P5028Shanghai score system into practice

Abstract Background The 2016 J Wave Syndrome Consensus Report proposed the Shanghai Brugada Syndrome (BS) Score, a diagnostic score based on ECG features, genetic results, family and clinical history. Purpose The aim of our study was to reclassify our institution's BS cohort using the Shanghai BS Score. Methods We collected 170 patients with BS diagnosis (according to guidelines) who attended our outpatient clinic between 1996 and 2019. Patients were followed on an yearly basis. During follow up (FU) we looked for the appearance of a spontaneous Type 1 ECG (either with a 12 lead ECG or a 12 lead Holter monitoring) and arrhythmic events (defined as unexplained cardiac arrest or documented VF/polymorphic VT, nocturnal agonal respirations, syncope, atrial flutter/fibrillation). Genetic analysis, limited to SCN5A and SCN1B variants, was performed. We examined each item of the Shanghai BS Score at the first and the last visit at our hospital. Results At baseline all patients presented a type 1 ECG: 26 (15%) spontaneously, 2 (1%) during a febrile illness and 142 (84%) after ajmaline pharmacological challenge. 158 (93%) patients were asymptomatic while 12 (7%) presented symptoms: 1 unexplained cardiac arrest, 1 nocturnal agonal respirations, 9 syncopes, 1 atrial flutter/fibrillation under the age of 30 years. Genetic testing was performed in 73 (43%) patients; an SCN5A mutation was detected in 11 patients while a SCN1B was detected in 1 patient. According to the score we divided the patients as follows: group A 2 to 3 points 107 (63%) patients; group B 3.5 points 13 (8%) patients; group C 4 to 5 points 39 (23%) patients; group D ≥5.5 points 11 (6%) patients. During FU (medium 59 months) there were 3 (2.8%) arrhythmic events in group A (2 unexplained cardiac arrests and 1 syncope; 0 in group B; 1 (2.6%) in group C (syncope); 3 (27%) in group D (1 FV and 2 syncopes). 19 patients (11%) modified their Shanghai score during FU: 16 because of the appearance of a spontaneous type 1 ECG, 3 for their clinical history. Conclusions Although the Shanghai Score has been proposed as a diagnostic tool, data from literature show that patients with a higher score are at a progressively higher risk for VT/VF. Unexpectedly, we observed 2 unexplained cardiac arrests in the group of patients with a score 2 to 3. This may suggest the need to the use other risk criteria such as fractionated potentials or other ECG markers. Additionaly, we have shown that this Score is prone to changes over time, stressing the importance of a regular FU with continuous risk assessment.