Magnetic Resonance Imaging Findings in Patients with Brugada Syndrome

Abstract

MRI Findings in Brugada Patients. Introduction: Cardiac magnetic resonance imaging (CMR) is a powerful diagnostic tool for evaluating cardiac structure and function. Recently, right ventricular wallmotion abnormalities were described using electron beam tomography in patients with Brugada syndrome. In the present study, we prospectively evaluated CMR findings in patients with Brugada syndrome compared to matched controls.Methods and Results: CMR was performed on 20 consecutive patients with proven Brugada syndrome. The imaging protocol included breath-hold dark blood prepared T1-weighted multislice turbo spin-echo and gradient-echo images. Ventricular volumes and dimensions were compared to age-and sex-matched normal volunteers. The right ventricular outflow tract area was significantly enlarged in patients with Brugada syndrome compared to controls (11 vs 9 cm 2 , P = 0.018). There was a trend to larger right ventricular end-diastolic and end-systolic volumes and lower right ventricular ejection fraction in patients with Brugada syndrome compared to controls. However, none of the differences reached significance (P = 0.3, P = 0.08, and P = 0.06, respectively). There was no statistically significant difference in the left ventricular parameters between patients and controls. High intramyocardial T1 signal similar to fat signal was observed in 4 (20%) of the 20 patients compared to none of the controls.Conclusion: The findings support the view that subtle structural changes, such as right ventricular outflow tract dilation may point to a localized arrhythmogenic substrate in patients with Brugada syndrome.

Endocardial Electrograms From the Right Ventricular Outflow Tract After Induced Ventricular Fibrillation in Patients With Brugada Syndrome

“…20 Recent papers report an overlap between BS and arrhythmogenic RV cardiomyopathy, [21][22][23] and electron-beam computed tomography and magnetic resonance imaging in patients with BS has revealed localized morphologic abnormalities and fat infiltration in the RV. 24,25 Furthermore, recent papers de- scribe histopathologic changes such as myocarditis, hypertrophy, fibrosis and fatty infiltration, in the RV. [26][27][28] BS has been defined as an autosomal dominant disease with incomplete penetrance and has been linked to mutations in the SCN5A gene encoding for the -subunit of the cardiac sodium channel.…”

The genetic basis of Brugada syndrome: A mutation update

“…Another contributor to the arrhythmic propensity may be the occurrence of structural changes in the heart tissue [Carlson et al, 1994;Meregalli et al, 2005]. The precise mechanism causing these has not, however, been clarified [Papavassiliu et al, 2004;Saffitz, 2005].…”

Surface ECG Characteristics of Ventricular Tachyarrhythmias Before Degeneration Into Ventricular Fibrillation in Patients With Brugada-Type ECG

“…Recent reports indicated an overlap between Brugada syndrome and arrhythmogenic RV cardiomyopathy, [33][34][35] and electron-beam CT and magnetic resonance imaging in patients with Brugada syndrome have revealed localized RV morphologic abnormalities and fat infiltration. 36,37) Furthermore, recent articles reported histopathologic changes such as myocarditis, hypertrophy, fibrosis, and fatty infiltration in the RV. [38][39][40] An animal study also found that mutation in the gene encoding SCN5A in mice resulted in impairment of atrial and ventricular conduction associated with myocardial rearrangement and fibrosis.…”