logging in or signing up Brugada Syndrome drabee2009 Download Post to : URL : Related Presentations : Share Add to Flag Embed Email Send to Blogs and Networks Add to Channel Uploaded from authorPOINT lite Insert YouTube videos in PowerPont slides with aS Desktop Copy embed code: Embed: Flash iPad Dynamic Copy Does not support media & animations Automatically changes to Flash or non-Flash embed WordPress Embed Customize Embed URL: Copy Thumbnail: Copy The presentation is successfully added In Your Favorites. Views: 2665 Category: Education License: All Rights Reserved Like it (8) Dislike it (2) Added: April 22, 2009 This Presentation is Public Favorites: 2 Presentation Description No description available. Comments Posting comment... By: deepu84in (27 month(s) ago) THANK U Saving..... Post Reply Close Saving..... Edit Comment Close By: thongvilaipan (28 month(s) ago) ^^ Saving..... Post Reply Close Saving..... Edit Comment Close By: alikocholo (34 month(s) ago) i want it Saving..... Post Reply Close Saving..... Edit Comment Close By: bmartini (53 month(s) ago) may be you should be interested in my opinions: Bortolo Martini MD http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=601144 http://digilander.libero.it/martini_syndrome/ Saving..... Post Reply Close Saving..... Edit Comment Close Premium member Presentation Transcript Case 1 : : On arrival in hospital he was fully alert although he felt light headed and He has been investigated,the noncardic causes of syncop are excluded & the cardic enzymes & echo are normal BUT; (ECG) shows changes were identical to those seen in the ECGs performed three years previously ,how to corrolate ECG findings to clinical picture, what is next step for evaluation of this case ?? Case 1 : Dr .RABIE Z. AL-ANSI 40 year old man presented to the emergency department after losing consciousness while driving his car. He had felt nauseous and light headed before managing to slow down and stop. A witness who stopped to help found him slumped unconscious over the steering wheel, and he regained consciousness a few seconds later. Slide 2: The same Pt. underwent check up investigations before 3 years, he had no symptoms & the heart & other systems by clincal exam.were nomal.& imaging studies including echo show no abnormalites, though ECG shows specific changes ; does it has potential effect ?? Dr .RABIE Z. AL-ANSI Slide 3: Dr .RABIE Z. AL-ANSI BRUGADA SYNDROME Al-KWAIT University Hospital Sanaa yemen Brugada syndrome has recently attracted great interest ?? : Brugada syndrome has recently attracted great interest ?? First, The disease takes the lives, in many instaces as a first event, of previously healthy indiviuals in their forties,during their most productive years. Dr .RABIE Z. AL-ANSI why because of : Second ,once thought to be very rare, the BS is now recognized world wide & has a high prevalance in certain parts of world. Slide 5: Brugada syndrome (BS) is : an inherited form of cardiac arrhythmia and sudden death presenting with : a typical electrocardiographic pattern of ST segment elevation in leadsV1 to V3, and displaying a right bundle branch block QRS morphology Definition : It is most frequently diagnosed in YOUNG MALES (8:1 ratio of males:females) of Asian origin. Slide 6: Interestingly, the natives knew the problem for many decades. In the Northeast of Thailand, this form of death was known as Lai Tai (death during sleep). The indigenous believe that the young men died during sleep because widow ghosts came to take them away. Many young men actually dress still as women to go to sleep at night - with the hope that it would mislead the widow ghost. In Philippines the phenomenon was known as Bangungut (scream followed by sudden death during sleep) In Japan was known as Pokkuri (unexpected sudden death at night).. The first patient with this syndrome was seen in 1986. The first paper including 8 patients was published in 1992 Since then, there has been an exponential increase in the number of patients recognized all over the world History Of Brugada syndrome Slide 7: Brugada syndrome is affecting 5/10 000 of the general population, But ,is estimated to be responsible for at least 4% of all sudden deaths and at least 20% of sudden deaths in patients with structurally normal hearts the Brugada syndrome has progressed from being a rare disease to one that is second only to automobile accidents as a cause of death among young adults in some countries. The Incidence Antzelevitch et al,Brugada Syndrome A Decade of Progress,Circ. Res. 2002;91;1114-1118 Clinical Characteristics : Clinical Characteristics PT. presents with : The syncope and cardiac arrest that typically occurring in the 3ed and 4th decade of life, and usually at rest or during sleep. The manifestations of BS are due to : episodes of polymorphic ventricular tachycardia-ventricular fibrilations Dr .RABIE Z. AL-ANSI When the episodes terminate syncopal attacks. sudden death When the episodes are sustained Thus ,these manifestations can range widely: at one end of the spectrum we have asymptomatic individuals & at other end those who die suddenly. Slide 9: Brugada syndrome is definitively diagnosed when : Type 1 ECG (elevation of the J wave 2 mm with (a negative T wave )and ST segment that is coved type and gradually descending) in more than one right precordial lead (V1-V3)*, with or without administration of a sodium channel blocker) and A. and/or B. below: A at least one of the following findings: Documented ventricular fibrillation Self-terminating polymorphic ventricular tachycardia A family Hx of sudden cardiac death Coved-type ECGs in family members Electrophysiologic inducibility Syncope or nocturnal agonal respiration B An SCN5A mutation Diagnostic Criteria @ Update 2005 @ : Dr .RABIE Z. AL-ANSI The ECG manifestations of the Brugada syndrome The ECG manifestations of the Brugada syndrome, : The ECG manifestations of the Brugada syndrome, Dr .RABIE Z. AL-ANSI The dynamic ECG manifestations of the Brugada syndrome, : The dynamic ECG manifestations of the Brugada syndrome, ‘saddle back coved (type 1). Dr .RABIE Z. AL-ANSI Slide 13: sodium channel blockers, a febrile state, vagotonic agents, hyperkalemia, hypokalemia, hypercalcemia, and alcohol and cocaine toxicity The ECG manifestations of Brugada syndrome are often concealed & dynamic but can be unmasked or modulated by: Dr .RABIE Z. AL-ANSI Slide 14: 1, 2, 3, 4 min during i.v. infusion of 50 mg ajmaline. ‘saddle back (type 2) unmasked & becomes coved (type 1). Dr .RABIE Z. AL-ANSI The use of Drug challenge to unmask BS Slide 15: VF and sudden death in Brugada syndrome usually occur at rest and at night. Circadian variation of sympathovagal balance, hormones, and other metabolic factors are likely to contribute to this circadian pattern. Circadian pattern of VF episodes in patients with BS Dr .RABIE Z. AL-ANSI We can notice ventricular episodes increase at mid night due to Circadian pattern of BS Genetic Factors Underlying Brugada Syndrome : Genetic Factors Underlying Brugada Syndrome The first and only gene to be linked to Brugada syndrome is SCN5A, the gene that encodes for the subunit of the cardiac sodium channel gene. SCN5A mutations account for 18% to 30% of Brugada syndrome cases Dr .RABIE Z. AL-ANSI Genetic testing is recommended, however, to Support the clinical diagnosis, For early detection of relatives at potential risk, Electrophysiological (EP) study : Electrophysiological (EP) study Electrophysiological (EP) study: This test may be performed to determine the inducibility of arrhythmias in B S. The predictive value of the EP study is still debated. Risk Stratification and Risk Stratification : Risk Stratification 1. Patients have a relatively high risk for sudden arrhythmic death, even in the absence of a history of cardiac arrest: 8.2% experienced sudden death 2.Individuals with a spontaneously abnormal type 1 ECG carried a 7.7-fold higher risk of developing an arrhythmic event during a lifetime as compared with individuals in whom the ECG diagnostic of Brugada syndrome was evident only after sodium channel blocker challenge. Dr .RABIE Z. AL-ANSI Risk Stratification : 3. Programmed electrical stimulation that induces a sustainedventricular arrhythmia is the strongest marker of risk, associated with an 8-fold higher risk of (aborted) sudden death than in noninducible patients. Risk Stratification 5. Familial forms of the disease are not associated with a worse prognosis than are sporadic cases because a positive Dr .RABIE Z. AL-ANSI 4. Male gender is another risk factor for sudden death. Men had a 5.5-fold higher risk of sudden death than did women. Therapeutic Recommendations forBrugada Syndrome : Therapeutic Recommendations forBrugada Syndrome Currently, an ICD is the only proven effective treatment for the disease Treatment with pharmacological agents is usually unsuccessful Dr .RABIE Z. AL-ANSI The pharmacological approach : The pharmacological approach The only agent on the US market is quinidine. Dr .RABIE Z. AL-ANSI Class 1C antiarrhythmic drugs (eg, flecainide and propafenone) and class IA agents (eg,procainamide) are contraindicated Antiarrhythmic agents such as amiodarone and B-blockers have been shown to be ineffective. The pharmacological approach : The most recent drug, cilostazol, which normalizes the ST segment most likely by augmenting the calcium current (ICa), as well as by reducing I to secondary to an increase in heart rate. An experimental agent, tedisamil, with potent action to block I to among other outward currents has been suggested as a therapeutic candidate The pharmacological approach Dr .RABIE Z. AL-ANSI Slide 24: 1 Symptomatic patients displaying the type 1 BrugadaECG (either spontaneously or after sodium channel blockade) + presenting with aborted sudden death. Symptomatic patients : Dr .RABIE Z. AL-ANSI Pt. should undergo ICD implantation 2 Similar patients + presenting related symptoms such as syncope, seizure, or nocturnal agonal respiration also Recommendations for ICD implantation Slide 25: 1 Asymptomatic patients displaying a type 1 ECG + a family history of sudden cardiac death Asymptomatic patients : Dr .RABIE Z. AL-ANSI should be closely followed up should undergo EPS If inducible, then the patient should receive an ICD. 2 Asymptomatic patients who develop a type 1 ECG only after sodium channel blockade + no family history Recommendations for ICD implantation The Prognosis : The Prognosis prognosis of asymptomatic individuals : One third of patients having suffered from syncopal episodes develops a new episode of polymorphic ventricular tachycardia within 2 years. Dr .RABIE Z. AL-ANSI This syndrome has a very poor prognosis when left untreated. Untreated Brugada’s syndrome is associated with a 10% mortality per year with up to 40% of patients experiencing arrhythmias in the future. Slide 27: THANK YOU Dr .RABIE Z. AL-ANSI You do not have the permission to view this presentation. In order to view it, please contact the author of the presentation.