Right Ventricle Morphology and Hemodynamics in BrS

Study Overview
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Brief Summary
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The study purpose is to evaluate the morphological, functional and electrophysiological characteristics of the right ventricle before and after ajmaline in patients diagnosed with Brugada syndrome as well as to correlate CMR findings and substrate size.

Detailed Description
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All consecutive patients with either spontaneous or ajmaline-induced BrS-ECG pattern will be screened. A total of 30 BrS patients and 30 normal age, sex and BSA matched normal controls will be selected and enrolled. Patients will perform cardiac magnetic resonance imaging to evaluate and compare morphological and functional characteristics of the 2 groups before and after ajmaline. BrS patients will also perform a standardized programmed ventricular stimulation protocol and electroanatomical mapping to determine the substrate size.

Official Title
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Morphological and Functional Characteristics of the Right Ventricle in Patients With Brugada Syndrome

Conditions
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Brugada Syndrome

Intervention / Treatment
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* Drug: Ajmaline 17-(Chloroacetate) Monohydrochloride


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Brugada syndrome with either spontaneous or ajmaline- induced type 1 ECG pattern Indication to ajmaline testing and programmed ventricular stimulation Age > or equal to 18 years Written informed consent Exclusion Criteria: Pregnancy Contraindication to CMRI or to ajmaline Live espectance < 12 months

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| BrS Group<br>Ajmaline 17-(Chloroacetate) Monohydrochloride | Drug: Ajmaline 17-(Chloroacetate) Monohydrochloride<br>* Ajmaline 17-(Chloroacetate) Monohydrochloride(1mg/kg in 5 minutes)<br>|
| No BrS group<br>Ajmaline 17-(Chloroacetate) Monohydrochloride | Drug: Ajmaline 17-(Chloroacetate) Monohydrochloride<br>* Ajmaline 17-(Chloroacetate) Monohydrochloride(1mg/kg in 5 minutes)<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Electrocardiographic changes | Changes of PR, QRS, and QT parameters, before and after ajmaline in Brugada patients as compared with control subjects | 1 day during Electrocardiography | 
| MRI changes of left and right ventricular function | MRI changes of left and right ventricular function before and after ajmaline in Brugada patients as compared with control subjects | 1day during MRI | 
| CMR parameters changes of the right and left ventricle function | Changes of right ventricular areal strain before and after ajmaline in Brugada patients as compared with controls | 1 day during CMR | 
| Electrical substrate changes of the right ventricle | electroanatomical epicardial mapping for substrate determination before and after ajmaline | 1 day after ICD implantation |