Royal Prince Alfred Hospital, Sydney, Australia.
Sydney Medical School, University of Sydney, Sydney, Australia.
PLoS One. 2018 Apr 13;13(4):e0195594. doi: 10.1371/journal.pone.0195594. eCollection 2018.
Increasing evidence suggests the presence of structural changes affecting the right ventricular outflow tract (RVOT) in patients with Brugada Syndrome (BrS). The aim of this study was to characterise the RV morphology in BrS and explore associations between morphologic, clinical, electrical, and genetic parameters using non-invasive multimodality testing.
Consecutive BrS patients (recruited 2013-2015) underwent clinical assessment, dedicated RV imaging using cardiac magnetic resonance (CMR) imaging (unless contra-indicated), electrical assessment (electrocardiogram, Holter monitoring, signal-averaged ECG[SAECG]) and genotyping. Morphologic data were compared to matched control and unmatched ARVC (arrhythmogenic right ventricular cardiomyopathy) cohorts, and potential associations between morphologic parameters and other variables were explored.
BrS patients (n = 42, male 86%, age 46±12 years) exhibited normal global RV volume and function, comparable to control, in contrast to significantly larger, impaired RVs in ARVC cohort (RVESV p = 0.0001; RVEDV p<0.0001, RVEF p = 0.002). Compared with control, BrS patients exhibited larger RVOT volumes (7.4 ± 0.7 vs 5.8 ± 0.7 mL/m2, p<0.0001) and wall motion abnormalities (RWMA) (31% vs 0%, p = 0.005); compared with ARVC cohort, the RVOT volumes were similar (7.4 ± 0.7 vs, 8.1 ± 1.7, p = 0.52) and there were less RWMA (31% vs 76%, p = 0.01). Overall 67% BrS patients had abnormal RVOT morphology. Patients with abnormal RVOT tended to be older (48 ± 12 y vs 41 ± 12y, p = 0.06). Rare genetic variants were only observed in patients with abnormal RVOT morphology (36% vs 0%, p = 0.02).
Patients with BrS frequently exhibit structural abnormalities localised to the RVOT and these changes may be age- and gene-dependent.
越来越多的证据表明，Brugada 综合征（BrS）患者存在影响右心室流出道（RVOT）的结构变化。本研究的目的是使用非侵入性多模态检查来描述 BrS 中的 RV 形态，并探讨形态学、临床、电生理和遗传参数之间的相关性。
连续招募 2013-2015 年的 BrS 患者进行临床评估、心脏磁共振成像（CMR）（除非有禁忌证）进行专门的 RV 成像、电生理评估（心电图、动态心电图监测、信号平均心电图[SAECG]）和基因分型。将形态学数据与匹配的对照组和不匹配的 ARVC（致心律失常性右心室心肌病）队列进行比较，并探讨形态学参数与其他变量之间的潜在相关性。
BrS 患者（n=42，男性 86%，年龄 46±12 岁）的 RV 整体容积和功能正常，与对照组相当，但 ARVC 队列的 RV 明显增大且功能受损（RVESV p=0.0001；RVEDV p<0.0001，RVEF p=0.002）。与对照组相比，BrS 患者的 RVOT 容积更大（7.4±0.7 vs 5.8±0.7 mL/m2，p<0.0001）且存在更广泛的 RVOT 壁运动异常（RWMA）（31% vs 0%，p=0.005）；与 ARVC 队列相比，RVOT 容积相似（7.4±0.7 vs，8.1±1.7，p=0.52）且 RWMA 较少（31% vs 76%，p=0.01）。总体而言，67%的 BrS 患者的 RVOT 形态异常。RVOT 形态异常的患者年龄较大（48±12 y 与 41±12y，p=0.06）。异常 RVOT 形态的患者仅发现罕见的基因突变（36%与 0%，p=0.02）。
BrS 患者常出现 RVOT 局部结构异常，这些变化可能与年龄和基因有关。
