Original development study, external validation studies, and additional calculator refinements
| . | Pts (n) . | Pts with ICD at baseline (n/%) . | Follow-up (years) . | Total events (n/%) . | ICD shocks (n/%) . | Findings . | Comments . |
|---|---|---|---|---|---|---|---|
| Original development study | |||||||
| Cadrin-Tourigny et al.6 (2019) | 528 | 218 (41.3) | 4.83 (2.44–9.33) | 146 (27.7) | 102 (19.3) | Overall C statistic: 0.77 (0.73–0.81) | Development of the ARVC risk calculator |
| External validation studies | |||||||
| Casella et al.32 (2020) | 82 | 54 (65.9) | 5.41 (2.59–8.37) | 28 (34.1) | 23 (28.0) | Good performance of risk calculator in classic ARVC forms | Risk calculator underpredicts risk in BiV/LD forms |
| Gasperetti et al.57 (2020) | 20 | 7 (35.0) | 5.3 (3.2–6.6) | 6 (30.0) | 5 (25.0) | Good performance of risk calculator in ARVC patients with a high exercise exposure | Very high-end endurance athlete cohort |
| Aquaro et al.41 (2020) | 140 | 51 (36.4) | 5.0 (2.0–8.0) | 48 (34) | 33 (23.6) | Good performance of risk calculator in classic ARVC forms | Mix of primary/secondary prevention pts; risk calculator underpredicts risk in BiV/LD forms |
| Baudinaud et al.53 (2021) | 115 | 1 (0.9) | 7.8 (6.1–9.7) | 15 (13.0) | 2 (1.7) | C statistic: 0.84 (0.74–0.93) | Risk overestimation for low risk patients |
| Zhang et al.54 (2022) | 88 | 70 (79.5) | 3.9 (1.6–6.9) | 57 (64.8) | 57 (64.8) | Overall C statistic: 0.681 (0.567–0.796) | Mix of primary and secondary prevention pts |
| Primary prevention C statistic: 0.833 (0.615–1.000) | |||||||
| Secondary prevention C statistic: 0.640 (0.510–0.770) | |||||||
| Protonotarios et al.26 (2022) | 554 | 263 (47.5) | 6.0 (3.1–12.5) | 100 (18.1) | 52 (9.3) | Overall C statistic: 0.75 (0.70–0.81) | Significant impact of genotype on risk calculator performance |
| Gene-positive C statistic: 0.82 (0.76–0.88) | |||||||
| Gene-elusive C statistic: 0.65 (0.57–0.74) | |||||||
| PKP-2 C statistic: 0.83 (0.75–0.91) | |||||||
| DSP C statistic: 0.80 (0.53–0.96) | |||||||
| Jordà et al55 (2022) | 429 | 175 (40.8) | 5.02 (2.05–7.90) | 103 (24) | 61 (14.2) | C statistic: 0.70 (0.65–0.75) | Main validation study |
| Additional calculator refinements | |||||||
| Bosman et al.27 (2022) | 176 | N/A | 5.4 (2.7–9.7) | 53 (30.1) | 40 (22.7) | C statistic: 0.77 (0.71–0.84) | No need for exercise correction in the risk calculator estimates |
| Gasperetti et al.33 (2022) | 288 | 78 (27.1) | 5.31 (2.89–10.17) | 120 (41.7) | 89 (30.9) | Integrated C statistic of risk calculator + PVS: 0.75 | Maximal benefit of PVS in moderate risk patients (<25% 5-year predicted risk) for ICD exclusion |
| Bourfiss et al.40 (2022) | 132 | 68 (51.5) | 4.3 (2.0–7.9) | 25 (19.0) | 22 (16.7) | C statistic Risk Calc: 0.76 (0.63–0.90) | Inclusion of CMR derived LV global and septal circumferential strain does not improve the model |
| Integrated C statistic risk calc + LV strain: 0.82 (0.72–0.92) | |||||||
| . | Pts (n) . | Pts with ICD at baseline (n/%) . | Follow-up (years) . | Total events (n/%) . | ICD shocks (n/%) . | Findings . | Comments . |
|---|---|---|---|---|---|---|---|
| Original development study | |||||||
| Cadrin-Tourigny et al.6 (2019) | 528 | 218 (41.3) | 4.83 (2.44–9.33) | 146 (27.7) | 102 (19.3) | Overall C statistic: 0.77 (0.73–0.81) | Development of the ARVC risk calculator |
| External validation studies | |||||||
| Casella et al.32 (2020) | 82 | 54 (65.9) | 5.41 (2.59–8.37) | 28 (34.1) | 23 (28.0) | Good performance of risk calculator in classic ARVC forms | Risk calculator underpredicts risk in BiV/LD forms |
| Gasperetti et al.57 (2020) | 20 | 7 (35.0) | 5.3 (3.2–6.6) | 6 (30.0) | 5 (25.0) | Good performance of risk calculator in ARVC patients with a high exercise exposure | Very high-end endurance athlete cohort |
| Aquaro et al.41 (2020) | 140 | 51 (36.4) | 5.0 (2.0–8.0) | 48 (34) | 33 (23.6) | Good performance of risk calculator in classic ARVC forms | Mix of primary/secondary prevention pts; risk calculator underpredicts risk in BiV/LD forms |
| Baudinaud et al.53 (2021) | 115 | 1 (0.9) | 7.8 (6.1–9.7) | 15 (13.0) | 2 (1.7) | C statistic: 0.84 (0.74–0.93) | Risk overestimation for low risk patients |
| Zhang et al.54 (2022) | 88 | 70 (79.5) | 3.9 (1.6–6.9) | 57 (64.8) | 57 (64.8) | Overall C statistic: 0.681 (0.567–0.796) | Mix of primary and secondary prevention pts |
| Primary prevention C statistic: 0.833 (0.615–1.000) | |||||||
| Secondary prevention C statistic: 0.640 (0.510–0.770) | |||||||
| Protonotarios et al.26 (2022) | 554 | 263 (47.5) | 6.0 (3.1–12.5) | 100 (18.1) | 52 (9.3) | Overall C statistic: 0.75 (0.70–0.81) | Significant impact of genotype on risk calculator performance |
| Gene-positive C statistic: 0.82 (0.76–0.88) | |||||||
| Gene-elusive C statistic: 0.65 (0.57–0.74) | |||||||
| PKP-2 C statistic: 0.83 (0.75–0.91) | |||||||
| DSP C statistic: 0.80 (0.53–0.96) | |||||||
| Jordà et al55 (2022) | 429 | 175 (40.8) | 5.02 (2.05–7.90) | 103 (24) | 61 (14.2) | C statistic: 0.70 (0.65–0.75) | Main validation study |
| Additional calculator refinements | |||||||
| Bosman et al.27 (2022) | 176 | N/A | 5.4 (2.7–9.7) | 53 (30.1) | 40 (22.7) | C statistic: 0.77 (0.71–0.84) | No need for exercise correction in the risk calculator estimates |
| Gasperetti et al.33 (2022) | 288 | 78 (27.1) | 5.31 (2.89–10.17) | 120 (41.7) | 89 (30.9) | Integrated C statistic of risk calculator + PVS: 0.75 | Maximal benefit of PVS in moderate risk patients (<25% 5-year predicted risk) for ICD exclusion |
| Bourfiss et al.40 (2022) | 132 | 68 (51.5) | 4.3 (2.0–7.9) | 25 (19.0) | 22 (16.7) | C statistic Risk Calc: 0.76 (0.63–0.90) | Inclusion of CMR derived LV global and septal circumferential strain does not improve the model |
| Integrated C statistic risk calc + LV strain: 0.82 (0.72–0.92) | |||||||
Original development study, external validation studies, and additional calculator refinements
| . | Pts (n) . | Pts with ICD at baseline (n/%) . | Follow-up (years) . | Total events (n/%) . | ICD shocks (n/%) . | Findings . | Comments . |
|---|---|---|---|---|---|---|---|
| Original development study | |||||||
| Cadrin-Tourigny et al.6 (2019) | 528 | 218 (41.3) | 4.83 (2.44–9.33) | 146 (27.7) | 102 (19.3) | Overall C statistic: 0.77 (0.73–0.81) | Development of the ARVC risk calculator |
| External validation studies | |||||||
| Casella et al.32 (2020) | 82 | 54 (65.9) | 5.41 (2.59–8.37) | 28 (34.1) | 23 (28.0) | Good performance of risk calculator in classic ARVC forms | Risk calculator underpredicts risk in BiV/LD forms |
| Gasperetti et al.57 (2020) | 20 | 7 (35.0) | 5.3 (3.2–6.6) | 6 (30.0) | 5 (25.0) | Good performance of risk calculator in ARVC patients with a high exercise exposure | Very high-end endurance athlete cohort |
| Aquaro et al.41 (2020) | 140 | 51 (36.4) | 5.0 (2.0–8.0) | 48 (34) | 33 (23.6) | Good performance of risk calculator in classic ARVC forms | Mix of primary/secondary prevention pts; risk calculator underpredicts risk in BiV/LD forms |
| Baudinaud et al.53 (2021) | 115 | 1 (0.9) | 7.8 (6.1–9.7) | 15 (13.0) | 2 (1.7) | C statistic: 0.84 (0.74–0.93) | Risk overestimation for low risk patients |
| Zhang et al.54 (2022) | 88 | 70 (79.5) | 3.9 (1.6–6.9) | 57 (64.8) | 57 (64.8) | Overall C statistic: 0.681 (0.567–0.796) | Mix of primary and secondary prevention pts |
| Primary prevention C statistic: 0.833 (0.615–1.000) | |||||||
| Secondary prevention C statistic: 0.640 (0.510–0.770) | |||||||
| Protonotarios et al.26 (2022) | 554 | 263 (47.5) | 6.0 (3.1–12.5) | 100 (18.1) | 52 (9.3) | Overall C statistic: 0.75 (0.70–0.81) | Significant impact of genotype on risk calculator performance |
| Gene-positive C statistic: 0.82 (0.76–0.88) | |||||||
| Gene-elusive C statistic: 0.65 (0.57–0.74) | |||||||
| PKP-2 C statistic: 0.83 (0.75–0.91) | |||||||
| DSP C statistic: 0.80 (0.53–0.96) | |||||||
| Jordà et al55 (2022) | 429 | 175 (40.8) | 5.02 (2.05–7.90) | 103 (24) | 61 (14.2) | C statistic: 0.70 (0.65–0.75) | Main validation study |
| Additional calculator refinements | |||||||
| Bosman et al.27 (2022) | 176 | N/A | 5.4 (2.7–9.7) | 53 (30.1) | 40 (22.7) | C statistic: 0.77 (0.71–0.84) | No need for exercise correction in the risk calculator estimates |
| Gasperetti et al.33 (2022) | 288 | 78 (27.1) | 5.31 (2.89–10.17) | 120 (41.7) | 89 (30.9) | Integrated C statistic of risk calculator + PVS: 0.75 | Maximal benefit of PVS in moderate risk patients (<25% 5-year predicted risk) for ICD exclusion |
| Bourfiss et al.40 (2022) | 132 | 68 (51.5) | 4.3 (2.0–7.9) | 25 (19.0) | 22 (16.7) | C statistic Risk Calc: 0.76 (0.63–0.90) | Inclusion of CMR derived LV global and septal circumferential strain does not improve the model |
| Integrated C statistic risk calc + LV strain: 0.82 (0.72–0.92) | |||||||
| . | Pts (n) . | Pts with ICD at baseline (n/%) . | Follow-up (years) . | Total events (n/%) . | ICD shocks (n/%) . | Findings . | Comments . |
|---|---|---|---|---|---|---|---|
| Original development study | |||||||
| Cadrin-Tourigny et al.6 (2019) | 528 | 218 (41.3) | 4.83 (2.44–9.33) | 146 (27.7) | 102 (19.3) | Overall C statistic: 0.77 (0.73–0.81) | Development of the ARVC risk calculator |
| External validation studies | |||||||
| Casella et al.32 (2020) | 82 | 54 (65.9) | 5.41 (2.59–8.37) | 28 (34.1) | 23 (28.0) | Good performance of risk calculator in classic ARVC forms | Risk calculator underpredicts risk in BiV/LD forms |
| Gasperetti et al.57 (2020) | 20 | 7 (35.0) | 5.3 (3.2–6.6) | 6 (30.0) | 5 (25.0) | Good performance of risk calculator in ARVC patients with a high exercise exposure | Very high-end endurance athlete cohort |
| Aquaro et al.41 (2020) | 140 | 51 (36.4) | 5.0 (2.0–8.0) | 48 (34) | 33 (23.6) | Good performance of risk calculator in classic ARVC forms | Mix of primary/secondary prevention pts; risk calculator underpredicts risk in BiV/LD forms |
| Baudinaud et al.53 (2021) | 115 | 1 (0.9) | 7.8 (6.1–9.7) | 15 (13.0) | 2 (1.7) | C statistic: 0.84 (0.74–0.93) | Risk overestimation for low risk patients |
| Zhang et al.54 (2022) | 88 | 70 (79.5) | 3.9 (1.6–6.9) | 57 (64.8) | 57 (64.8) | Overall C statistic: 0.681 (0.567–0.796) | Mix of primary and secondary prevention pts |
| Primary prevention C statistic: 0.833 (0.615–1.000) | |||||||
| Secondary prevention C statistic: 0.640 (0.510–0.770) | |||||||
| Protonotarios et al.26 (2022) | 554 | 263 (47.5) | 6.0 (3.1–12.5) | 100 (18.1) | 52 (9.3) | Overall C statistic: 0.75 (0.70–0.81) | Significant impact of genotype on risk calculator performance |
| Gene-positive C statistic: 0.82 (0.76–0.88) | |||||||
| Gene-elusive C statistic: 0.65 (0.57–0.74) | |||||||
| PKP-2 C statistic: 0.83 (0.75–0.91) | |||||||
| DSP C statistic: 0.80 (0.53–0.96) | |||||||
| Jordà et al55 (2022) | 429 | 175 (40.8) | 5.02 (2.05–7.90) | 103 (24) | 61 (14.2) | C statistic: 0.70 (0.65–0.75) | Main validation study |
| Additional calculator refinements | |||||||
| Bosman et al.27 (2022) | 176 | N/A | 5.4 (2.7–9.7) | 53 (30.1) | 40 (22.7) | C statistic: 0.77 (0.71–0.84) | No need for exercise correction in the risk calculator estimates |
| Gasperetti et al.33 (2022) | 288 | 78 (27.1) | 5.31 (2.89–10.17) | 120 (41.7) | 89 (30.9) | Integrated C statistic of risk calculator + PVS: 0.75 | Maximal benefit of PVS in moderate risk patients (<25% 5-year predicted risk) for ICD exclusion |
| Bourfiss et al.40 (2022) | 132 | 68 (51.5) | 4.3 (2.0–7.9) | 25 (19.0) | 22 (16.7) | C statistic Risk Calc: 0.76 (0.63–0.90) | Inclusion of CMR derived LV global and septal circumferential strain does not improve the model |
| Integrated C statistic risk calc + LV strain: 0.82 (0.72–0.92) | |||||||
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