Table 1
Baseline Characteristics of 187 Patients with Hypertrophic Cardiomyopathy (HCM).
| CHARACTERISTICS | TOTAL (n = 187) | PRIMARY OUTCOME | p-VALUE |
|---|---|---|---|
| Age, mean ± SD | 41.5 ± 14.0 | 41.6 | 0.793 |
| Male sex, n (%) | 105 (56.1%) | 10 (62%) | 0.187 |
| Follow-up (years), median (IQR) | 8.38 [7.41; 9.1] | 7.2 | 0.187 |
| Echocardiogram (mm) | |||
| Septum, median (IQR) | 23.8 [20.0; 27.0] | 23.8 | 0.984 |
| Left atrium, median (IQR) | 42.9 [38.0; 47.0] | 41.7 | 0.406 |
| Left ventricle, mm | 43.2 ± 5.51 | 41.8 | 0.293 |
| Ejection fraction (%), mean ± SD | 71.5 ± 8.58 | 70.5 | 0.615 |
| Obstructive form, n (%) | 57 (26%) | 5 (31%) | 0.572 |
| SCD Risk Factors | |||
| Syncope, n (%) | 44 (23%) | 8 (50%) | 0.026 |
| NSVT on Holter, n (%) | 58 (31%) | 4 (25%) | 0.771 |
| Family history of SCD, n (%) | 68 (36%) | 8 (50%) | 0.280 |
| Septum ≥ 30 mm, n (%) | 65 (35%) | 7 (43%) | 0.425 |
| ACC/AHA 2020 | |||
| High Risk, n (%) | 106 (56%) | 13 (81%) | 0.062 |
| ESC 2014 | |||
| HCM Risk-SCD ≥ 4%, n (%) | 89 (47%) | 10 (62%) | 0.296 |
| HCM Risk-SCD ≥ 6%, n (%) | 54 (29%) | 7 (44%) | 0.246 |
| NYHA class | |||
| I | 100 (53,8%) | 5 (31%) | 0.066 |
| II | 68 (36,4%) | 7 (41,2%) | 0.059 |
| III | 19 (9,8%) | 4 (27,8%) | 0.035 |
| Medication | |||
| Beta-blocker, n (%) | 140 (75%) | 14 (87%) | 0.366 |
| Ca+ Channel Blocker, n (%) | 44 (23%) | 3 (19%) | 0.767 |
| Amiodarone, n (%) | 42 (22%) | 5 (31%) | 0.361 |
[i] Abbreviations: SD, standard deviation; IQR, interquartile range; SCD, sudden cardiac death; NSVT, non-sustained ventricular tachycardia; NYHA, New York Heart Association; ACC/AHA, American College of Cardiology/American Heart Association; ESC, European Society of Cardiology; HCM, Hypertrophic Cardiomyopathy; Ca+, calcium.
Table 2
Comparison of Odds Ratio and Number Needed to Treat between Event Prediction Models.
| MODEL | HIGH RISK (CDI INDICATED) | LOW RISK (CDI NOT INDICATED) | OR (95% CI) | p-VALUE | NNT (N) |
|---|---|---|---|---|---|
| ACC/AHA 2020 | 13/106 | 3/81 | 3.63 (1.0–13.2) | 0.06 | 12 |
| ESC 2014 ≥ 4% | 10/89 | 6/98 | 1.94 (0.67–5.57) | 0.29 | 16 |
| ESC 2014 ≥ 6% | 7/54 | 9/133 | 2.05 (0.72–5.82) | 0.24 | 19 |
[i] Abbreviations: CDI, cardioverter-defibrillator implantation; OR, odds ratio; NNT, number needed to treat.
Figure 1
ROC Curves Comparing ACC/AHA 2020 and ESC 2014 Risk Prediction Models.
ROC Curves for Risk Prediction Models of ACC/AHA 2020 and ESC 2014 with 4% and 6% Cut-offs. The Sudden Cardiac Death Risk Stratification in ACC/AHA 2020 Guideline Demonstrates a Higher Area under the Curve (0.63) Compared to ESC 2014 Models.
ROC: Receiver operating characteristic, ACC/AHA: American College of Cardiology/American Heart Association, ESC: European Society of Cardiology.
Table 3
Comparison of Sensitivity, Specificity, and Accuracy between Event Prediction Models.
| MODEL | AUC (95% CI) | SENSITIVITY (%) (95% CI) | SPECIFICITY (%) (95% CI) | NPV (%) (95% CI) | ACCURACY (%) (95% CI) |
|---|---|---|---|---|---|
| ACC/AHA 2020 | 0.63 | 81 (62.1–99.9) | 45 (38.1–53.1) | 96 (92.2–99.9) | 49 (41.5–55.8) |
| ESC 2014 ≥ 4% | 0.58 | 62 (38.8–86.2) | 54 (46.3–61.3) | 94 (89.1–98.6) | 54 (47.4–61.7) |
| ESC 2014 ≥ 6% | 0.58 | 44 (19.4–68.1) | 72 (65.8–79.2) | 93 (89.0–97.5) | 54 (63.5–76.6) |
[i] Abbreviations: AUC, area under the curve; CI, confidence interval; NPV, negative predictive value.
Figure 2
Kaplan-Meier Curves for Survival Probabilities by Risk Stratification Models.
Kaplan-Meier Curves for Overall Survival Free from All-Cause Mortality by Risk Stratifications Based on ACC/AHA 2020 (A), ESC 2014 Guideline with 4% Cut-off (B), and 6% Cut-off (C).
ACC/AHA: American College of Cardiology/American Heart Association, ESC: European Society of Cardiology.
Graphical Abstract
Comparative Validation of ACC/AHA and ESC Guidelines for Sudden Death Risk Stratification in a Brazilian Hypertrophic Cardiomyopathy Cohort.
ACC/AHA and ESC guidelines for sudden death (SD) risk in 187 Brazilian hypertrophic cardiomyopathy (HCM) patients. The ACC/AHA guidelines identified 56% as high risk, while the ESC guidelines identified 29% (threshold >6%). Over 8.3 years, primary outcomes (VF, STV, ICD therapy, SCD/CRP) had event rates of 12% (ACC/AHA) and 13% (ESC). Kaplan-Meier curves show overall survival, comparing high- and low-risk groups, illustrating the guidelines’ effectiveness in predicting outcomes.
ACC/AHA: American College of Cardiology/American Heart Association, ESC: European Society of Cardiology, HCM: Hypertrophic Cardiomyopathy, SD: Sudden Death, VF: Ventricular Fibrillation, STV: Sustained Ventricular Tachycardia, ICD: Implantable Cardioverter-Defibrillator, SCD: Sudden Cardiac Death, CRP: Cardiac-Related Procedures.