[ARVD-FORUM] Brugada-like ECG pattern. Dr. Perez Riera. Dr.
Schapachnik. Dr. Dubner
ARVD Symposium
info at arvd-symposium.org
Sat Apr 9 08:55:51 ART 2005
English - Portuguese
ARRHYTHMOGENIC RIGHT VENTRICULAR CARDIOMYOPATHY AND OTHERS CAUSES AS
PHENOCOPIES OF BRUGADA SIGNAL: BRUGADA-LIKE ECG PATTERN
Andrés Ricardo Perez Riera; Edgardo Schapachnik; Sergio Dubner
Brugada ECG pattern and Brugada sign have many phenocopies between them and
ARVD. There are drugs and clinical circumstances that may cause J point and
ST segment elevation that will probably generate diagnostic doubts by ECG
(Brugada-like ECG pattern (1;2)). They are called pseudo-pseudo-Brugada
syndrome (3).
Next, we will show the causes that may originate Brugada sign. In this list,
we find the entity that is the object of our current study: ARVD.
1) Idiopathic (4);
2) Early repolarization syndrome (ERS): syndrome observed in 1% to 2% of
adult population, and generally in absence of heart disease. It may mimick
acute infarction, pericarditis and rarely, BrS pattern. Diagnosis of ERS
must always be ruled out in the context of the presence of doubtful
precordial pain (5). This condition in approximately 8% of the cases among
athletes, may cause repolarization pattern, very similar to Brugada syndrome
(6;7;8);
3) Pectus excavatum: probably by chronic lesion in RVOT (9);
4) Coronary heart failure: during ischemia, depression of the ICa-L
channel and activation of IK-ATP is observed;
(4A) Vasospastic Prinzmetal variant angina developing into RVOT. Both
entities and myocardial bypass have been reported in the same patient (10);
(4B) Obstruction of left coronary trunk (11);
(4C) Acute infarction of right ventricle (12);
On the other hand, the syndrome may mimick acute infarction (13);
5) Ventricular aneurysm;
6) Dissecting aneurysm of the aorta;
7) Pulmonary embolism (14);
8) Hyperpotassemia may mimick BrS manifestations (15). Severe
electrolytic alterations may mimick ECG phenotype of BrS (16);
9) Hypercalcemia: this dyselectrolytemia may cause the presence of
idiopathic J wave similar to Brugada sign. The presence of short QT interval
at the expense of ST segment helps in the differential diagnosis. The
presence of milk-alkali syndrome may help in diagnosis (17;18;19);
10) Certain drugs may induce Brugada electrocardiographic phenotypical
pattern (20). These are considered as "acquired forms of the syndrome",
having recently been grouped in the following way by Dr. Wataru Shimizu
(modified from this author);
(10a) Antiarrhythmic drugs:
Sodium channel blockers:
Class IA: ajmaline, procainamide, disopyramide.
Class IC: flecainide, pilsicainide, propafenone.
Calcium antagonists: verapamil.
Beta-blockers: propranolol.
(10b) Anti-angina agents:
Calcium antagonists
Nitrates
Potassium channel openers
(10c) Psychotropic agents:
Tricyclic antidepressants
Tetracyclic antidepressants
Phenothiazine:
Selective serotonin reuptake inhibitors
(10d) Other drugs
H1 anti-histaminic receptor antagonists
Intoxication by cocaine: use or intoxication by cocaine. Accidental
administration of overdose or recreational ingestion of the drug may trigger
Brugada pattern of repolarization. The mechanism is due to block of the fast
sodium channel INa+ (20;21;22;23);
Bupivacaine anesthetics: by epidural via may induce appearance of Brugada
electrocardiographic pattern. Bupivacaine has the potential to induce
appearance of dangerous arrhythmias in patients carriers of BrS (24);
11) Increase of serum insulin level: it may increase ST segment elevation
in patients carriers of Brugada syndrome by increase of Ito ICa-L and
activation of Na+/K+ pump (25;26;27;28). A significant accentuation of
abnormal configuration of J-ST was observed in all the patients in a mean of
51 +/- 40 minutes after glycose and insulin infusion. The glycose and
insulin concentrations of blood may be factors that modulate circadian or
daily variations of ECG in this syndrome (29;30);
12) After transplantation of autologous peripheral stem cells to treat
acute myeloid leukemia, the syndrome may become manifest (31);
13) Vagal stimulation (32;33);
14) Acute cholecystitis (34);
15) Pericarditis (35);
16) Arrhythmogenic Right Ventricular Dysplasia (ARVD): certain forms
called concealed may present an electrocardiographic pattern
indistinguishable from true BrS (36);
17) Fever: it may trigger an event in a BrS carrier. Dumaine et al, showed
for the first time that the ECG of patients carriers of BrS present
arrhythmogenicity only with temperatures close to the physiological range,
what suggests that in some patients there may be a higher risk during
febrile states (37;38). In other words, ion mechanisms responsible for the
electrocardiographic phenotype in BrS are dependent on temperature
(39;40;41). Monk et al., demonstrated clinically that in the mutation
SCN5A-H681P, fever causes appearance of electrocardiographic phenotype
pattern of BrS (42). There are references that febrile state may trigger
incessant electric storm, difficult to reverse, which may lead to death
(43). This evidence points out the need of new therapeutic strategies (44).
Morita et al, observed in a patient carrier of BrS, besides worsening of ST
segment elevation, appearance of T wave alternans and ventricular
extrasystoles that disappeared when the patient's fever subsided (45).
18) Hypothermia: it causes appearance of idiopathic J wave or Osborn wave,
probably by increase of Ito channel (46). Experimentally, in canine models,
regional cooling of epicardium in RVOT (47);
19) Autonomous alterations of the central nervous system as in
subarachnoid hemorrhage, may originate Brugada-like J waves or similar to
hypothermia (48);
20) Steiner's myotonia;
21) Duchenne's muscular dystrophy;
22) Mechanical compression of RV outflow tract by tumor, infectious mass
or hemopericardium (49;50;51). The pressure exercised induces changes in the
functional state of ion channels in the epicardium of the RVOT (52);
23) Post-operative of Fallot's tetralogy correction.
24) During several hours after electrical cardioversion.
25) After surgical castration (53).
Many of these conditions are considered acquired forms of the syndrome.
----------------------------------------------------------
DISPLASIA ARRITMOGÊNICA DO VENTRÍCULO DIREITO E OUTRAS CAUSAS FENÓPIAS DO
SINAL DE BRUGADA: PADRÃO ECG SEMELHANTE AO BRUGADA
Andrés Ricardo Perez Riera; Edgardo Schapachnik; Sergio Dubner
O padrão EGG Brugada, e o sinal de Brugada possui numerosas fenocópias
entre elas a DAVD. Existem drogas e circunstâncias clínicas que podem
ocasionar supradesnivelamento do ponto J e do segmento ST que eventualmente
suscitam dúvidas, diagnósticas pelo ECG (Brugada-like ECG pattern (1; 2) Tem
sido denominadas pseudo-pseudo-Brugada syndrome (3).
A seguir mostramos as causas que podem ocasionar o sinal de Brugada Nesta
lista se encontra a entidade objeto de nosso atual estudo: a DAVD.
1) Idiopático (4) ;
2) Síndrome da Repolarização Precoce (SRP): A síndrome é observada em
1% a 2% da população adulta e geralmente na ausência de doença cardíaca Pode
simular infarto agudo, pericardite e raramente o padrão da SBr O diagnóstico
de SRP sempre deve ser de exclusão no contexto da presença de dor precordial
duvidoso (5). Esta condição em aproximadamente 8% dos casos entre atletas
pode ocasionar padrão de repolarização muito semelhante a síndrome de
Brugada6; (7; 8);
3) Pectus excavatum: provavelmente por lesão crônica na VSVD; (9) ;
4) Insuficiência coronária: Durante a isquemia se observa depressão do
canal ICa-L e ativação do IK-ATP.
(4A) Angina variante vasoespática de Prinzmetal envolvendo a VSVD.
Ambas entidades e ponte miocárdica tem sido referidas num mesmo paciente
(10);
(4B) Obstrução do tronco da coronária esquerda (11);
(4C) Infarto agudo do ventrículo direito (12);
Por outra parte a síndrome pode simular infarto agudo (13) ;
5) Aneurisma ventricular;
6) Aneurisma dissecante da aorta;
7) Embolia pulmonar (14);
8) Hiperpotassemia pode similar um quadro de SBr (15). Severas
alterações eletrolícas podem simular o fenótipo ECG da SBr (16);
9) Hipercalcemia: esta diseletrolitemia pode ocasionar a presença de
onda J idiopática semelhante ao sinal de Brugada. Ajuda no diagnóstico
diferencial a presença de intervalo QT curto as custas do segmento ST. A
presença de syndrome do leite alcalino ("milk-alkali syndrome") pode ajudar
no diagnóstico (17; 18; 19);
10) Certas drogas podem induzir o padrão fenotípico eletrocardiográfico
Brugada (20). Estas são consideradas como "formas adquiridas da síndrome,
tendo recentemente sido agrupadas da seguinte maneira pelo Dr Wataru Shimizu
(modificado de este autor)
(10a) Antiarritmicos
Bloqueadores dos canais de Sódio:
Classe IA: Ajmalina, procainamida, dispyramida.
Classe IC: Flecainide, pilsicainide, propafenona.
Antagonistas do cálcio: Verapamil
Beta-bloqueadores: propranolol.
(10b) Antianginosos
Antagonistas do cálcio:
Nitratos:
Abridores dos canais de potássio:
(10c) Psicotrópicos
Antidepressivos tricíclicos
Antidepressivos tetracíclicos
Fenotiazinas:
Inibidores da recaptação seletiva de serotonina:
(10d) Outras drogas
Antagonista dos receptores anti-histamínicos H1:
Intoxicação por cocaína: Uso ou Intoxicação por cocaína. A
administração acidental de sobredoses ou mesmo a ingestão recreacional da
droga pode desencadear o padrão Brugada de repolarização. O mecanismo obedece
a bloqueio do canal rápido de sódio INa+ (20; 21; 22; 23) ;
Anestésico bupivacaine: pela via epidural pode induzir o aparecimento do
padrão eletrocardiográfico Brugada. bupivacaina, possui potencial de induzir
ao aparecimento de arritmias perigoss em pacientes portadores da SBr (24);
11) Aumento do nível de insulina sérica: pode aumentar o
supradesnivelamento do segmento ST em pacientes portadores da síndrome de
Brugada por aumento do Ito ICa-L e da ativação da bomba Na+/K+ (25; 26; 27;
28). Um acentuatuação significativa da configuração anormal de J-ST foi
observado em todos os pacientes em um meio de 51 +/- 40 minutos após o
infusão de glicose e de insulina. As concentrações de glicose e de insulina
do sangue podem ser fatores que modulam as variações circadianas ou
cotidianas do ECG nesta síndrome (29; 30);
12) Após transplante de células tronco periféricas autólogas para o
tratamento de leucemia mielóide aguda pode por de manifesto a síndrome (31);
13) Estímulo vagal (32; 33);
14) Colecistite aguda (34);
15) Pericardite (35);
16) Displasia Arritmogênica do Ventrículo Direito (DAVD): Certas formas
denominadas ocultas pode apresentar um padrão eletrocardiográfico
indistinguível da verdadeira SBr (36);
17) Febre: pode desencadear um evento num portador da SBr. Dumaine et al,
demostraram pela primeira vez que o ECG dos pacientes portadores da SBr
apresentam sua arritmogenicidade apenas a temperaturas próximas da faixa
fisiológica o que sugere que em alguns pacientes podem ter maior risco
durante os estados febris (37; 38). Em outras palavras, os mecanismos
iônicos responsáveis pelo fenótipo eletrocardiográfico na SBr são dependentes
da temperatura (39; 40; 41) Monk e col demonstraram clinicamente que na
mutação, SCN5A-H681P a febre ocasiona o aparecimento do fenótipo
eletrocardiográfico da SBr (42). Há referência que o estado febril pode
desencadear tormenta elétrica incesante de difícil reversão que pode conduzir
a óbito (43). Estas evidências assinalam a necessidade de novas estratégias
terapêuticas (44).
Morita et al observararam num paciente portador da SBr além da piora do
supradesnivelamento do segmento ST o aparecimento de alternância na onda T
e extra-sístoles ventriculares que desapareceram quando o paciente ficou
afebril (45)
18) Hipotermia: ocasiona o aparecimento da onda J idiopática ou onda de
Osborn provavelmente por aumento do canal Ito (46). Experimentalmente no
modelo canino o esfriamento regional do epicárdio da VSVD (47);
19) Alterações autonômicas do Sistema nervoso central como na hemorragia
subaracnóide podem originar ondas J Brugada-like ou hipoermico simile (48);
20) Miotonia de Steiner;
21) Distrofia muscular de Duchenne;
22) Compressão mecânica da via de saída do VD por tumor, massa infecciosa
ou hemopericárdio (49; 50; 51); A pressão exercida induz a mudanças no estado
funcional dos canais iônicos no epicardio da VSVD (52);
23) Pós-operatório de correção na tétrade de Fallot.
24) Durante várias horas após cardioversão elétrica.
25) Após castração cirúrgica (53).
Muitas destas condições são consideradas formas adquiridas da síndrome
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