Materiały źródłowe

• #1 The Brugada Syndrome – Diagnosis, Clinical Implications and Risk Stratification

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6159405/

  The Brugada syndrome (BrS) is a hereditary arrhythmic syndrome manifesting as syncope or sudden cardiac death (SCD) in individuals without overt structural heart disease. Currently, its diagnosis is mainly based on the presence of a spontaneous or Na+-channel blocker induced so-called type 1 Brugada electrocardiographic (ECG) pattern typically seen in leads V1 and V2 recorded from the 4th to 2nd intercostal spaces. […] Currently there are no reliable methods to identify these potential victims of SCD. […] The standard 12-lead ECG (with some additional modifications, as explained below) is crucial for the diagnosis and likely also for determining the prognosis (i.e. the level of arrhythmic risk) in the BrS. The diagnostic hallmark of syndrome is the so-called coved or type 1 Brugada ECG pattern characterised by J-point elevation with slowly descending or concave ST segment elevation merging into a negative or reaching the isoelectric line symmetric T wave.

• #1 Brugada Syndrome Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/163751-guidelines

  In its 2013 expert consensus statement on inherited primary arrhythmia syndromes, the Heart Rhythm Society/European Heart Rhythm Association/Asia Pacific Heart Rhythm Society (HRS/EHRA/APHRS) recommended a diagnosis of Brugada syndrome (BrS) when the following criteria are met: ST-segment elevation with type I morphology 2 mm in 1 lead among the right precordial leads V1,V2 positioned in the 2nd, 3rd, or 4th intercostal space occurring either spontaneously or after provocative drug test with intravenous administration of Class I antiarrhythmic drugs […] In 2015, the European Society of Cardiology (ESC) released guidelines for the management of ventricular arrhythmias and the prevention of sudden cardiac death (SCD) that included the following specific recommendation for diagnosis of BrS. Brugada syndrome is diagnosed in patients with ST-segment elevation with type 1 morphology 2 mm in one or more leads among the right precordial leads V1 and/or V2 positioned in the second, third, or fourth intercostal space, occurring either spontaneously or after provocative drug test with intravenous administration of sodium channel blockers (such as ajmaline, flecainide, procainamide or pilsicainide)

• #1 The Diagnosis, Risk Stratification, and Treatment of Brugada Syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4498009/

  The saddle-back type 2 and type 3 ECG patterns are suspicious for, but not diagnostic of BrS. […] Asymptomatic patients should be reassessed at regular intervals; in particular, a spontaneous type 1 ECG indicates an elevated risk for ventricular arrhythmias. […] With the clinical diagnostic criterion no longer being used, risk stratification plays a critical role, both to identify patients at high risk of sudden cardiac death, who actually would benefit from placement of an implantable cardioverter-defibrillator (ICD), and to identify asymptomatic patients with a very low risk. […] Established risk markers are sudden cardiac arrest and a spontaneous type 1 ECG in association with the occurrence of arrhythmic syncope. […] Asymptomatic patients with BrS show, over the follow-up periods reported to date, a very low incidence of malignant arrhythmias.

• #1 Diagnostic drugs for Brugada syndrome | BrugadaDrugs.org

  https://www.brugadadrugs.org/druglist_diagnostic/

  These sodium channel blocking drugs are used to test for Brugada syndrome in patients suspected of Brugada syndrome but without a spontaneous type-1 Brugada ECG. […] To diagnose Brugada syndrome instead of solely a Brugada ECG, there are several other criteria which should be met. […] The diagnosis of the characteristic type-1 (or coved type) Brugada ECG is made from the right precordial ECG leads. […] Sensitivity of the ECG can be increased with alternative placement of ECG leads to the intercostal space above V1 and V2.

• #1 Brugada Syndrome Workup: Approach Considerations, Laboratory Studies, Genetic Testing

  https://emedicine.medscape.com/article/163751-workup

  Exercise stress testing may suppress ECG changes and arrhythmias. […] According to a consensus report, the type 1 ECG pattern is diagnostic of Brugada syndrome. […] Recently, the QRS duration on 12-lead ECG has been suggested as a risk marker for vulnerability to dangerous arrhythmias. […] Asymptomatic patients with a type 1 ECG pattern on routine ECG represent a difficult case. According to the latest consensus guidelines, a clinical electrophysiologist should evaluate patients in this situation. […] In some patients, the intravenous administration of drugs that block sodium channels may unmask or modify the ECG pattern, aiding in diagnosis and/or risk stratification in some individuals. […] This drug test should not be performed in patients with a type 1 ECG pattern because it adds no new information.

• #1 Brugada Syndrome Workup: Approach Considerations, Laboratory Studies, Genetic Testing

  https://emedicine.medscape.com/article/163751-workup

  In patients with the type 2 or 3 patterns, the drug challenge is recommended to clarify the diagnosis. […] Some investigators use an electrophysiologic study (EPS) to determine the inducibility of arrhythmias, in an effort to risk-stratify patients with Brugada syndrome. However, the predictive value of this approach is debated. […] Significant predictors of arrhythmia in this study included syncope and a spontaneous type I ECG pattern, a ventricular effective refractory period of less than 200 ms on EPS, and a fragmented QRS in the anterior precordial ECG leads.

• #1 Brugada Syndrome Workup: Approach Considerations, Laboratory Studies, Genetic Testing

  https://emedicine.medscape.com/article/163751-workup

  Many patients with Brugada syndrome are young and otherwise healthy and may present with syncope. Patients with syncope should not be assumed to have a benign condition, and a 12-lead ECG should be performed. […] A drug challenge with a sodium channel blocker should be considered in patients with syncope in whom no obvious cause is found. An experienced physician should interpret the ECGs, and an electrophysiologist should review them if possible. […] Further testing may be indicated to exclude other diagnostic possibilities. […] Check serum potassium and calcium levels in patients presenting with ST-segment elevation in the right precordial leads. Both hypercalcemia and hyperkalemia may generate an ECG pattern similar to that of Brugada syndrome. […] Laboratory markers, such as creatine kinase-MB (CK-MB) and troponin, should be checked in patients who have symptoms compatible with an acute coronary syndrome. Elevations indicate cardiac injury.

• #1 Management of patients with a Brugada ECG pattern

  https://www.escardio.org/Journals/E-Journal-of-Cardiology-Practice/Volume-7/Management-of-patients-with-a-Brugada-ECG-pattern

  A drug challenge for the diagnosis of BS is indicated in cases in which the disease is suspected, but in which the basal ECG is normal (e.g. familial screening) or suspicious, but not diagnostic (types 2 or 3). […] BS is definitely diagnosed when the patient presents: 1) a type 1 ECG (either spontaneously or elicited by Class I AADs) and 2) at least one of the above-mentioned clinical criteria. […] Once the diagnosis of BS is made, the next step is risk stratification for which the main objective is the accurate identification and treatment of individuals at high risk of SCD. […] To date, the only proven effective therapeutic strategy for the prevention of SCD in BS patients is the ICD. […] Genetic testing, when available, is recommended to support clinical diagnosis, early detection of other affected family members and for research purposes.

• #1 Brugada syndrome – causes, symptoms and treatment | healthdirect

  https://www.healthdirect.gov.au/brugada-syndrome

  Brugada syndrome can be diagnosed by a heart specialist. […] Brugada syndrome is often diagnosed by an electrocardiogram (ECG) test showing the characteristic 'Brugada pattern’. Your doctor may identify this during a routine ECG test without you having any symptoms. […] Other tests to look at your heart, such as an echocardiogram, may also be done. […] Sometimes a test is done where a specific medicine is given before an ECG. This is to see if it brings on changes in your ECG. This test is done in hospital so you can be monitored for symptoms and any problems can be treated quickly. […] Genetic testing will also be recommended.

• #1 The Brugada Syndrome – Diagnosis, Clinical Implications and Risk Stratification

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6159405/

  Currently the BrS is definitely diagnosed when type 1 pattern is observed in at least one of leads V1 and V2 recorded from the 4th, 3rd or 2nd i.c. space either spontaneously or following administration of Na+channel. […] The presence of gene mutations is not considered essential for the diagnosis. […] The identification of BrS patients with high arrhythmic risk especially among those without previous history of arrhythmia-related symptoms is currently the most important and yet unresolved clinical problem in the BrS. […] In BrS patients with a previous history of arrhythmic syncope or aborted cardiac arrest the annual event rate of sustained VT or VF is relatively high between 1.9 % and 8.8 % and between 7.7 % and 13.8 %, respectively. […] The majority of BrS patients (64 % in the largest reported series of 1029 BrS patients) have no symptoms at the time of establishment of the diagnosis. […] Currently, there are no firmly established reliable methods for the identification of these patients.

• #1 Brugada Syndrome Workup: Approach Considerations, Laboratory Studies, Genetic Testing

  https://emedicine.medscape.com/article/163751-workup

  Patients with high likelihood of Brugada syndrome may be genetically tested for a mutation in SCN5A, which codes for the alpha subunit Nav 1.5 of the cardiac sodium channel. The results of this test support the clinical diagnosis and are important for the early identification of family members at potential risk. However, the yield of genetic testing remains relatively low at this time, with mutations in SCN5A found in only 11-28% of index cases. […] Echocardiography and/or MRI should be performed, mainly to exclude arrhythmogenic right ventricular cardiomyopathy. However, these studies are also used to assess for other potential causes of arrhythmias, such as hypertrophic cardiomyopathy, unsuspected myocardial injury, myocarditis, or aberrant coronary origins. […] Three ECG patterns have been described in Brugada syndrome. Placing the right precordial leads in the second intercostal space has been proposed to add sensitivity to the ECG diagnosis of Brugada syndrome.

• #1 Invitae Brugada Syndrome Test | Test catalog | Invitae

  https://www.invitae.com/us/providers/test-catalog/test-02212

  This test is for individuals with a clinical diagnosis of Brugada syndrome. […] Individuals with clinical symptoms of Brugada syndrome may benefit from diagnostic genetic testing to establish or confirm diagnosis, clarify risks, or inform management. […] Brugada syndrome is defined by characteristic ST-segment elevations on an electrocardiogram (ECG). […] Individuals with Brugada syndrome may have symptoms related to arrhythmia, such as dizziness, syncope (fainting), or cardiac arrest. […] This test covers the most common genetic cause of Brugada syndrome: pathogenic variants in the gene SCN5A. […] Pathogenic variants in the SCN5A gene account for 15%-30% of Brugada syndrome cases.

• #1 Brugada Syndrome Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/163751-guidelines

  Consider comprehensive or BrS1 (SCN5A) targeted genetic testing for individuals with strong clinical index of suspicion for BrS based on clinical history, family history, and expressed electrocardiographic phenotype (class IIa) […] Mutation specific genetic testing for family members following identification of BrS mutation in an index case (class I) […] Genetic testing is not indicated in the setting of an isolated type 2 or type 3 Brugada ECG pattern (class III).

• #1 Brugada syndrome | Great Ormond Street Hospital

  https://www.gosh.nhs.uk/conditions-and-treatments/conditions-we-treat/brugada-syndrome/

  Brugada syndrome is an inherited condition caused by a change in a persons DNA. […] The condition can put affected individuals at increased risk of developing an abnormal heart rhythm, known as an arrhythmia. […] It is therefore important that the condition is detected early so that doctors can identify and treat those patients who have a higher risk of developing dangerous heart rhythms. […] An electrocardiogram (ECG) is one of the main tests for Brugada syndrome. […] Some people with Brugada syndrome have a very typical pattern on their ECG at rest, and this is sufficient to make a diagnosis. […] In other individuals, an ECG may not show the typical abnormalities, and a different test called an ajmaline provocation test might be suggested. […] The ajmaline test is widely used by specialist cardiologists around the world to help diagnose Brugada syndrome in adults and teenagers. […] It is not always possible to use genetic testing to diagnose Brugada syndrome, because we do not know all of the gene changes which can cause the condition. Clinical screening with ECGs and ajmaline testing are the recommended means of diagnosis.

• #1 Brugada Syndrome: Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/16813-brugada-syndrome

  To make a Brugada syndrome diagnosis, a healthcare provider will: […] Tests for diagnosing Brugada syndrome include: […] Genetic testing: Using your saliva or blood sample, this test can confirm that you have a specific genetic variation that indicates Brugada syndrome. […] Electrocardiogram (EKG): This test records the electrical activity that makes your heart beat. Brugada syndrome type 1 describes a specific EKG pattern a provider sees in your results. […] EKG with medication: Before doing an EKG, your provider may give you medication that helps the Brugada pattern show up on the EKG results if you have the condition. […] Based on your EKG results, you may also have: […] Electrophysiology (EP) testing: Providers place catheters inside your heart through your femoral vein in your leg. […] Lab tests: Blood tests check for a normal electrolyte balance, like potassium, calcium and magnesium, and can rule out other causes of an abnormal heart rhythm.

• #1 Brugada Syndrome: Progress in Diagnosis and Management | AER Journal

  https://www.aerjournal.com/articles/brugada-syndrome-progress-diagnosis-and-management?language_content_entity=en

  Brugada syndrome (BrS) represents an inherited disorder associated with risk of sudden cardiac death due to VF in patients without structural heart disease. Currently, BrS is diagnosed by typical cove-shaped ST-segment elevation 2 mm in 1 RV precordial lead V1, V2 occurring spontaneously or after a sodium-channel blocker provocation test without any further evidence of malignant arrhythmias. […] An electrophysiological study may be performed in asymptomatic patients with spontaneous type 1 ECG to assess the need for an ICD. […] In 2012, an expert consensus panel clarified ECG characteristics and diagnostic criteria and established two ECG patterns for BrS. Type 1 (coved-type) represents the only diagnostic pattern for BrS, while type 2 (saddle-back type) is only suggestive of BrS. […] Further investigation is needed in cases where there is a suspicion of BrS (syncope, dizziness, agonal respiration, resuscitated cardiac arrest, family history of BrS or suggestive ECG pattern) but patients do not have a spontaneous type 1 ECG pattern.

• #1 The Definition of the Brugada Syndrome

  https://www.acc.org/latest-in-cardiology/ten-points-to-remember/2017/09/18/14/06/the-definition-of-the-brugada-syndrome

  The following are key points to remember from this review of the diagnostic criteria for the Brugada syndrome (BS): […] The contemporary definition of BS is the type 1 pattern in 1 right precordial lead. […] The type 1 pattern is diagnostic of BS whether occurring spontaneously or with an intravenous drug challenge using a class I antiarrhythmic drug. […] A type 2 pattern raises the suspicion of BS, but the diagnosis depends on the emergence of a type 1 pattern with a drug challenge. […] If the diagnosis is not clear-cut, vectorcardiography can help differentiate BS from other conditions such as early repolarization or right bundle branch block.

• #1 Brugada Syndrome – Diagnosis and Treatment : Emergency Care BC

  https://emergencycarebc.ca/clinical_resource/clinical-summary/brugada-syndrome-diagnosis-and-treatment/

  Brugada Syndrome – Diagnosis and Treatment […] Diagnosis = Type 1 ECG pattern (Brugada’s Sign) in combination with clinical features including family history of SCD. […] Recommended diagnostic decision aid is the Shanghai Score which considers ECG findings, clinical history, family history, and genetic testing. […] Diagnosis: probable if ≥ 3.5 points; possible if 2-3 points; nondiagnostic if <2 points. [...] RBBB and STEMI are common misdiagnoses.

• #1 Brugada Syndrome – Cardiovascular Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/cardiovascular-disorders/arrhythmogenic-cardiac-disorders/brugada-syndrome

  Brugada syndrome is an inherited channelopathy, characterized by large J-waves and coved ST elevation with associated T-wave inversion in leads V1-V3, that causes an increased risk of ventricular tachycardia (VT) and ventricular fibrillation (VF), leading to syncope and sudden death. Diagnosis is by ECG and often provocative electrodiagnostic testing and/or genetic testing. […] Diagnosis should be considered in patients with unexplained cardiac arrest or syncope or a family history of such when the affected people do not have structural heart disease. […] Initial diagnosis of Brugada syndrome is based on a characteristic ECG pattern, the type 1 Brugada ECG pattern. […] Only a spontaneous type 1 pattern is considered diagnostic for Brugada syndrome according to the Shanghai Brugada syndrome score.

• #1 Brugada syndrome – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/3000313

  Brugada syndrome (BrS) (also known as sudden unexplained [or unexpected] nocturnal death syndrome and as idiopathic ventricular fibrillation) should be suspected in any patient with a past history of any one or more of unexplained cardiac arrest, ventricular arrhythmia (e.g., documented polymorphic ventricular tachycardia or ventricular fibrillation) of unknown cause, or cardiogenic syncope. […] Diagnosis of BrS is considered probable or definite if spontaneous type 1 Brugada pattern is recorded on ECG from the 2nd to 4th intercostal spaces. Induced type 1 Brugada pattern (e.g., by fever, medications such as sodium-channel blockers, or by psychotropic drugs, alcohol, or illicit drugs) or type 2 or 3 Brugada pattern on the ECG is not diagnostic, and requires further investigation. However, diagnosis of BrS should be made by a specialist, and requires synthesis of ECG findings and features of the history (which should include relevant family history) and physical examination.

• #1 Brugada syndrome differential diagnosis – wikidoc

  https://www.wikidoc.org/index.php/Brugada_syndrome_differential_diagnosis

  Brugada syndrome should be differentiated from other cardiac disorders, electrolyte disturbances, and drug intoxication syndromes. The condition which most similarly presents to Brugada syndrome is arrhythmogenic right ventricular dysplasia, as they both cause sudden cardiac death in children. Brugada syndrome can be differentiated from arrhythmogenic right ventricular dysplasia by the genetic counterpart of SCN5A, the lack of structural abnormalities within the heart, the association with polymorphic ventricular tachycardia during sleep, and EKG changes that are enhanced by vagotonic agents. […] Although both Brugada syndrome and arrhythmogenic right ventricular dysplasia are associated with sudden cardiac death in young patients, the two syndromes are fairly easy to distinguish electrocardiographically and clinically.

• #1 Brugada Syndrome – Cardiovascular Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/cardiovascular-disorders/arrhythmogenic-cardiac-disorders/brugada-syndrome

  The role of electrophysiologic testing is currently debated. […] Genetic testing is usually recommended but has a yield of approximately 20%. […] The diagnosis of Brugada syndrome includes considering, and ruling out, other disorders that produce similar cardiac findings, including hypothermia, hypocalcemia, right bundle branch block, arrhythmogenic right ventricular cardiomyopathy, acute pulmonary embolism, and left anterior descending coronary artery of conus branch of the right coronary artery occlusion. […] Scoring systems have been developed to aid in determining if an ICD is appropriate therapy in such patients.

• #1

  https://www.gehealthcare.com/insights/article/diagnosis-and-management-of-patients-with-a-brugada-pattern-ecg?srsltid=AfmBOooSUjTYcZNPcm3tTcYDc20M9El4763Sqoqy3KTibM_iHSliKDMW

  The ECG is probably the single most important screening tool for Brugada pattern or Brugada Syndrome. […] Brugada Syndrome, which is marked by ECG changes and a history of sustained ventricular tachycardia or sudden cardiac death, is even rarer. […] ECG serves a critical role in the diagnosis of Brugada pattern. […] After my patient’s EP study showed no inducible type 1 ECG pattern, I told him that his risk of sudden cardiac death was low (although his family history was unknown), so no ICD was indicated. […] I also discussed the role of genetic testing and electrophysiologic testing with him, which may be appropriate to risk-stratify patients if there are inducible arrhythmias present. […] The negative predictive value of this finding is quite high. […] It also can be helpful to obtain a signal-averaged ECG to identify patients at risk of future arrhythmic events.

• #1 Brugada Syndrome • LITFL • ECG Library Diagnosis

  https://litfl.com/brugada-syndrome-ecg-library/

  Brugada Syndrome is a cardiac abnormality with a high incidence of sudden death in patients with structurally normal hearts. […] Diagnosis depends on a characteristic ECG finding AND clinical criteria. […] This is the only ECG abnormality that is potentially diagnostic. […] It is often referred to as Brugada sign. […] This ECG abnormality must be associated with one of the following clinical criteria to make the diagnosis: Documented ventricular fibrillation (VF) or polymorphic ventricular tachycardia (VT). […] The other two types of Brugada are non-diagnostic but possibly warrant further investigation. […] Pharmacological assessment has been suggested by some in Type 2 + 3 patterns, if Brugada syndrome is suspected clinically the administration of sodium channel blocking drugs may convert these non-diagnostic forms into the diagnostic type 1, however the sensitivity of this test is unknown and it would appear that this subgroup is at extremely low / no increased mortality when compared to the general population.

• #1 Brugada syndrome: ECG, clinical features and management – The Cardiovascular

  https://ecgwaves.com/topic/brugada-syndrome-ecg-treatment-type-1-2-3/

  The diagnosis is still missed despite obvious clinical presentation. […] It follows that Brugada syndrome is a likely diagnosis in patients presenting with these symptoms and typical ST-segment elevations in V1V3. […] If Brugada syndrome is suspected, the ECG changes may be induced pharmacologically during controlled circumstances. […] The characteristic ECG changes may be intermittent, which is why the diagnosis may be missed. […] The Brugada syndrome may present with three different ECG patterns, referred to as type 1, type 2, and type 2 Brugada syndrome ECG. […] For type 2 and type 3 ECG patterns, a diagnosis of Brugada syndrome is only established if the patient converts to type 1 ECG pattern upon administration of class I antiarrhythmic drugs. […] It is motivated to screen family members of patients with Brugada syndrome.

• #1 Brugada Syndrome

  https://www.pulsecardiology.org/cardiac-genetic-disease-1/brugada-syndrome

  Brugada Syndrome is diagnosed on the 12 lead ECG, usually in the context of symptoms or a typical family history. […] Sometimes the ECG changes of Brugada are discovered incidentally when an ECG is performed for another reason unrelated to the condition e.g. chest pain. […] A standard ECG may miss the typical changes of Brugada which will only appear when the ECG 'dots’ are moved to a higher location on the chest. […] As the ECG changes of Brugada come and go, a 'provocation study’ using medication (either ajmaline or flecainide) is sometimes used to bring out the typical ECG changes. […] The doctor may refer to your Brugada as (1) 'spontaneous’ – that is, it appears in the absence of fever or a 'provocation study’. (2) 'fever induced’ – appearing in the context of fever or (3) 'provoked’ – appearing when a drug like ajmaline or flecainide is given.

• #1 Brugada syndrome – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/3000313

  The diagnosis and management of BrS in children is not covered in this topic. […] Key diagnostic factors: past history of unexplained cardiac arrest or documented PMVT or VF, cardiogenic syncope. […] 1st investigations to order: ECG, echocardiogram. […] Investigations to consider: provocative drug testing with sodium channel blockade, genetic testing for BrS, advanced cardiac imaging (MRI or CT), invasive electrophysiological (EP) study with inducibility testing for ventricular arrhythmias.

• #1 Brugada syndrome: Clinical presentation, diagnosis, and evaluation – UpToDate

  https://www.uptodate.com/contents/brugada-syndrome-clinical-presentation-diagnosis-and-evaluation

  Brugada syndrome is a genetic disorder that can cause life-threatening ventricular tachyarrhythmias and thereby sudden cardiac arrest and sudden cardiac death. Patients have abnormal findings on the surface electrocardiogram (ECG) but do not usually have any apparent cardiac structural abnormalities. […] The clinical manifestations, evaluation, and diagnosis of Brugada syndrome will be reviewed here. […] Initial steps to diagnose Brugada syndrome include medical history, 12-lead ECG, and testing for underlying heart disease. […] Drug challenge for type 2 or equivocal ECG is indicated in certain cases. […] Risk stratification for those with uncertain diagnosis may involve signal-averaged ECG (SAECG), electrophysiology testing, insertable cardiac monitor, and genetic testing.

• #1 The r’-Wave Algorithm: A New Diagnostic Tool to Predict the Diagnosis of Brugada Syndrome after a Sodium Channel Blocker Provocation Test

  https://www.mdpi.com/1424-8220/23/6/3159

  A diagnosis of Brugada syndrome (BrS) is based on the presence of a type 1 electrocardiogram (ECG) pattern, either spontaneously or after a Sodium Channel Blocker Provocation Test (SCBPT). […] Diagnosis of BrS is made by electrocardiogram (ECG) when a Brugada type 1 pattern is present either spontaneously or after a Sodium Channel Blocker Provocation Test (SCBPT). […] The aim of our study was to test all previously proposed ECG criteria in a large cohort study and to evaluate an r’-wave algorithm for predicting a type 1 ECG pattern after an SCBPT. […] The r’-wave algorithm was proved to have the best diagnostic accuracy, compared with single electrocardiographic criteria, in predicting the diagnosis of BrS after provocative testing with flecainide. […] The r’-wave algorithm with a cut-off value of ≥2 showed a sensitivity of 90% and a specificity of 83%.

• #1 The Diagnosis, Risk Stratification, and Treatment of Brugada Syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4498009/

  A relatively new risk marker and sign of disturbed depolarization has been described in patients with BrS: a fragmented QRS complex (fQRS). […] According to the study results, a family history of sudden cardiac death or the presence of a SCN5A mutation has no prognostic impact and is therefore currently not included in risk stratification. […] In symptomatic BrS patients (aborted sudden cardiac death, documented VT with or without syncope), implantation of an ICD is clearly indicated. […] For asymptomatic BrS patients, individual risk assessment including consideration of other risk factors (age, sex, baseline ECG, and inducibility) is recommended.

• #1 Brugada Syndrome: Progress in Diagnosis and Management | AER Journal

  https://www.aerjournal.com/articles/brugada-syndrome-progress-diagnosis-and-management?language_content_entity=en

  Many patients with type 1 ECG pattern are asymptomatic. Therefore, the 2015 European Society of Cardiology (ESC) guidelines proposed a new diagnosis for BrS. This is essentially based on the typical ECG pattern, either spontaneous or after sodium-channel blocker, showing in at least one right precordial lead (V1 and V2) positioned in the second, third or fourth intercostal space, without requiring any evidence of malignant arrhythmia. […] Diagnosis of BrS requires exclusion of other causes of ST-segment elevation (Brugada phenocopies). […] Asymptomatic people are the majority (about 63%) of newly diagnosed Brugada patients. […] Unfortunately, for most patients the first symptom is cardiac arrest or sudden cardiac death. Therefore, risk stratification of asymptomatic patients is of utmost importance.

• #1 Brugada Syndrome – Melbourne Heart Rhythm

  https://melbourneheartrhythm.com.au/learn/conditions/52-brugada-syndrome

  Brugada Syndrome is a rare inherited cardiac arrhythmia syndrome that is characterized by a 'coved-shaped’ atypical right bundle branch pattern on a 12-lead ECG (Type-1 Brugada pattern ECG) and is associated with ventricular arrhythmias and sudden cardiac death. […] The diagnosis of Brugada Syndrome may also be made on family screening of patients with Brugada Syndrome or from a routine ECG. […] The most important sign is the presence of the diagnostic Type-1 Brugada pattern on an electrocardiogram, or ECG. Pattern, only detected on an ECG. Only Type 1 ECG pattern is diagnostic of Brugada Syndrome. […] The diagnosis of Brugada Syndrome can only be considered when a diagnostic Type-1 Brugada pattern ECG occurs spontaneously or following drug provocation. The diagnosis is further confirmed when the ECG pattern occurs in conjunction with one of the following: Documented Ventricular Fibrillation (VF), polymorphic VT, a family history of sudden cardiac death at <45 years, a diagnostic Type-1 Brugada pattern ECG in family members, inducibility of VT during an electrical study, syncope or nocturnal agonal respiration (attributed to self-terminating polymorphic VT or VF).

• #1 The Brugada Syndrome – Diagnosis, Clinical Implications and Risk Stratification | ECR Journal

  https://www.ecrjournal.com/articles/brugada-syndrome-diagnosis-clinical-implications-and-risk-stratification?language_content_entity=en

  The Brugada syndrome (BrS) is a hereditary arrhythmic syndrome manifesting as syncope or sudden cardiac death (SCD) in individuals without overt structural heart disease. Currently, its diagnosis is mainly based on the presence of a spontaneous or Na+-channel blocker induced so-called type 1 Brugada electrocardiographic (ECG) pattern typically seen in leads V1 and V2 recorded from the 4th to 2nd intercostal spaces. […] Currently there are no reliable methods to identify these potential victims of SCD. Although some ECG markers such as QRS fragmentation and infero-lateral early repolarisation have been demonstrated to signify increased arrhythmic risk their value still needs to be confirmed in large prospective studies. […] The standard 12-lead ECG (with some additional modifications, as explained below) is crucial for the diagnosis and likely also for determining the prognosis (i.e. the level of arrhythmic risk) in the BrS. The diagnostic hallmark of syndrome is the so-called coved or type 1 Brugada ECG pattern characterised by J-point elevation with slowly descending or concave ST segment elevation merging into a negative or reaching the isoelectric line symmetric T wave.

• #1 The Brugada Syndrome – Diagnosis, Clinical Implications and Risk Stratification | ECR Journal

  https://www.ecrjournal.com/articles/brugada-syndrome-diagnosis-clinical-implications-and-risk-stratification?language_content_entity=en

  Currently the BrS is definitely diagnosed when type 1 pattern is observed in at least one of leads V1 and V2 recorded from the 4th, 3rd or 2nd i.c. space either spontaneously or following administration of Na+channel. […] The identification of BrS patients with high arrhythmic risk especially among those without previous history of arrhythmia-related symptoms is currently the most important and yet unresolved clinical problem in the BrS. […] Currently the only class I indications for ICD implantation in patients diagnosed with the BrS endorsed by the 2013 HRS/EHRA/ APHRS Expert Consensus Statement is history of aborted cardiac arrest or documented spontaneous sustained VT, whereas syncope judged to be likely of arrhythmic origin is only a Class IIa indication which mainly reflects the difficulty of excluding a non-cardiac origin of syncope.

• #1 Brugada Syndrome

  https://www.pulsecardiology.org/cardiac-genetic-disease-1/brugada-syndrome

  If you have Brugada ECG changes which occur spontaneously but have no symptoms, the risk of cardiac arrest is a little less than 1% per year, ~ 7% over 10 years. […] If you do not have spontaneous ECG changes of Brugada, for instance if your ECG is only abnormal when you are given a drug like ajmaline or flecainide, then the risk of cardiac arrest over 10 years is ~ 2%. […] Historically, electrophysiology studies, or the presence of other ECG markers (such as fragmentation, or early repolarisation), have been used to refine the risk of arrhythmia in an individual.

• #1 Brugada Syndrome: Progress in Diagnosis and Management | AER Journal

  https://www.aerjournal.com/articles/brugada-syndrome-progress-diagnosis-and-management?language_content_entity=en

  Syncope in combination with a spontaneous type 1 ECG pattern is a universally accepted risk factor because up to 62% of symptomatic BrS patients will experience a new event 4884 months after diagnosis, leading to sudden death. […] The recent guidelines neither encourage nor discourage electrophysiological study and VT/VF inducibility patterns for BrS stratification in patients with BrS.

• #1 Brugada Syndrome: Diagnosis, Clinical Manifestations, Risk Stratification and Treatment – Nova Science Publishers

  https://novapublishers.com/shop/brugada-syndrome-diagnosis-clinical-manifestations-risk-stratification-and-treatment/

  Electrocardiographically characterized by a distinct ST-segment elevation in the right precordial leads, Brugada syndrome (BrS) is an arrhythmogenic disease reported to be responsible for at least 4% of all sudden deaths (at least 20% of sudden deaths in those without structural heart disease), and is a leading cause of death in subjects under the age of forty years. […] The diagnosis is mainly based on electrocardiographic features and may be hampered by incomplete penetrance, particularly because of dynamic ECG manifestations. […] Identifying patients with BrS at risk of malignant arrhythmias and sudden cardiac death remains the most important objective and therefore the fundamental question remains on the best strategy for assessing the real disease-associated arrhythmic risk, especially in asymptomatic patients.

• #1 Brugada Syndrome: Diagnosis, Clinical Manifestations, Risk Stratification and Treatment – Nova Science Publishers

  https://novapublishers.com/shop/brugada-syndrome-diagnosis-clinical-manifestations-risk-stratification-and-treatment/

  An accurate individual risk stratification to select patients for ICD implantation should be accurately performed, particularly in asymptomatic patients. […] The study of the areas of delayed depolarization in patients with BrS and the procedure of catheter ablation of the substrate located in the anterior epicardial region therefore opens new horizons in regards to the management of patients with extremely frequent ventricular tachycardia/ventricular fibrillation (VT/VF), for patients at high risk who refuse the implantation of an ICD, and for a better prognostic stratification of patients with BrS not classified as high risk for SCD.

• #1 Psychological profile of patients with Brugada syndrome and the impact of its diagnosis and management | npj Cardiovascular Health

  https://www.nature.com/articles/s44325-024-00042-6

  Screening BrS patients for previous psychiatric illnesses is important for a proper follow over time. […] The psychological impact of out-of-hospital cardiac arrest in BrS patients is significant, as they may experience psychological distress, such as the development of depression and anxiety, cognitive impairment, and fatigue, which negatively affect the quality of life of both patients and their relatives. […] The recognition of varying levels of psychological distress over time holds significance as it may impact cardiac outcomes differently among patient groups. […] The diagnosis of Brugada syndrome can lead to psychological and mental health repercussions in several ways. […] A multidisciplinary approach including cardiologists and psychiatrists in order to establish the most suitable psychopharmacological treatment should be considered.

• #2 Brugada syndrome: Clinical presentation, diagnosis, and evaluation – UpToDate

  https://www.uptodate.com/contents/brugada-syndrome-clinical-presentation-diagnosis-and-evaluation

  Brugada syndrome is a genetic disorder that can cause life-threatening ventricular tachyarrhythmias and thereby sudden cardiac arrest and sudden cardiac death. Patients have abnormal findings on the surface electrocardiogram (ECG) but do not usually have any apparent cardiac structural abnormalities. […] The clinical manifestations, evaluation, and diagnosis of Brugada syndrome will be reviewed here. […] Initial steps to diagnose Brugada syndrome include medical history, 12-lead ECG, and testing for underlying heart disease. […] Drug challenge for type 2 or equivocal ECG is indicated in certain cases. […] Risk stratification for those with uncertain diagnosis may involve signal-averaged ECG (SAECG), electrophysiology testing, insertable cardiac monitor, and genetic testing.

• #2 The Brugada Syndrome – Diagnosis, Clinical Implications and Risk Stratification | ECR Journal

  https://www.ecrjournal.com/articles/brugada-syndrome-diagnosis-clinical-implications-and-risk-stratification?language_content_entity=en

  The Brugada syndrome (BrS) is a hereditary arrhythmic syndrome manifesting as syncope or sudden cardiac death (SCD) in individuals without overt structural heart disease. Currently, its diagnosis is mainly based on the presence of a spontaneous or Na+-channel blocker induced so-called type 1 Brugada electrocardiographic (ECG) pattern typically seen in leads V1 and V2 recorded from the 4th to 2nd intercostal spaces. […] Currently there are no reliable methods to identify these potential victims of SCD. Although some ECG markers such as QRS fragmentation and infero-lateral early repolarisation have been demonstrated to signify increased arrhythmic risk their value still needs to be confirmed in large prospective studies. […] The standard 12-lead ECG (with some additional modifications, as explained below) is crucial for the diagnosis and likely also for determining the prognosis (i.e. the level of arrhythmic risk) in the BrS. The diagnostic hallmark of syndrome is the so-called coved or type 1 Brugada ECG pattern characterised by J-point elevation with slowly descending or concave ST segment elevation merging into a negative or reaching the isoelectric line symmetric T wave.

• #2 The Diagnosis, Risk Stratification, and Treatment of Brugada Syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4498009/

  Brugada syndrome (BrS) is among the more common familial arrhythmia syndromes, with an estimated prevalence of 1 to 5 per 10 000 persons. […] According to the new diagnostic criteria, the diagnosis of BrS requires typical ECG changes in only one precordial lead. […] BrS is usually inherited in an autosomal dominant manner. […] To date, the diagnosis of BrS has required a typical type 1 ECG (coved type) in at least two precordial leads (V1-V3) and the presence of one clinical criterion: […] To increase diagnostic sensitivity, the expert consensus statement of 2013 on inherited arrhythmogenic diseases omits any clinical criterion and demands diagnostic ECG changes in only one right precordial lead. […] The new diagnostic criteria have already been validated in a first case series of patients with known BrS.

• #2 Management of patients with a Brugada ECG pattern

  https://www.escardio.org/Journals/E-Journal-of-Cardiology-Practice/Volume-7/Management-of-patients-with-a-Brugada-ECG-pattern

  A drug challenge for the diagnosis of BS is indicated in cases in which the disease is suspected, but in which the basal ECG is normal (e.g. familial screening) or suspicious, but not diagnostic (types 2 or 3). […] BS is definitely diagnosed when the patient presents: 1) a type 1 ECG (either spontaneously or elicited by Class I AADs) and 2) at least one of the above-mentioned clinical criteria. […] Once the diagnosis of BS is made, the next step is risk stratification for which the main objective is the accurate identification and treatment of individuals at high risk of SCD. […] To date, the only proven effective therapeutic strategy for the prevention of SCD in BS patients is the ICD. […] Genetic testing, when available, is recommended to support clinical diagnosis, early detection of other affected family members and for research purposes.

• #2 Brugada Syndrome Workup: Approach Considerations, Laboratory Studies, Genetic Testing

  https://emedicine.medscape.com/article/163751-workup

  Patients with high likelihood of Brugada syndrome may be genetically tested for a mutation in SCN5A, which codes for the alpha subunit Nav 1.5 of the cardiac sodium channel. The results of this test support the clinical diagnosis and are important for the early identification of family members at potential risk. However, the yield of genetic testing remains relatively low at this time, with mutations in SCN5A found in only 11-28% of index cases. […] Echocardiography and/or MRI should be performed, mainly to exclude arrhythmogenic right ventricular cardiomyopathy. However, these studies are also used to assess for other potential causes of arrhythmias, such as hypertrophic cardiomyopathy, unsuspected myocardial injury, myocarditis, or aberrant coronary origins. […] Three ECG patterns have been described in Brugada syndrome. Placing the right precordial leads in the second intercostal space has been proposed to add sensitivity to the ECG diagnosis of Brugada syndrome.

• #2 Brugada Syndrome: Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/16813-brugada-syndrome

  To make a Brugada syndrome diagnosis, a healthcare provider will: […] Tests for diagnosing Brugada syndrome include: […] Genetic testing: Using your saliva or blood sample, this test can confirm that you have a specific genetic variation that indicates Brugada syndrome. […] Electrocardiogram (EKG): This test records the electrical activity that makes your heart beat. Brugada syndrome type 1 describes a specific EKG pattern a provider sees in your results. […] EKG with medication: Before doing an EKG, your provider may give you medication that helps the Brugada pattern show up on the EKG results if you have the condition. […] Based on your EKG results, you may also have: […] Electrophysiology (EP) testing: Providers place catheters inside your heart through your femoral vein in your leg. […] Lab tests: Blood tests check for a normal electrolyte balance, like potassium, calcium and magnesium, and can rule out other causes of an abnormal heart rhythm.

• #2 Brugada Syndrome: Progress in Diagnosis and Management | AER Journal

  https://www.aerjournal.com/articles/brugada-syndrome-progress-diagnosis-and-management?language_content_entity=en

  Brugada syndrome (BrS) represents an inherited disorder associated with risk of sudden cardiac death due to VF in patients without structural heart disease. Currently, BrS is diagnosed by typical cove-shaped ST-segment elevation 2 mm in 1 RV precordial lead V1, V2 occurring spontaneously or after a sodium-channel blocker provocation test without any further evidence of malignant arrhythmias. […] An electrophysiological study may be performed in asymptomatic patients with spontaneous type 1 ECG to assess the need for an ICD. […] In 2012, an expert consensus panel clarified ECG characteristics and diagnostic criteria and established two ECG patterns for BrS. Type 1 (coved-type) represents the only diagnostic pattern for BrS, while type 2 (saddle-back type) is only suggestive of BrS. […] Further investigation is needed in cases where there is a suspicion of BrS (syncope, dizziness, agonal respiration, resuscitated cardiac arrest, family history of BrS or suggestive ECG pattern) but patients do not have a spontaneous type 1 ECG pattern.

• #2 Brugada Syndrome Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/163751-guidelines

  In its 2013 expert consensus statement on inherited primary arrhythmia syndromes, the Heart Rhythm Society/European Heart Rhythm Association/Asia Pacific Heart Rhythm Society (HRS/EHRA/APHRS) recommended a diagnosis of Brugada syndrome (BrS) when the following criteria are met: ST-segment elevation with type I morphology 2 mm in 1 lead among the right precordial leads V1,V2 positioned in the 2nd, 3rd, or 4th intercostal space occurring either spontaneously or after provocative drug test with intravenous administration of Class I antiarrhythmic drugs […] In 2015, the European Society of Cardiology (ESC) released guidelines for the management of ventricular arrhythmias and the prevention of sudden cardiac death (SCD) that included the following specific recommendation for diagnosis of BrS. Brugada syndrome is diagnosed in patients with ST-segment elevation with type 1 morphology 2 mm in one or more leads among the right precordial leads V1 and/or V2 positioned in the second, third, or fourth intercostal space, occurring either spontaneously or after provocative drug test with intravenous administration of sodium channel blockers (such as ajmaline, flecainide, procainamide or pilsicainide)

• #2 Brugada Syndrome – Diagnosis and Treatment : Emergency Care BC

  https://emergencycarebc.ca/clinical_resource/clinical-summary/brugada-syndrome-diagnosis-and-treatment/

  Brugada Syndrome – Diagnosis and Treatment […] Diagnosis = Type 1 ECG pattern (Brugada’s Sign) in combination with clinical features including family history of SCD. […] Recommended diagnostic decision aid is the Shanghai Score which considers ECG findings, clinical history, family history, and genetic testing. […] Diagnosis: probable if ≥ 3.5 points; possible if 2-3 points; nondiagnostic if <2 points. [...] RBBB and STEMI are common misdiagnoses.

• #2 Brugada Syndrome – Cardiovascular Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/cardiovascular-disorders/arrhythmogenic-cardiac-disorders/brugada-syndrome

  The role of electrophysiologic testing is currently debated. […] Genetic testing is usually recommended but has a yield of approximately 20%. […] The diagnosis of Brugada syndrome includes considering, and ruling out, other disorders that produce similar cardiac findings, including hypothermia, hypocalcemia, right bundle branch block, arrhythmogenic right ventricular cardiomyopathy, acute pulmonary embolism, and left anterior descending coronary artery of conus branch of the right coronary artery occlusion. […] Scoring systems have been developed to aid in determining if an ICD is appropriate therapy in such patients.

• #2 Brugada syndrome differential diagnosis – wikidoc

  https://www.wikidoc.org/index.php/Brugada_syndrome_differential_diagnosis

  Brugada syndrome should be differentiated from other cardiac disorders, electrolyte disturbances, and drug intoxication syndromes. The condition which most similarly presents to Brugada syndrome is arrhythmogenic right ventricular dysplasia, as they both cause sudden cardiac death in children. Brugada syndrome can be differentiated from arrhythmogenic right ventricular dysplasia by the genetic counterpart of SCN5A, the lack of structural abnormalities within the heart, the association with polymorphic ventricular tachycardia during sleep, and EKG changes that are enhanced by vagotonic agents. […] Although both Brugada syndrome and arrhythmogenic right ventricular dysplasia are associated with sudden cardiac death in young patients, the two syndromes are fairly easy to distinguish electrocardiographically and clinically.

• #2 Brugada Syndrome – Melbourne Heart Rhythm

  https://melbourneheartrhythm.com.au/learn/conditions/52-brugada-syndrome

  Brugada Syndrome is a rare inherited cardiac arrhythmia syndrome that is characterized by a 'coved-shaped’ atypical right bundle branch pattern on a 12-lead ECG (Type-1 Brugada pattern ECG) and is associated with ventricular arrhythmias and sudden cardiac death. […] The diagnosis of Brugada Syndrome may also be made on family screening of patients with Brugada Syndrome or from a routine ECG. […] The most important sign is the presence of the diagnostic Type-1 Brugada pattern on an electrocardiogram, or ECG. Pattern, only detected on an ECG. Only Type 1 ECG pattern is diagnostic of Brugada Syndrome. […] The diagnosis of Brugada Syndrome can only be considered when a diagnostic Type-1 Brugada pattern ECG occurs spontaneously or following drug provocation. The diagnosis is further confirmed when the ECG pattern occurs in conjunction with one of the following: Documented Ventricular Fibrillation (VF), polymorphic VT, a family history of sudden cardiac death at <45 years, a diagnostic Type-1 Brugada pattern ECG in family members, inducibility of VT during an electrical study, syncope or nocturnal agonal respiration (attributed to self-terminating polymorphic VT or VF).

• #2

  https://www.gehealthcare.com/insights/article/diagnosis-and-management-of-patients-with-a-brugada-pattern-ecg?srsltid=AfmBOooSUjTYcZNPcm3tTcYDc20M9El4763Sqoqy3KTibM_iHSliKDMW

  The ECG is probably the single most important screening tool for Brugada pattern or Brugada Syndrome. […] Brugada Syndrome, which is marked by ECG changes and a history of sustained ventricular tachycardia or sudden cardiac death, is even rarer. […] ECG serves a critical role in the diagnosis of Brugada pattern. […] After my patient’s EP study showed no inducible type 1 ECG pattern, I told him that his risk of sudden cardiac death was low (although his family history was unknown), so no ICD was indicated. […] I also discussed the role of genetic testing and electrophysiologic testing with him, which may be appropriate to risk-stratify patients if there are inducible arrhythmias present. […] The negative predictive value of this finding is quite high. […] It also can be helpful to obtain a signal-averaged ECG to identify patients at risk of future arrhythmic events.

• #2 Brugada Syndrome Workup: Approach Considerations, Laboratory Studies, Genetic Testing

  https://emedicine.medscape.com/article/163751-workup

  Many patients with Brugada syndrome are young and otherwise healthy and may present with syncope. Patients with syncope should not be assumed to have a benign condition, and a 12-lead ECG should be performed. […] A drug challenge with a sodium channel blocker should be considered in patients with syncope in whom no obvious cause is found. An experienced physician should interpret the ECGs, and an electrophysiologist should review them if possible. […] Further testing may be indicated to exclude other diagnostic possibilities. […] Check serum potassium and calcium levels in patients presenting with ST-segment elevation in the right precordial leads. Both hypercalcemia and hyperkalemia may generate an ECG pattern similar to that of Brugada syndrome. […] Laboratory markers, such as creatine kinase-MB (CK-MB) and troponin, should be checked in patients who have symptoms compatible with an acute coronary syndrome. Elevations indicate cardiac injury.

• #2 The r’-Wave Algorithm: A New Diagnostic Tool to Predict the Diagnosis of Brugada Syndrome after a Sodium Channel Blocker Provocation Test

  https://www.mdpi.com/1424-8220/23/6/3159

  ROC analysis of the validation cohort showed that the AUC of the r’-wave algorithm (AUC: 0.92; CI 0.85–0.99) was significantly better than the AUC of the β-angle (AUC: 0.82; 95% CI 0.71–0.92), the α-angle (AUC: 0.77; 95% CI 0.66–0.90), the DBT- 5 mm (AUC: 0.75; 95% CI 0.64–0.87), the DBT- iso (AUC: 0.79; 95% CI 0.67–0.91), and the triangle base/height (AUC: 0.61; 95% CI 0.48–0.75) (p < 0.001), making it the best predictor of a BrS diagnosis after an SCBPT.

• #2 The Definition of the Brugada Syndrome

  https://www.acc.org/latest-in-cardiology/ten-points-to-remember/2017/09/18/14/06/the-definition-of-the-brugada-syndrome

  The following are key points to remember from this review of the diagnostic criteria for the Brugada syndrome (BS): […] The contemporary definition of BS is the type 1 pattern in 1 right precordial lead. […] The type 1 pattern is diagnostic of BS whether occurring spontaneously or with an intravenous drug challenge using a class I antiarrhythmic drug. […] A type 2 pattern raises the suspicion of BS, but the diagnosis depends on the emergence of a type 1 pattern with a drug challenge. […] If the diagnosis is not clear-cut, vectorcardiography can help differentiate BS from other conditions such as early repolarization or right bundle branch block.

• #2 The Brugada Syndrome – Diagnosis, Clinical Implications and Risk Stratification | ECR Journal

  https://www.ecrjournal.com/articles/brugada-syndrome-diagnosis-clinical-implications-and-risk-stratification?language_content_entity=en

  Currently the BrS is definitely diagnosed when type 1 pattern is observed in at least one of leads V1 and V2 recorded from the 4th, 3rd or 2nd i.c. space either spontaneously or following administration of Na+channel. […] The identification of BrS patients with high arrhythmic risk especially among those without previous history of arrhythmia-related symptoms is currently the most important and yet unresolved clinical problem in the BrS. […] Currently the only class I indications for ICD implantation in patients diagnosed with the BrS endorsed by the 2013 HRS/EHRA/ APHRS Expert Consensus Statement is history of aborted cardiac arrest or documented spontaneous sustained VT, whereas syncope judged to be likely of arrhythmic origin is only a Class IIa indication which mainly reflects the difficulty of excluding a non-cardiac origin of syncope.

• #2 Brugada Syndrome: Progress in Diagnosis and Management | AER Journal

  https://www.aerjournal.com/articles/brugada-syndrome-progress-diagnosis-and-management?language_content_entity=en

  Many patients with type 1 ECG pattern are asymptomatic. Therefore, the 2015 European Society of Cardiology (ESC) guidelines proposed a new diagnosis for BrS. This is essentially based on the typical ECG pattern, either spontaneous or after sodium-channel blocker, showing in at least one right precordial lead (V1 and V2) positioned in the second, third or fourth intercostal space, without requiring any evidence of malignant arrhythmia. […] Diagnosis of BrS requires exclusion of other causes of ST-segment elevation (Brugada phenocopies). […] Asymptomatic people are the majority (about 63%) of newly diagnosed Brugada patients. […] Unfortunately, for most patients the first symptom is cardiac arrest or sudden cardiac death. Therefore, risk stratification of asymptomatic patients is of utmost importance.

• #2 Brugada Syndrome Workup: Approach Considerations, Laboratory Studies, Genetic Testing

  https://emedicine.medscape.com/article/163751-workup

  In patients with the type 2 or 3 patterns, the drug challenge is recommended to clarify the diagnosis. […] Some investigators use an electrophysiologic study (EPS) to determine the inducibility of arrhythmias, in an effort to risk-stratify patients with Brugada syndrome. However, the predictive value of this approach is debated. […] Significant predictors of arrhythmia in this study included syncope and a spontaneous type I ECG pattern, a ventricular effective refractory period of less than 200 ms on EPS, and a fragmented QRS in the anterior precordial ECG leads.

• #2 The Brugada Syndrome – Diagnosis, Clinical Implications and Risk Stratification

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6159405/

  Currently the BrS is definitely diagnosed when type 1 pattern is observed in at least one of leads V1 and V2 recorded from the 4th, 3rd or 2nd i.c. space either spontaneously or following administration of Na+channel. […] The presence of gene mutations is not considered essential for the diagnosis. […] The identification of BrS patients with high arrhythmic risk especially among those without previous history of arrhythmia-related symptoms is currently the most important and yet unresolved clinical problem in the BrS. […] In BrS patients with a previous history of arrhythmic syncope or aborted cardiac arrest the annual event rate of sustained VT or VF is relatively high between 1.9 % and 8.8 % and between 7.7 % and 13.8 %, respectively. […] The majority of BrS patients (64 % in the largest reported series of 1029 BrS patients) have no symptoms at the time of establishment of the diagnosis. […] Currently, there are no firmly established reliable methods for the identification of these patients.

• #2 The Diagnosis, Risk Stratification, and Treatment of Brugada Syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4498009/

  A relatively new risk marker and sign of disturbed depolarization has been described in patients with BrS: a fragmented QRS complex (fQRS). […] According to the study results, a family history of sudden cardiac death or the presence of a SCN5A mutation has no prognostic impact and is therefore currently not included in risk stratification. […] In symptomatic BrS patients (aborted sudden cardiac death, documented VT with or without syncope), implantation of an ICD is clearly indicated. […] For asymptomatic BrS patients, individual risk assessment including consideration of other risk factors (age, sex, baseline ECG, and inducibility) is recommended.

• #2 The Diagnosis, Risk Stratification, and Treatment of Brugada Syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4498009/

  The saddle-back type 2 and type 3 ECG patterns are suspicious for, but not diagnostic of BrS. […] Asymptomatic patients should be reassessed at regular intervals; in particular, a spontaneous type 1 ECG indicates an elevated risk for ventricular arrhythmias. […] With the clinical diagnostic criterion no longer being used, risk stratification plays a critical role, both to identify patients at high risk of sudden cardiac death, who actually would benefit from placement of an implantable cardioverter-defibrillator (ICD), and to identify asymptomatic patients with a very low risk. […] Established risk markers are sudden cardiac arrest and a spontaneous type 1 ECG in association with the occurrence of arrhythmic syncope. […] Asymptomatic patients with BrS show, over the follow-up periods reported to date, a very low incidence of malignant arrhythmias.

• #3 Brugada Syndrome – Cardiovascular Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/cardiovascular-disorders/arrhythmogenic-cardiac-disorders/brugada-syndrome

  The role of electrophysiologic testing is currently debated. […] Genetic testing is usually recommended but has a yield of approximately 20%. […] The diagnosis of Brugada syndrome includes considering, and ruling out, other disorders that produce similar cardiac findings, including hypothermia, hypocalcemia, right bundle branch block, arrhythmogenic right ventricular cardiomyopathy, acute pulmonary embolism, and left anterior descending coronary artery of conus branch of the right coronary artery occlusion. […] Scoring systems have been developed to aid in determining if an ICD is appropriate therapy in such patients.

• #3 The Brugada Syndrome – Diagnosis, Clinical Implications and Risk Stratification

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6159405/

  Currently the BrS is definitely diagnosed when type 1 pattern is observed in at least one of leads V1 and V2 recorded from the 4th, 3rd or 2nd i.c. space either spontaneously or following administration of Na+channel. […] The presence of gene mutations is not considered essential for the diagnosis. […] The identification of BrS patients with high arrhythmic risk especially among those without previous history of arrhythmia-related symptoms is currently the most important and yet unresolved clinical problem in the BrS. […] In BrS patients with a previous history of arrhythmic syncope or aborted cardiac arrest the annual event rate of sustained VT or VF is relatively high between 1.9 % and 8.8 % and between 7.7 % and 13.8 %, respectively. […] The majority of BrS patients (64 % in the largest reported series of 1029 BrS patients) have no symptoms at the time of establishment of the diagnosis. […] Currently, there are no firmly established reliable methods for the identification of these patients.

• #3 Brugada Syndrome: Progress in Diagnosis and Management | AER Journal

  https://www.aerjournal.com/articles/brugada-syndrome-progress-diagnosis-and-management?language_content_entity=en

  Many patients with type 1 ECG pattern are asymptomatic. Therefore, the 2015 European Society of Cardiology (ESC) guidelines proposed a new diagnosis for BrS. This is essentially based on the typical ECG pattern, either spontaneous or after sodium-channel blocker, showing in at least one right precordial lead (V1 and V2) positioned in the second, third or fourth intercostal space, without requiring any evidence of malignant arrhythmia. […] Diagnosis of BrS requires exclusion of other causes of ST-segment elevation (Brugada phenocopies). […] Asymptomatic people are the majority (about 63%) of newly diagnosed Brugada patients. […] Unfortunately, for most patients the first symptom is cardiac arrest or sudden cardiac death. Therefore, risk stratification of asymptomatic patients is of utmost importance.

• #3 The Diagnosis, Risk Stratification, and Treatment of Brugada Syndrome

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4498009/

  A relatively new risk marker and sign of disturbed depolarization has been described in patients with BrS: a fragmented QRS complex (fQRS). […] According to the study results, a family history of sudden cardiac death or the presence of a SCN5A mutation has no prognostic impact and is therefore currently not included in risk stratification. […] In symptomatic BrS patients (aborted sudden cardiac death, documented VT with or without syncope), implantation of an ICD is clearly indicated. […] For asymptomatic BrS patients, individual risk assessment including consideration of other risk factors (age, sex, baseline ECG, and inducibility) is recommended.

• #4 The Brugada Syndrome – Diagnosis, Clinical Implications and Risk Stratification

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6159405/

  Currently the BrS is definitely diagnosed when type 1 pattern is observed in at least one of leads V1 and V2 recorded from the 4th, 3rd or 2nd i.c. space either spontaneously or following administration of Na+channel. […] The presence of gene mutations is not considered essential for the diagnosis. […] The identification of BrS patients with high arrhythmic risk especially among those without previous history of arrhythmia-related symptoms is currently the most important and yet unresolved clinical problem in the BrS. […] In BrS patients with a previous history of arrhythmic syncope or aborted cardiac arrest the annual event rate of sustained VT or VF is relatively high between 1.9 % and 8.8 % and between 7.7 % and 13.8 %, respectively. […] The majority of BrS patients (64 % in the largest reported series of 1029 BrS patients) have no symptoms at the time of establishment of the diagnosis. […] Currently, there are no firmly established reliable methods for the identification of these patients.

• #4 Brugada Syndrome: Progress in Diagnosis and Management | AER Journal

  https://www.aerjournal.com/articles/brugada-syndrome-progress-diagnosis-and-management?language_content_entity=en

  Many patients with type 1 ECG pattern are asymptomatic. Therefore, the 2015 European Society of Cardiology (ESC) guidelines proposed a new diagnosis for BrS. This is essentially based on the typical ECG pattern, either spontaneous or after sodium-channel blocker, showing in at least one right precordial lead (V1 and V2) positioned in the second, third or fourth intercostal space, without requiring any evidence of malignant arrhythmia. […] Diagnosis of BrS requires exclusion of other causes of ST-segment elevation (Brugada phenocopies). […] Asymptomatic people are the majority (about 63%) of newly diagnosed Brugada patients. […] Unfortunately, for most patients the first symptom is cardiac arrest or sudden cardiac death. Therefore, risk stratification of asymptomatic patients is of utmost importance.

Zobacz więcej