PracticeUpdate Cardiology Best of 2018

EXPERT OPINION 14

MY APPROACH to Evaluating a Patient With Newly Discovered Brugada Syndrome By Josep Brugada MD, PhD, FESC Dr. Brugada is a Cardiologist and Specialist in Biology and Sport Medicine. He is Medical Director of Hospital Clínic de Barcelona in Spain.

I f a patient who is supposed to have Brugada syndrome is referred to me, the first thing I do is to be sure that his ECG meets the criteria for a type I Brugada ECG, which is the only one that allows the diagnosis to be made. If this is the case, then I try to exclude any other condition that might mimic this ECG pattern. If the ECG does not meet the criteria but is suspi- cious (the so-called type II and type III ECG), I would consider performing a pharmaco- logical provocation test with IV ajmaline, flecainide, or procainamide if there are symptoms of syncope, if there is a family history of Brugada syndrome or a family history of unexplained sudden death at under 50 years of age. Any time an ECG is done while the patient has fever can be very useful and would be the equivalent of a provocative pharmacological test. Any of the ECGs done from now on should use a special ECG lead configuration to cover all of the right ventricular surface. I recom- mend to use leads V1–V2 in the second intercostal space, leads V3–V4 as V1–V2 in the third intercostal space, and leads V5– V6 as V1–V2 in the fourth intercostal space. Any of the leads can have a type I ECG pat- tern and would be considered diagnostic. Thus, we assume now that the patient has a type I ECG either spontaneously, during fever, or after drug test provocation and no other condition that might provoke the same type of ECG pattern. In this case, I initiate the risk-stratification process. I want to know what risk this patient has for a ventricular arrhythmia and eventual sudden cardiac death. I take into consider- ation several issues. The first one is clinical presentation: Has the patient presented

" …we have the very promising approach of using right ventricular epicardial radiofrequency ablation… I am pretty well convinced that, when the long-term results of this procedure become available, our approach to patients at intermediate risk will change… "

• Family history, knowing that no sta- tistical relationship has been made between family history and sudden death. However, if there is a strong family history of sudden death related to Brugada syndrome, I would clearly consider it as a risk factor, but also as a psychologically important factor for survivors in the family; • Spontaneous or drug-induced ECG abnormality , knowing that a sponta- neous abnormal ECG pattern might represent a poor situation; • Inducibility of arrhythmias during the electrophysiological investigation that I perform systematically any time I risk stratify a patient. Considering all of these factors, I will com- municate my treatment decision to the patient/family based on our own and oth- ers’ results that show that asymptomatic patients with a basal abnormal ECG have a probability of malignant arrhythmic events around 1% per year cumulative over 10 years. In our own series, inducible sus- tained ventricular arrhythmias identify the group at highest risk among asymptomatic individuals. In this case I will recommend, after extensive discussion with the patient/ family, implanting an ICD. In any of the scenarios listed above, I will instruct the patient to avoid drugs that might

with aborted sudden death? In this case, if the cardiac evaluation is negative for other conditions (particularly coronary artery dis- ease and right ventricular cardiomyopathy), I would recommend immediate implant of an ICD. If the clinical presentation is syn- cope, and the clinical history suggests a cardiogenic origin, I will still perform all nec- essary tests to exclude a noncardiogenic cause of the syncope, including a tilt test and an electrophysiological investigation. I would like to know conduction times and inducibility of atrial and ventricular arrhyth- mias. If, after all the tests, all results suggest a cardiogenic origin, I will recommend an ICD to avoid the possibility of sudden death. If I have doubts about the true car- diogenic origin of the syncope, I might, in agreement with the patient, implant a loop recorder instead of the ICD as a first option, and, only in the case of documented, I hope nonsustained, ventricular arrhythmias, I will implant an ICD. If the patient is asymptomatic at the moment of the diagnosis, I would consider different factors: • Gender, knowing that females have by far fewer ventricular arrhythmias than men ; • Age, knowing that very young and very old individuals who are asymptomatic have fewer arrhythmic events ;

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