ECG-based score estimates the probability to detect Fabry Disease cardiac involvement

Stefano Figliozzi; Antonia Camporeale; Sara Boveri; Federico Pieruzzi; Maurizio Pieroni; Paola Lusardi; Marco Spada; Renzo Mignani; Alessandro Burlina; Francesca Graziani; Silvia Pica; Lara Tondi; Andrea Bernardini; Kelvin Chow; Mehdi Namdar; Massimo Lombardi

doi:10.1016/j.ijcard.2021.07.022

ECG-based score estimates the probability to detect Fabry Disease cardiac involvement

Stefano Figliozzi, Antonia Camporeale, Sara Boveri, Federico Pieruzzi, Maurizio Pieroni, Paola Lusardi, Marco Spada, Renzo Mignani, Alessandro Burlina, Francesca Graziani, Silvia Pica, Lara Tondi, Andrea Bernardini, Kelvin Chow, Mehdi Namdar, Massimo Lombardi

Research output: Contribution to journal › Article › peer-review

Abstract

OBJECTIVES: To elaborate an ECG-based nomogram estimating the probability to detect cardiac involvement by cardiac magnetic resonance (CMR) in Fabry Disease (FD).

METHODS: 119 FD patients and 26 healthy controls underwent ECG and CMR. Test (n = 88, 60%) and validation cohorts (n = 57, 40%) were randomly derived. Cardiac involvement was defined as the presence of low myocardial T1 value, a CMR-surrogate of myocardial glycosphingolipid storage. ECG changes associated with low T1 value were identified in the test cohort, included in the nomogram and then tested in the validation cohort.

RESULTS: Sokolow-Lyon index (AUC = 0.769), ratio between P-wave and PR-segment durations (Pwave/PRsegment) (AUC = 0.778), QRS duration (AUC = 0.703), QT (AUC = 0.769) duration were independently associated with the presence of low T1 on CMR at multivariate analysis. An ECG-based nomogram including these four parameters was accurate in identifying patients with CMR evidence of glycosphingolipid storage (c-index of the derived-nomogram = 0.90 in the test group; 0.81 in the validation group).

CONCLUSION: We propose a practical ECG-based nomogram accurately estimating the probability to detect low T1 values by CMR in FD patients. The application of this tool in clinical practice could improve early detection of FD cardiac involvement.

Original language	English
Pages (from-to)	110-117
Number of pages	8
Journal	Int. J. Cardiol.
Volume	339
DOIs	https://doi.org/10.1016/j.ijcard.2021.07.022
Publication status	Published - Sept 15 2021

Keywords

Cross-Sectional Studies
Early Diagnosis
Electrocardiography
Fabry Disease/diagnostic imaging
Humans
Magnetic Resonance Imaging, Cine
Myocardium
Predictive Value of Tests
Probability

Access to Document

10.1016/j.ijcard.2021.07.022

Cite this

Figliozzi, S., Camporeale, A., Boveri, S., Pieruzzi, F., Pieroni, M., Lusardi, P., Spada, M., Mignani, R., Burlina, A., Graziani, F., Pica, S., Tondi, L., Bernardini, A., Chow, K., Namdar, M., & Lombardi, M. (2021). ECG-based score estimates the probability to detect Fabry Disease cardiac involvement. Int. J. Cardiol., 339, 110-117. https://doi.org/10.1016/j.ijcard.2021.07.022

Figliozzi, S, Camporeale, A, Boveri, S, Pieruzzi, F, Pieroni, M, Lusardi, P, Spada, M, Mignani, R, Burlina, A, Graziani, F, Pica, S, Tondi, L, Bernardini, A, Chow, K, Namdar, M & Lombardi, M 2021, 'ECG-based score estimates the probability to detect Fabry Disease cardiac involvement', Int. J. Cardiol., vol. 339, pp. 110-117. https://doi.org/10.1016/j.ijcard.2021.07.022

@article{3c9a527d3bb7485e86698803ea9d5167,

title = "ECG-based score estimates the probability to detect Fabry Disease cardiac involvement",

abstract = "OBJECTIVES: To elaborate an ECG-based nomogram estimating the probability to detect cardiac involvement by cardiac magnetic resonance (CMR) in Fabry Disease (FD).METHODS: 119 FD patients and 26 healthy controls underwent ECG and CMR. Test (n = 88, 60%) and validation cohorts (n = 57, 40%) were randomly derived. Cardiac involvement was defined as the presence of low myocardial T1 value, a CMR-surrogate of myocardial glycosphingolipid storage. ECG changes associated with low T1 value were identified in the test cohort, included in the nomogram and then tested in the validation cohort.RESULTS: Sokolow-Lyon index (AUC = 0.769), ratio between P-wave and PR-segment durations (Pwave/PRsegment) (AUC = 0.778), QRS duration (AUC = 0.703), QT (AUC = 0.769) duration were independently associated with the presence of low T1 on CMR at multivariate analysis. An ECG-based nomogram including these four parameters was accurate in identifying patients with CMR evidence of glycosphingolipid storage (c-index of the derived-nomogram = 0.90 in the test group; 0.81 in the validation group).CONCLUSION: We propose a practical ECG-based nomogram accurately estimating the probability to detect low T1 values by CMR in FD patients. The application of this tool in clinical practice could improve early detection of FD cardiac involvement.",

keywords = "Cross-Sectional Studies, Early Diagnosis, Electrocardiography, Fabry Disease/diagnostic imaging, Humans, Magnetic Resonance Imaging, Cine, Myocardium, Predictive Value of Tests, Probability",

author = "Stefano Figliozzi and Antonia Camporeale and Sara Boveri and Federico Pieruzzi and Maurizio Pieroni and Paola Lusardi and Marco Spada and Renzo Mignani and Alessandro Burlina and Francesca Graziani and Silvia Pica and Lara Tondi and Andrea Bernardini and Kelvin Chow and Mehdi Namdar and Massimo Lombardi",

year = "2021",

month = sep,

day = "15",

doi = "10.1016/j.ijcard.2021.07.022",

language = "English",

volume = "339",

pages = "110--117",

journal = "Int. J. Cardiol.",

issn = "0167-5273",

publisher = "Elsevier Ireland Ltd",

}

TY - JOUR

T1 - ECG-based score estimates the probability to detect Fabry Disease cardiac involvement

AU - Figliozzi, Stefano

AU - Camporeale, Antonia

AU - Boveri, Sara

AU - Pieruzzi, Federico

AU - Pieroni, Maurizio

AU - Lusardi, Paola

AU - Spada, Marco

AU - Mignani, Renzo

AU - Burlina, Alessandro

AU - Graziani, Francesca

AU - Pica, Silvia

AU - Tondi, Lara

AU - Bernardini, Andrea

AU - Chow, Kelvin

AU - Namdar, Mehdi

AU - Lombardi, Massimo

PY - 2021/9/15

Y1 - 2021/9/15

N2 - OBJECTIVES: To elaborate an ECG-based nomogram estimating the probability to detect cardiac involvement by cardiac magnetic resonance (CMR) in Fabry Disease (FD).METHODS: 119 FD patients and 26 healthy controls underwent ECG and CMR. Test (n = 88, 60%) and validation cohorts (n = 57, 40%) were randomly derived. Cardiac involvement was defined as the presence of low myocardial T1 value, a CMR-surrogate of myocardial glycosphingolipid storage. ECG changes associated with low T1 value were identified in the test cohort, included in the nomogram and then tested in the validation cohort.RESULTS: Sokolow-Lyon index (AUC = 0.769), ratio between P-wave and PR-segment durations (Pwave/PRsegment) (AUC = 0.778), QRS duration (AUC = 0.703), QT (AUC = 0.769) duration were independently associated with the presence of low T1 on CMR at multivariate analysis. An ECG-based nomogram including these four parameters was accurate in identifying patients with CMR evidence of glycosphingolipid storage (c-index of the derived-nomogram = 0.90 in the test group; 0.81 in the validation group).CONCLUSION: We propose a practical ECG-based nomogram accurately estimating the probability to detect low T1 values by CMR in FD patients. The application of this tool in clinical practice could improve early detection of FD cardiac involvement.

AB - OBJECTIVES: To elaborate an ECG-based nomogram estimating the probability to detect cardiac involvement by cardiac magnetic resonance (CMR) in Fabry Disease (FD).METHODS: 119 FD patients and 26 healthy controls underwent ECG and CMR. Test (n = 88, 60%) and validation cohorts (n = 57, 40%) were randomly derived. Cardiac involvement was defined as the presence of low myocardial T1 value, a CMR-surrogate of myocardial glycosphingolipid storage. ECG changes associated with low T1 value were identified in the test cohort, included in the nomogram and then tested in the validation cohort.RESULTS: Sokolow-Lyon index (AUC = 0.769), ratio between P-wave and PR-segment durations (Pwave/PRsegment) (AUC = 0.778), QRS duration (AUC = 0.703), QT (AUC = 0.769) duration were independently associated with the presence of low T1 on CMR at multivariate analysis. An ECG-based nomogram including these four parameters was accurate in identifying patients with CMR evidence of glycosphingolipid storage (c-index of the derived-nomogram = 0.90 in the test group; 0.81 in the validation group).CONCLUSION: We propose a practical ECG-based nomogram accurately estimating the probability to detect low T1 values by CMR in FD patients. The application of this tool in clinical practice could improve early detection of FD cardiac involvement.

KW - Cross-Sectional Studies

KW - Early Diagnosis

KW - Electrocardiography

KW - Fabry Disease/diagnostic imaging

KW - Humans

KW - Magnetic Resonance Imaging, Cine

KW - Myocardium

KW - Predictive Value of Tests

KW - Probability

U2 - 10.1016/j.ijcard.2021.07.022

DO - 10.1016/j.ijcard.2021.07.022

M3 - Article

C2 - 34274410

SN - 0167-5273

VL - 339

SP - 110

EP - 117

JO - Int. J. Cardiol.

JF - Int. J. Cardiol.

ER -

ECG-based score estimates the probability to detect Fabry Disease cardiac involvement

Abstract

Keywords

Access to Document

Fingerprint

Cite this