Brugada Syndrome: Sudden Cardiac Death | Literature Review

Brugada Syndrome choppy Cardiac cobblers last belles-lettres ReviewBRUGADA SYNDROME SUDDEN CARDIAC DEATHReview of Literature and Case Report.Dr. Nanda PaiDr. Sanjeeta UmbarkarDr. Akshay BafnaDr. Jinal VaghelaKEYWORDSBrugada Syndrome, fast unexpected decease syndrome (SUDS)ABSTRACTBrugada Syndrome or explosive Unexpected Death Syndrome was first discovered by P. Brugada and J. Brugada in 19921. It is a r be genetic disorder characterised by ST incision f raze in V1 V3 leads on cardiogram, ventricular fibrillation and ventricular arrhythmias which ignore cause sudden unexpected cobblers last in an otherwise normal unhurried. We wish to highlight the event that with thorough pre-operative anaesthetic and cardiac evaluation these potentially life operose diligents can be effectively treated for minor oral working(a) single-valued functions using regional anaesthesia with lignocaine thereby avoiding general anaesthesia.INTRODUCTIONIn 1992, Pedro and Josep Brugada for the first time introduced a new clinical entity with ST segment nurture in V1 V3 leads and right bundle branch pig out (RBBB) pattern associated with a high incidence of ventricular tachycardia/ventricular fibrillation (VT/VF)1. This new entity was termed Brugada Syndrome (BrS) or Sudden Unexpected Death Syndrome (SUDS), occurring in structurally healthy hearts in young individuals, causing life threatening arrhythmias and sudden death. Most of the patients are between second and fourth decades of life however the youngest patient clinically diagnosed with the syndrome is 2 days old and the oldest is 84 years old2. at that place is a male predilection, due to the presence of more prominent Ito conduct in males than in females3 and in many countries it is the 2nd highest cause of death in younger men later vehicular accidents.Signs and symptoms include presyncopal and syncopal attacks and cardiac arrest (many a times during sleep). Routine cardiogram shows ST segment raising in leads V1 V3. Fever whitethorn precede syncope or tachycardia. There are 3 types of Brugada electrocardiogram PatternsType 1 coved type, where ST segment eyeshade 2 mm.Type 2 saddle gumption type, where ST segment elevation 2mm with positive T wave.Type 3 coved or saddle back type, where ST segment elevation Brugada Syndrome is inherited as an autosomal dominant trait. In 1998, the Syndrome was linked to mutations in SCN5A, the gene that encodes the alpha subunit for the sodium telephone line and since then over 300 mutations of SCN5A have been identified4. Mutations of gene SCN5A cause deviation of expression of sodium pass protein which decreases the sodium current resulting in indisposed conductivity in the heart. Bezzina et al presented evidence nominateing the theory that an SCN5A promoter polymorphism, common in Asian modulates, variability in cardiac conduction and may contribute to the high prevalence of Brugada Syndrome in Asian population5. theatrical role REPO RTA 27 year old male patient reported in the de vocalizationment of dentistry, with excruciating smart in lower right second molar and insisted on getting it extracted. Clinical enquiry and orthopantomogram revealed an extremely carious second molar. The patient was a recently diagnosed suit of Brugada Syndrome (Type 3). He gave a history of federal agency pain well-nigh 7 years ago, however, a couple of months ago he had persistent titty pain for which he was admitted in the intensive care unit for about 10 days, during which time he had 3 presyncopal attacks. On cardiac evaluation, electrocardiogram revealed an elevated ST segment in V1 V3 leads and partial RBBB pattern but structurally normal heart valves with normal pericardium and absence clots or vegetation. His left ventricular ejection fraction was 60%. CST (Cardiac Stress Test) was per organise by Bruce protocol where patient walked for 30 minutes with 10.1 METS which showed no angina/arrhythmia. Basal ECG showed RB BB persisted passim the test. However there were no significant ST segment changes during the test. Adequate chronotropic and ionotropic result was achieved. CST was negative for stress induced reversible ischaemia/and for arrhytmia. His family history revealed sudden death of his father at a younger age (42 years) with unbeknown(predicate) cause. However, there was no diagnosed case of Brugada Syndrome in the family. His past surgical history revealed an appendicectomy and septoplasty. He was a chronic smoker and occasionally consumed alcohol.Since it was a minor alveolar surgical procedure and given the patients history, the tooth extraction was planned chthonian local anaesthesia using lignocaine hydrochloride with adrenaline (12, 00,000) thereby avoiding general anaesthesia and the various drugs used with it that could trigger ventricular tachycardia in a BrS patient. Given the patients history of chest pain and diagnosis of Brugada Syndrome, patient was thoroughly evaluat ed by the anaesthetist and cardiologist prior to the dental treatment. High risk fitness was obtained.The patient was interpreted up in the intensive care unit. A ventilator and a defibrillator were kept standby. A 12 lead ECG was attached and was monitored unceasingly throughout the procedure. An I.V. line was secured. The anaesthetist and cardiologist along with the maxillofacial surgeons formed the surgical team. A right inferior alveolar nerve block was given using 3 ml lignocaine with adrenaline solution. Another 1 ml was used for intra-pulpal infiltration. After checking for subjective and objective signs the tooth was surgically extracted after sectioning the roots. The wound was closed using 3 0 vicryl. Patient tolerated the procedure well. Intra operatively patient was given 4 mg Dexamethasone along with injection Augmentin (Amoxicillin Clavulanate) 1.2 gm. Post operatively he was put on oral tablet Augmentin 625 mg and tablet Paracetamol twice a day. Patient was dischar ged the same day and was followed up in the dental department.DISCUSSIONBrugada Syndrome is a major cause of sudden unexplained death syndrome (SUDS) and death is caused by ventricular tachycardia and fibrillation (a lethal arrhythmia) in the heart which appears with no warning. The diagnosis in Brugada Syndrome is based on the characteristic patterns on an electrocardiogram, which may be routinely flowd by administration of certain drugs (ajmaline or flecainide). Brugada ECG pattern is very often hidden, but certain factors can debunk or trigger it same sodium channel blockers, febrile state, vagotonic agents, autonomic nervous system changes, excessive stress, tricyclic or tetracyclic antidepressants, first generation antihistamines (dimenhydrinate), a combination of glucose and insulin, hyperkalaemia, hypokalaemia, hypercalcaemia, alcohol toxicity, heavy meals at night unspoiled before sleeping, excessive vomiting, hot humid climatic conditions6.According to Nademanee and Vee rakul6, north-eastern part of Thailand where SUDS is prevalent and where temperatures can soar to 41oC a study is afoot(predicate) to gauge the climatic influences on occurrence of SUDS and they feel that physicians should factor in temperature as a cause of arrhythmogenesis in BrS. Several drugs could precipitate ventricular tachycardia and fibrillation which are listed in world Brugada registry in http//www.brugadadrugs.org (Accessibility confirm July 04, 2014). All Brugada patients and their treating physicians should be aware of these precipitating drugs at all times. numerous Brugada patients are asymptomatic and the classical pattern on ECG is picked up only by an experienced and trained physician. This pattern should be fit with age of patient, family history, chest pain, fever and presyncopal/syncopal attacks. Bupivacaine has been reported to unmask Brugada like ECG patterns when administered epidurally7. Hence we avoided bupivacaine and used lidocaine with adrenaline (12, 00,000 dilution) instead for our patient which was well tolerated by him. Lignocaine (class 1b antiarrythmic agent) displays rapid dissociation kinetics and produces little to no ST segment elevation in patients with congenital BrS8. The ventricular tachycardia in BrS can be prevented by avoiding certain aggravating factors like medication, drugs, fever and excessive stress. Brugada patients need to be regularly followed up over a long period of time. In severe cases the only line of treatment is placement of an implantable cardioverter defibrillator (ICD).Kloesel et al9 in 2011 did a literature search and compared results of previous reports with theirs regarding outcomes of patients with BrS who underwent surgeries and anaesthetic care and found 21 case reports and 4 case series. They collected data of 52 anaesthetics and 43 patients. In our literature search we found mention of only 2 patients of BrS who underwent surgeries in the maxillofacial region. 1) Plate fixation for mand ibular fracture in 56 year old male. 2) Tooth extraction, incision and drainage of odontogenic infection in 55 year old male10. However both these patients were treated under general anaesthesia. We decided to avoid general anaesthesia thereby retentivity the drugs to be used to the borderline.By thorough pre-anaesthetic evaluation, proper patient counselling, intra-operative pain control using optimum amount of lignocaine, 12 lead ECG continuously monitored at all times during procedure, constant blood insistence monitoring, avoiding use of certain drugs like bupivacaine, keeping a defibrillator standby and by having a cardiologist and anaesthetist in your surgical team these patients can be successfully managed. Post-operatively ICU monitoring is must for a minimum of 4 hours. There is a dearth of articles in the Maxillofacial and dental consonant literature regarding the management of these patients and we feel there is a need of more awareness of this not so rare cardiac cond ition among the dental and maxillofacial surgeons. With proper planning these patients with potentially life threatening and unequaled cardiac conditions can be safely and efficiently managed by maxillofacial surgeons for dental treatment.FUNDINGNone.COMPETING INTERESTSNone declared.ETHICAL APPROVALNot Required.ACKNOWLEDGEMENTSThe authors would like to thank Dr. Kuldeep and Dr. Arvind Singh, 1st year Residents, Department of Cardiology, KEMH Dr. Yogesh Naik, Assistant Professor, Department of anaesthesia, KEMH for their support throughout the treatment and co-operation.REFERENCESBrugada P, Brugada J. Right bundle branch block, persistent ST segment elevation and sudden cardiac death A distinct clinical and electrocardiographic syndrome. A multicenter report. J. Am Coll Cardiology 1992 20 1391-1396.Antzelvich C, Brugada P, Borggrefe M, Brugada J, Brugada R, Coraddo P, et al. Brugada Syndrome Report of the second consensus conference. Endorsed by the Heart Rhythm Society and the Eur opean Heart Rhythm friendship Circulation 2005 111 659-70.Diego J M, Condiero J M, Goodrow R J, Fish J M, Zygmunt A C, Perez G J, et al. Ionic and cellular basis for the predominance of the Brugada Syndrome phenotype in males. Circulation 2002 106 2004-11.Chen Q, Kirsch G E, Zhang D, et al. Genetic basis and molecular mechanism for idiopathic ventricular fibrillation nature. 199 392 293.C R Bezzina, W Simizu, P Yang, Tamara T, Koopmann M Tanck, et al. Common sodium channel promoter haplotype in Asian subjects underlies variability in cardiac conduction. Circulation 2006 113 338-344.Gumpanart Veerakul M D, Koonlawee Nademanee M D. Brugada Syndrome two decades of progress. Circ. Journal 2012 76 2713-2722.Phillips N, Priestly M, Denniss A R et al. Brugada type electrocardiographic pattern induced by epidural bupivacaine. Anaesthesia Analogue. 97 264 2003.8. Hideki Itoh, Keiko Tsuji,Tomoko Sakaguchi,Iori Nagaoka,Yuko Oka,et al . A paradoxical effect of lidocaine for the N406S mutation of SCN5Aassociated with Brugada syndrome. foreign Journal Of Cardiology. 2007 121 (3) 239-248.Benjamin Kloesel, Michael J Ackerman, Juraj Sprung, Bradly J. Narr, Toby N. Weingarter. Anaesthetic management of patients with Brugada Syndrome A case series and literature review. groundwork Journal Anaesthesia / Can Anaes 2011. 58 824-836.Nicholas Theododu, Joseph E. Cillo. Brugada Syndrome (Sudden Unexpected Death Syndrome) Perioperative and Anaesthetic Management in viva voce and Maxillofacial Surgery. J Oral Maxillofac Surg. 2009 67 (9) 20121-25.