Brugada, or not Brugada, that is the question

Brugadaphobia

This post is also available in: Italiano (Italian)

“Brugadaphobia”… Between fiction and reality.

Brugadaphobia: The fear of Brugada Syndrome. I heard for first time the term “brugadaphobia” in an interesting and meaningful speech by Prof. Sami Viskin at VT2017 titled: “The Best Way to Treat Brugada Syndrome Patient is quinidine” [1].

brugadaphobiaFig. 1. Slide of Prof. Sami Viskin on "Brugadaphobia" .VT2017. "The best way to treat the patient with Brugada syndrome is quinidine". Between "fiction" and reality: It is interesting to note the "terrifying" perception given by an image which is nothing but the  shadow produced by the innocuous gesture of a small hand [1].

A definition of “brugadaphobia” may be as follows: constant fear of impending and sudden death, that afflicts asymptomatic patients who have been diagnosed or even only suspected of Brugada syndrome [2].

This terrifying fear, however, does not find any justification in reality, but is constantly fueled by the media, especially the web, and brings with it serious psychological consequences, as well as influencing the choices of patients, willing to undergo at every possible diagnostic tests and therapeutic procedures, sometimes also experimental, invasive and not without consequences.

The fiction… That feeds the “Brugadaphobia”:

Just look for the term “Brugada syndrome” on Google to get information like:

  • “Brugada’s syndrome: that heart that is Suddenly stops
  • “Brugada A Ticking time bomb In the chest… “
  • “Brugada syndrome… At least 50,000 deaths a year
  • “Sudden cardiac death. Here’s how the “Brugada” Kills Young Sportsmen “
  • “Brugada syndrome… It is one of the diseases Mainly Responsible for sudden cardiac death in young adults… “
  • “Brugada syndrome… Cause of deaths Sudden and devastating… In young people apparently Very healthy
  • etc…

Recently, in England, the news of a tragic and sudden death of a young man, that went viral over Facebook, led >10,000 British citizens to petition their parliament, requesting that all patients asymptomatic with diagnosis of syndrome of Brugada, could obtain the implantation of a defibrillator (ICD), even in the absence of clear indicators of increased arrhythmic risk [2].

petizioneFig.2. Petition to the British parliament for the possibility of being able to choose a defibrillator implant.

In Italy, the tragic death of a footballer, Davide Astori, has been associated by media with the Brugada Syndrome, before the medical investigations on the causes of this death were concluded and in the absence of any evidence to support that association. The news reported about it, have created very havoc in the patients diagnosed with Brugada Syndrome and in the families of young athletes, making appear the Brugada Syndrome as a disease that suddenly and unexpectedly kills young athletes perfectly healthy. To get an example of this news, just a simple search on Google:

  • “Davide Astori, Brugada syndrome” behind the captain’s death… “
  • “DAVIDE ASTORI KILLED BY BRUGADA SYNDROME?…”
  • “Brugada syndrome… Pathology that may have panned the life of David Astori.. “
  • “Death David Astori: Here is the syndrome that might have killed him…”
  • “Brugada’s syndrome: What is the sudden death that killed Astori…”
  • “Nurse dies in sleep: Brugada syndrome, as Astori”
  • “Brugada: After Astori dies footballer French 19 years old!”
  • “Brugada syndrome: Cyclist in cardiac arrest in France”
  • “Brugada… The disturbing pathology that killed David Astori…

The scientific evidence:

It’s true! every year, in Italy, about 50,000 people die for sudden cardiac death. However, it is important to specify that in most cases, particularly in individuals with age > 40 years, the cause of these deaths is an underlying cardiac defect such as coronary artery disease, valvulopathies, and heart failure. In younger subjects, the cause is mainly due to cardiomyopathies, some of them of genetic origin such as hypertrophic cardiomyopathy, dilated cardiomyopathy, and arrhythmogenic heart disease. In a smaller percentage, some of these deaths are caused by “channelopathies” on a genetic basis such as Long QT syndrome, Brugada syndrome, short QT syndrome or catecholaminergic polymorphic ventricular tachycardia. Other causes may be due to the use of drugs and/or narcotic substances. Finally, when cardiac arrest occurs in subjects without known heart disease, in most cases it is caused by asymptomatic Coronary Artery Disease [3].

Based on these evidences, it is easy to deduce that 50,000 sudden deaths per year, certainly do not mean 50,000 sudden deaths for Brugada! But Brugada syndrome can rarely be a cause of these deaths.

In the majority of the available studies (and in all recent studies), the incidence of arrhythmic events in asymptomatic individuals, with a diagnosis of Brugada syndrome, was 1% per year.

In the PRELUDE registry, the asymptomatic individuals with type 1 ECG pattern exhibited an annual rate of arrhythmic events of about 1% [6].

The recent study of Sacher et al., reported data on 166 asymptomatic individuals with Brugada Syndrome with an ICD who had an average follow-up period 77±42 months and the annual incidence of appropriate therapies was 1%. [7].

In a recent meta-analysis of 13 studies including 2,743 subjects, the annual event rate for asymptomatic individuals was 1% [8].

In the largest cohort study on Brugada syndrome (FINGER registry), the incidence of ventricular arrhythmic events in asymptomatic individuals was 0.5% per year [5], a level of risk that differs minimally from the risk of death for all causes In the general middle-aged population and which stands at 0.4% per year [9].

It is important to emphasize that asymptomatic patients who Brugada pattern type 1 occurring after pharmacological challenge (sodium channel blocking test) with a Class I antiarrhythmic agent (ajmaline, flecainide, procainamide or pilsicainide), have a lower arrhythmic risk compared to those who type 1 occurring spontaneously:

FINGER registry [5], reported that in asymptomatic individuals with type 1 pattern induced by pharmacological tests, the risk was 0.3% in 3 years.

Again, in the recent and larger “pooled Analisys” on Brugada syndrome, the arrhythmic risk, in 696 asymptomatic patients with drug-induced type 1 pattern, was 0.3% per year. Interestingly, the risk was low even though the inducibility of ventricular fibrillation at the electrophysiological study in these patients [10].

Recently, the Pedro Brugada’s group, published the new data relating to their register in 20 years of follow up (1992-2013). The arrhythmic risk in asymptomatic patients with drug-induced type 1 pattern was 0.4% per year [16]. Interestingly, this registry includes patients who have been followed since 1992. In the early period, patients with more “malignant” expressions of the disease were diagnosed. Conversely patients who have been diagnosed with Brugada syndrome in the last 10 years showed more benign expressions of the disease, which results in lower the long term risk [11].

But, there is very fundamental points: all the major studies available, including those mentioned, are based on registries consisting of patients with and without ICD and, when assessing the occurrence of cardiac arrest in the time, it was considered the appropriate shock of the ICD compared to the cardiac arrest itself. But, the ICD may operate appropriately to interrupt potentially non-fatal arrhythmias or which, if not treated, would still terminate spontaneously. The appropriate ICD shock, therefore, represents only a “surrogate” of cardiac arrest. It is therefore realistic to think that the actual risk of cardiac arrest in asymptomatic patients diagnosed with Brugada syndrome is less than 1% per year [4] [12].

About that, an important and recent meta-analysis and cumulative analysis of seven large prospective studies involving 1568 patients who had not received a prophylactic ICD in primary prevention. The patients were mainly at low risk (85% asymptomatic, and 50% with drug-induced type 1). This type of population, truly represents the majority of those who, as a result of cardiac evaluation, Brugada syndrome is suspected or  diagnosed. Well! In these subjects the incidence of sudden death was found to be the 0.38%The carriers of spontaneous type 1 patterns and the 0.06% In those who presented a type 1 pattern only after pharmacological tests! [4][12]

The reality of the facts…..

According to all scientific evidence, there is a very different reality from news what are reported by media and web, which then become idea, culture and collective fear! Brugada syndrome EXISTS! It manifests itself mainly with ventricular tachyarrhythmias that can often be spontaneously interrupted in some cases by generating a syncope (fainting), or, more rarely, can lead to cardiac arrest. In some of these rare cases, cardiac arrest may represent the first manifestation of the disease. It is a clinical entity defined about 25 years ago as an association between cardiac arrest and a peculiar electrocardiogram. Over the course of these 25 years, however, it has been widely demonstrated that the syndrome (understood as signs and clear symptoms), is a very rare condition, while the characteristic electrocardiogram is of relatively frequent detection in the population! It is also understood that this electrocardiogram may be of nonspecific significance and can also be found due to conditions other than Brugada syndrome, some of which are completely physiological. This is particularly the case when the typical electrocardiographic pattern is only found after pharmacological tests [17]. Nevertheless, a significant proportion of patients with low-risk arrhythmics has received over time an implantable defibrillator (ICD) in primary prevention. A recent cumulative analysis of the largest available studies, has shown that 1/3 asymptomatic patients with type 1 brugada pattern have received an ICD implant in primary prevention. Of these patients, 65% had a spontaneous ECG type 1 pattern, 35% had only a drug-induced pattern of type 1, whereas only 45% was symptomatic for syncope [13]. Today, the main reason for the implantation of a defibrillator (ICD) is “a positivity to the ajmaline test with ventricular fibrillation inducible to the electrophysiological study” [14], although the specificity of the pharmacological test and the positive predictive value of the electrophysiological study is still unclear [14]. Moreover, in this category of patients, the number of appropriate interventions by the ICD has been shown to be extremely low [5], compared to a high risk of complications relating to the implantation of the ICD, including a considerable proportion of inappropriate shocks. [7][15]

The consequences of “Brugadaphobia”….

Fear is a useful and functional mechanism! It is triggered when the senses perceive a harmful or potentially damaging stimulus to the organism, practically when a threat is incumbent. To fear follows a state of neurophysiological activation that allows the individual to respond to the initial stimulus through attack, avoidance-escape or at worst with a blockage.

The boundary between fear and phobia is in the adaptive function of the response, when the emotional instinct is triggered in a way inappropriate, that is without a real threat, Then it becomes a wrong and pathological mechanism.

In the specific case, the problem arises when, mainly because of unconsciousness and lack of knowledge, is generates unmotivated fear, which grows in sharing with the fears of others and that, in the end, fueled by an uncontrolled and massive flow of incorrect information turns into collective phobia! The “Brugadaphobia” is just a collective “evil”, which pushes the individuals involved to assume an extremely protective attitude, which translates into the desire to do whatever it takes, to take shelter from a terrifying but unrealistic threat, without considering that “whatever it takes” does not necessarily mean “whatever is best ” [2].

For this reason, patients wants undergo at any cost to every diagnostic test and therapy, without considering that in some cases certain diagnostic tests may be positive in the absence of disease with the  consequence of labelling healthy peoples as “diseased” or, as a consequence of the positivity of these examinations, to undergo invasive therapeutic procedures, often disabling in social and working life, and not without consequences, sometimes even serious. And when it happens that medical centers or specialists advise against, on the basis of current evidences, to undergo certain examinations or therapies, patients constantly changes center or specialist in a vicious circle, until  to achieve the objective. 

To conclude, some of my personal considerations….

Sudden death is a natural fact, it exists, and it happens for very diversified causes, some still unknown, and we must accept it! How it is necessary to accept that in life a certain degree of uncertainty exists. The main cause of sudden cardiac death is ischemic heart disease, yet it does not seem to generate similar fears or phobias in the population or that particular attention is paid to risk factors such as smoking, hypertension, obesity etc… that are certainly more risk factors than an occasional response of “Brugada Pattern” to the ECG! the only effective means, to prevent sudden death in the general population, as well as eliminate the risk factors for ischemic heart disease, is to diffuse, in a capillary way on the territory, the culture of first aid and the use of the semiautomatic/automatic external defibrillator!!! … I hope that “Brugadaphobia” will soon be replaced by embracing all and with full civic sense the culture of first aid, so that it becomes an integral part of our daily and social life!

Clearly, we need more certainty and less doubt about this disease, especially in the stratification of the arrhythmic risk in asymptomatic patients, in such a way as to be able to better identify and punctually the subjects actually at risk and lay off the majority of those who simply having an ECG  “a little particular”. I hope that scientific research will soon be able to give us more answers… Meanwhile….

… It’s time to a little break…. breathe slowly…. reflect!!!

Bibliography

[1] Sami Viskin. VT2017-Session VI-Sami Viskin-Debate The Best Way to Treat Brugada Syndrome Patient is Quinidine

[2] Sami Viskin. Radiofrequency Ablation of Asymptomatic Brugada Syndrome: Don’t Go Burning My Heart.

[3] Priori SG et al., 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: The Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC)
Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC)

[4] Delise P. et al., Clinical outcome of patients with the Brugada type 1 electrocardiogram without prophylactic implantable cardioverter defibrillator in primary prevention: a cumulative analysis of seven large prospective studies.

[5] Probst V. el al., Long-term prognosis of patients diagnosed with Brugada syndrome: Results from the FINGER Brugada Syndrome Registry.Circulation2010;121:635–643.

[6] Priori SG et al., Risk stratification in Brugada syndrome: results of the PRELUDE (PRogrammed ELectrical stimUlation preDictive valuE) registry.

[7] Sacher F. et al., Outcome after implantation of a cardioverter-defibrillator in patients with Brugada syndrome: a multicenter study-part 2.

[8] Faucher L. et. al., Prognostic value of programmed ventricular stimulation in Brugada syndrome according to clinical presentation: an updated meta-analysis of worldwide published data.

[9] Office for National Statistics. Mortality statistics. UK Gov National Statistics Series DH2 no.32. London: ONS, 2006.

[10] Sroubek J, Probst V, Mazzanti A, et al. Programmed ventricular stimulation for risk stratification in the Brugada syndrome: a pooled analysis. Circulation 2016;133:622–30.

[11] Casado-Arroyo R, Berne P, Rao JY, et al. Longterm trends in newly diagnosed Brugada syndrome: implications for risk stratification. J Am Coll Cardiol 2016;68:614–23.

[12] Delise P, Ten questions on Brugada syndrome. G Ital Cardiol (Rome). 2017 Nov;18(11):754-759.

[13] Delise P, Allocca G, Sitta N, DiStefano P. Event rates and risk factors in patients with Brugada syndrome and no prior cardiac arrest: acumulative analysis of the largest available studies distinguishing ICD-recorded fast ventricular arrhythmias and sudden death. Heart Rhythm 2014; 11: 252-258 [PMID: 24513918 DOI: 10.1016/j.hrthm. 2013.10.039]

[14] Viskin S. et al.,Everybody has Brugada syndrome until proven otherwise? Heart Rhythm. 2015 Jul;12(7):1595-8.

[15] Conte G,SieiraJ,CiconteG,etal.Implantablecardioverter-deibrillatortherapy
in Brugadasyndrome:a20-yearsingle-centerexperience.JAmCollCardiol
2015;65:879–888.

[16] Sieira J. et al., Long-term prognosis of drug-induced Brugada syndrome.Heart Rhythm. 2017 Oct;14(10):1427-1433

[17] Delise P. et al., Brugada type 1 electrocardiogram: Should we treat the electrocardiogram or the patient? World J Cardiol. 2017 Sep 26; 9(9): 737–741.

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2 Comments

  1. Emmanuel Kearney

    Thanks for that, that is a read I really needed, it placed some sense and reality back in my life, thanks again

  2. Baturalp

    Brugadaphobia is the thing why I can not sleep properly for weeks. Thanks to enlighten us.

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