The value of electrocardiography and echocardiography in distinguishing Fabry disease from sarcomeric hypertrophic cardiomyopathy

被引:6
|
作者
Junqua, Nicolas [1 ]
Legallois, Damien [1 ,2 ]
Segard, Sophie [1 ]
Lairez, Olivier [3 ]
Reant, Patricia [4 ,5 ]
Goizet, Cyril [6 ]
Maillard, Helene [7 ]
Charron, Philippe [8 ,9 ]
Milliez, Paul [1 ,2 ]
Labombarda, Fabien [1 ,2 ]
机构
[1] Caen Univ Hosp, Dept Cardiol, F-14000 Caen, France
[2] Caen Normandie Univ UNICAEN, Caen Univ Hosp, Med Sch, EA 4650 Signalisation Electrophysiol & Imagerie L, F-14000 Caen, France
[3] Univ Paul Sabatier, Rangueil Univ Hosp, Rangueil Med Sch, Dept Cardiol, F-31400 Toulouse, France
[4] Bordeaux Univ Hosp, Dept Cardiol, F-33000 Bordeaux, France
[5] Bordeaux Univ, IHU Liryc, INSERM U1045, F-33604 Pessac, France
[6] Univ Bordeaux, Bordeaux Univ Hosp, Dept Med Genet, INSERM Unit 1211,Lab MRGM, F-33076 Bordeaux, France
[7] Univ Lille, Claude Huriez Hosp, Dept Internal Med, F-59000 Lille, France
[8] Sorbonne Univ, Pitie Salpetriere Univ Hosp, Ctr Reference Malad Cardiaques Hereditaires, INSERM UMR S 1166, F-75013 Paris, France
[9] Sorbonne Univ, Pitie Salpetriere Univ Hosp, Inst Cardiometab & Nutr ICAN, F-75013 Paris, France
关键词
Fabry disease; Hypertrophic cardiomyopathy; Electrocardiogram; HEART-DISEASE; STORAGE; RECOMMENDATIONS; MANAGEMENT; DIAGNOSIS; SOCIETY; DIFFERENTIATION; IDENTIFICATION; ASSOCIATION; AMYLOIDOSIS;
D O I
10.1016/j.acvd.2020.04.008
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Background. - Screening for Fabry disease is sub-optimal in non-specialised centres. Aim. - To assess the diagnostic value of electrocardiographic scores of left ventricular hyper-trophy and a combined electrocardiographic and echocardiographic model in Fabry disease. Methods. - We retrospectively reviewed the electrocardiograms and echocardiograms of 61 patients (mean age 55.6 +/- 11.5 years; 57% men) with Fabry disease and left ventricular hyper-trophy, and compared them with those from 59 patients (mean age 44.8 +/- 18.3 years; 66% men) with sarcomeric hypertrophic cardiomyopathy. Six electrocardiography criteria for left ventricular hypertrophy were specifically analysed: Sokolow-Lyon voltage index; Cornell voltage index; Gubner index; Romhilt-Estes score; Sokolow-Lyon product (voltage index x QRS duration); and Cornell product (voltage index x QRS duration). Results. - Right bundle branch block was more frequent in patients with Fabry disease (54% vs. 22%; P = 0.001). QRS duration, Gubner score and Sokolow-Lyon product were significantly higher in patients with Fabry disease. Maximal wall thickness was higher in patients with sarcomeric hypertrophic cardiomyopathy (21.9 +/- 5.1 vs. 15.5 +/- 2.9 mm; P < 0.001). Indexed sinus of Valsalva diameter was larger in patients with Fabry disease. After multivariable analysis, right bundle branch block, Sokolow-Lyon product, maximal wall thickness and aortic diameter were independently associated with Fabry disease. A model including these four variables yielded an area under the receiver operating characteristic curve of 0.918 (95% confidence interval 0.868-0.968) for Fabry disease. Conclusion. - Our model combining easy-to-assess electrocardiographic and echocardiographic variables may be helpful in improving screening and reducing diagnosis delay in Fabry disease. (C) 2020 Elsevier Masson SAS. All rights reserved.
引用
收藏
页码:542 / 550
页数:9
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