A comprehensive electrocardiographic, molecular, and echocardiographic study of Brugada syndrome: Validation of the 2013 diagnostic criteria

作者：Savastano Simone; Rordorf Roberto; Vicentini Alessandro; Petracci Barbara; Taravelli Erika; Castelletti Silvia; D'Errico Alessandra; Torchio Margherita; Dossena Cinzia; Novara Paola; Dagradi Federica; Landolina Maurizio; Spazzolini Carla; Crotti Lia; Schwartz Peter J^*

来源：Heart Rhythm, 2014, 11(7): 1176-1183.

DOI：10.1016/j.hrthm.2014.04.010

• Brugada syndrome
• Electrocardiography
• Echocardiography
• Diagnostic criteria
• SCN5A

BACKGROUND The debate on the diagnostic value of high intercostal spaces (ICSs) and of the number of diagnostic leads in Brugada syndrome (BrS) has been settled by a recent expert consensus statement.
OBJECTIVE To test the validity, and the underlying anatomy, of the new electrocardiographic (ECG) diagnostic criteria using echocardiographic, molecular, and clinical evidence in 1 clinical study population with BrS.
METHODS We analyzed 114 patients with BrS and with a spontaneous or drug-induced type 1 ECG pattern recorded in 1 or more right precordial leads in fourth, third, and second ICSs. The right ventricular outflow tract (RVOT) was localized by using echocardiography. All probands were screened on the SCN5A gene.
RESULTS The percentage of mutation carriers (MCs) and the event rate were similar regardless of the diagnostic ICS (fourth vs high ICSs: MCs 23% vs 19%; event rate 22% vs 28%) and the number of diagnostic leads (1 vs >= 2: MCs 20% vs 22%; event rate 22% vs 27%). The concordance between RVOT anatomical location and the diagnostic ICSs was 86%. The percentage of the diagnostic ECG pattern recorded was significantly increased by the exploration of the ICSs showing RVOT by echocardiography (echocardiography-guided approach vs conventional approach 100% vs 43%; P < .001).
CONCLUSION The high ICSs are not inferior to the standard fourth ICS for the ECG diagnosis of BrS, and the interindividual variability depends on the anatomical location of the RVOT as assessed by using echocardiography. This approach significantly increases diagnostic sensitivity without decreasing specificity and fully supports the recently published new diagnostic criteria.

• 出版日期2014-7