Validation of the new American College of Cardiology/American Heart Association Guidelines for the risk stratification of sudden cardiac death in a large Mediterranean cohort with Hypertrophic Cardiomyopathy

Zegkos, Thomas; Parcharidou, Despοina; Ntelios, Dimitrios; Papanastasiou, Christos A.; Karagiannidis, Efstratios; Gossios, Thomas D.; Rouskas, Pavlos; Katranas, Sotiris; Paraskevaidis, Stylianos; Karvounis, Haralambos; Efthimiadis, Georgios K.

doi:10.1016/j.hjc.2021.06.005

Cited by 14 publications

(12 citation statements)

References 23 publications

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“…The level of prediction by RFs with AUCs ∼0.67–0.74 from five studies is at the lower limit of the definition of acceptability 18 and implies that only half of the at-risk patients are identified for a 20% implantation rate. This was reported in a database study, 23 which appears to be within the limits of O’Mahony et al, 6 the ACC/AHA and ESC guidelines have poor specificity 24 for a reasonable sensitivity, the ESC guidelines had an AUC of 0.68 in a study of 511 Chinese patients, 25 and a study comparing the ESC guidelines to the ACC guidelines had a C -index of 0.69. 26 However, with an AUC of 0.89, the EP method appears to have useful predictive capacity.…”

Section: Discussionsupporting

confidence: 52%

“…This high AUC is due to zero mortality in the low-risk group (348 patients), while applying the estimates from O’Mahony et al and O’Mahony et al 3 , 6 between 3 and 9 deaths or 3 to 31 5 would be expected, which would have lowered the AUC. Events occurred in low-risk patients in most studies 3 , 5 , 6 , 7 , 8 , 22 , 23 , 24 and are in the 2–4% event range, and so the high predictive accuracy in this study 28 stems from an unlikely event. The C -index of 0.86 for ESC method in 288 patients 29 is within the 2.5% limits of 0’Mahony ‘Model B’ (288 patients, AUC = 0.57–0.87) (as shown in Figure 2B ) and has a 50% sensitivity.…”

Section: Discussionmentioning

confidence: 79%

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Should lethal arrhythmias in hypertrophic cardiomyopathy be predicted using non-electrophysiological methods?

Saumarez¹,

Silberbauer

Scannell

et al. 2023

Europace

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While sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM) is due to arrhythmias, the guidelines for prediction of SCD are based solely on non-electrophysiological methods. This study aims to stimulate thinking about whether the interests of patients with HCM are better served by using current, ‘risk factor’, methods of prediction or by further development of electrophysiological methods to determine arrhythmic risk. Five published predictive studies of SCD in HCM, which contain sufficient data to permit analysis, were analysed to compute receiver operating characteristics together with their confidence bounds to compare their formal prediction either by bootstrapping or Monte Carlo analysis. Four are based on clinical risk factors, one with additional MRI analysis, and were regarded as exemplars of the risk factor approach. The other used an electrophysiological method and directly compared this method to risk factors in the same patients. Prediction methods that use conventional clinical risk factors and MRI have low predictive capacities that will only detect 50–60% of patients at risk with a 15–30% false positive rate [area under the curve (AUC) = ∼0.7], while the electrophysiological method detects 90% of events with a 20% false positive rate (AUC = ∼0.89). Given improved understanding of complex arrhythmogenesis, arrhythmic SCD is likely to be more accurately predictable using electrophysiologically based approaches as opposed to current guidelines and should drive further development of electrophysiologically based methods.

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Section: Discussionsupporting

confidence: 52%

Section: Discussionmentioning

confidence: 79%

Should lethal arrhythmias in hypertrophic cardiomyopathy be predicted using non-electrophysiological methods?

Saumarez¹,

Silberbauer

Scannell

et al. 2023

Europace

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“…According to the analysis presented here, the Krakow DCM Risk Score shows adequate performance in external validation, with an accuracy of over 70% [14]. This precision is comparable to the most widely available tools currently in use, such as the GRACE risk score 2.0 for mortality outcomes in acute cardiac syndrome, the HCM Risk-SCD score for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM), or CHA 2 DS 2 -VASc for stroke in atrial fibrillation, and is at least similar to prognostic scores in general HF cohorts, including the Heart Failure Survival Score (HFSS), the Seattle Heart Failure Model (SHFM) and the Meta-Analysis Global Group in Chronic Heart Failure (MUSIC) [14,15,[33][34][35][36]. The Krakow DCM Risk Score provided good discrimination for at least 7 out of 10 patients.…”

Section: Krakow Dcm Risk Score Performancementioning

confidence: 99%

Clinical Utility and Validation of the Krakow DCM Risk Score—A Prognostic Model Dedicated to Dilated Cardiomyopathy

Dziewięcka

Winiarczyk

Wiśniowska‐Śmiałek

et al. 2022

JPM

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Background: One of the most common causes of heart failure is dilated cardiomyopathy (DCM). In DCM, the mortality risk is high and reaches approximately 20% in 5 years. A patient’s prognosis should be established for appropriate HF management. However, so far, no validated tools have been available for the DCM population. Methods: The study population consisted of 735 DCM patients: 406 from the derivation cohort (previously described) and 329 from the validation cohort (from 2009 to 2020, with outcome data after a mean of 42 months). For each DCM patient, the individual mortality risk was calculated based on the Krakow DCM Risk Score. Results: During follow-up, 49 (15%) patients of the validation cohort died. They had shown significantly higher calculated 1-to-5-year mortality risks. The Krakow DCM Risk Score yielded good discrimination in terms of overall mortality risk, with an AUC of 0.704–0.765. Based on a 2-year mortality risk, patients were divided into non-high (≤6%) and high (>6%) mortality risk groups. The observed mortality rates were 8.3% (n = 44) vs. 42.6% (n = 75), respectively (HR 3.37; 95%CI 1.88–6.05; p < 0.0001). Conclusions: The Krakow DCM Risk Score was found to have good predictive accuracy. The 2-year mortality risk > 6% has good discrimination for the identification of high-risk patients and can be applied in everyday practice.

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“…Hypertrophic cardiomyopathy (HCM) is among the most common forms of cardiomyopathy [1,2], with a recorded prevalence of 1 in 500 people among the general population [3][4][5][6], and recent studies estimating a prevalence of up to 1 in 200 people [5,[7][8][9][10][11]. It is an important cause of disability and mortality across all ages [12], and one of the leading causes of sudden cardiac deaths among the young [13,14].…”

Section: Introductionmentioning

confidence: 99%

Gene-Specific Discriminative Echocardiogram Findings in Hypertrophic Cardiomyopathy Determined Using Artificial Intelligence: A Pilot Study

Glavaški,

Ilić,

Velicki

2023

Cardiogenetics

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Hypertrophic cardiomyopathy (HCM) is among the most common forms of cardiomyopathies, with a prevalence of 1:200 to 1:500 people. HCM is caused by variants in genes encoding cardiac sarcomeric proteins, of which a majority reside in MYH7, MYBPC3, and TNNT2. Up to 40% of the HCM cases do not have any known HCM variant. Genotype–phenotype associations in HCM remain incompletely understood. This study involved two visits of 46 adult patients with a confirmed diagnosis of HCM. In total, 174 genes were analyzed on the Next-Generation Sequencing platform, and transthoracic echocardiography was performed. Gene-specific discriminative echocardiogram findings were identified using the computer vision library Fast AI. This was accomplished with the generation of deep learning models for the classification of ultrasonic images based on the underlying genotype and a later analysis of the most decisive image regions. Gene-specific echocardiogram findings were identified: for variants in the MYH7 gene (vs. variant not detected), the most discriminative structures were the septum, left ventricular outflow tract (LVOT) segment, anterior wall, apex, right ventricle, and mitral apparatus; for variants in MYBPC3 gene (vs. variant not detected) these were the septum, left ventricle, and left ventricle/chamber; while for variants in the TNNT2 gene (vs. variant not detected), the most discriminative structures were the septum and right ventricle.

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Validation of the new American College of Cardiology/American Heart Association Guidelines for the risk stratification of sudden cardiac death in a large Mediterranean cohort with Hypertrophic Cardiomyopathy

Cited by 14 publications

References 23 publications

Should lethal arrhythmias in hypertrophic cardiomyopathy be predicted using non-electrophysiological methods?

Should lethal arrhythmias in hypertrophic cardiomyopathy be predicted using non-electrophysiological methods?

Clinical Utility and Validation of the Krakow DCM Risk Score—A Prognostic Model Dedicated to Dilated Cardiomyopathy

Gene-Specific Discriminative Echocardiogram Findings in Hypertrophic Cardiomyopathy Determined Using Artificial Intelligence: A Pilot Study

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