left ventricular hypertrophy, non-sustained tachycardia and history of aborted sudden death [18][19]. Maron et al demonstrated that the implant was effective in 11% of patients as secondary prevention and 5% as primary prevention in high-risk HCM subjects [12].…”

Sleep Disordered Breathing in Hypertrophic Cardiomyopathy—Current State and Future Directions

“…Other risk modifiers that are considered are: (1) late-gadolinium enhancement (LGE) on cardiac MRIs [69], (2) double mutations with compound heterozygotes and digenic carriers [70], (3) marked LVOT obstruction (Gradient ≥ 30 mmHg), and (4) LV apical aneurysms with LGE [71].…”

Asymmetric Hypertrophic Cardiomyopathy in Athletes: Always a Higher Risk for Sudden Death?

“…Heterogeneous delayed enhanced compatibility with fibrosis was detected in the middle myocardium of the inferior and superior segments of the septal wall. The risk of sudden death was 2.68% using the HCM Risk-SCD calculator, which did not recommended ICD implantation 2,4 . He was recommended to completely stop physical exercise.…”