Brugada syndrome: A brand new case

Abstract

Background: Brugada syndrome (BS) is a disorder characterized by syncope or sudden death associated with one of several electrocardiographic (ECG) patterns characterized by incomplete right bundle branch block and ST elevation in the anterior precordial leads. Patients with BS are prone to develop ventricular tachyarrhythmias that may lead to syncope, cardiac arrest, or sudden cardiac death. Case report. A 58-year-old woman is the first described case of Brugada syndrome in Serbia with intermittent typical changes in basic electrocardiography (ECG): ST segment elevation in the precordial chest leads like dome or coved - major form or type I. For the last 27 years the patient had suffered of palpitations and dizziness, without syncopal events. Her sister had died suddenly during the night in sleep. During 24-hour Holter monitoring the patient had ventricular premature beats during the night with R/T phenomenon and during the recovery phase of exercise testing had rare premature ventricular beats as the consequence of parasympatethic stimulation. Late potentials were positive. Echocardiography revealed left ventricular ejection fraction of 60%. We performed coronary angiography and epicardial coronary arteries were without significant stenosis and structural heart disease was excluded. In the bigining of the electrophysiological study ECG was normal, and after administration of Propaphenon i.v. Brugada syndrome unmasked with appearance of type I ECG pattern. A programed ventricular stimulation induced non sustained ventricular tachycardia. One-chamber implantable cardioverter defibrillator was implanted and the patient was treated with a combination od amiodarone and metoprolol per os. After one-year follow-up, there were no episodes of ventricular tachycardia and ventricular fibrillation. Conclusion. Brugada syndrome is a myocardial disorder which prognosis and therapy are related to presence of ventricular fibrillation or ventricular tachycardia. Electrophysiologicaly induced malignant ventricular disorders class I are indication for implantation of cardioverter defibrilator, as also occurred in presented patient.Uvod. Sindrom Brugada (SB) je poremecaj koji moze dovesti do sinkope i iznenadne smrti, a udruzen je sa jednom od nekoliko elektrokardiografskih (EKG) manifestacija: nekompletni blok desne grane i elevacija ST segmenta u prednjim prekordijalnim odvodima. Kod bolesnika sa SB cesto se razvija ventrikularna tahiaritmija koja moze dovesti do sinkope, srcanog aresta ili iznenadne srcane smrti. Prikaz bolesnika. Bolesnica stara 58 godina prvi je opisan slucaj sindroma Brugada u Srbiji, sa intermitentnim tipicnim promenama za ovo oboljenje: u bazicnom elektrokardiogramu (EKG)-u: elevacija ST segmenta u prekordijalnim odvodima V1-V3 oblika platoa ili svoda - oblik major ili tip I. Poslednjih 27 godina imala je palpitacije u vidu preskoka i ubrzanog rada srca pracenim nesvesticom, bez gubitka svesti. U porodicnoj anamnezi, rodjena sestra umrla je naprasno u toku sna. Na 24- casovnom Holter monitoringu zabelezene su pojedinacne komorske ekstrasistole u toku noci sa fenomenom R/T, a na testu opterecenja retke pojedinacne komorske ekstrasistole u fazi oporavka, sto je posledica predominacije tonusa parasimpatikusa. Kasni potencijali bili su pozitivni. Ultrazvuk srca pokazao je ocuvanu globalnu sistolnu funkciju leve komore, ejekciona frakcija leve komore procenjena je na 60%, ehokardiografski nalazi srca bili su, takodje, normalni. Uradjena je koronarografija koja nije detektovala znacajne promene na epikardnim koronarnim arterijama, cime je iskljuceno strukturno oboljenje miokarda. U nastavku procedure sprovedeno je elektrofiziolosko ispitivanje: na pocetku ispitivanja EKG je bio normalan, a zatim je uradjen propafenski test koji je demaskirao sindrom Brugada (doslo je do pojave elevacije ST segmenta tipa I). Programiranom ventrikularnom stimulacijom izazvana je kratkotrajna ventrikularna tahikardija koja je spontano prosla. Bolesnici je indikovana ugradnja jednokomorskog implantabilnog kardioverter-defibrilatora, koji je implantiran, a u terapiju su uvedni amiodaron i metoprolol. Nakon jednogodisnjeg pracenja nije bilo epizoda ventrikularne tahikardije i ventrikularne fibrilacije. Zakljucak. Sindrom Brugada je bolest miokarda cija su prognoza i terapija odredjeni prisustvom ventrikularne fibrilacije ili ventrikularne tahikardije. Elektrofizioloskim ispitivanjem indukovani maligni ventrikularni poremecaji klase I srcanog ritma indikacija su za ugradnju kardioverterdefibrilatora, sto je bio slucaj i kod nase bolesnice.