Objectives: Inherited cardiac arrhythmias are life-threatening syndromes that often occur in young people and are characterized by palpitations, syncope, and an increased risk of sudden death. The etiology can be ion-channel dysfunctions as well as structural heart disease. Common features are single gene mutations that produce the dysfunction, an autosomal-dominant inheritance, incomplete penetrance and variable expressivity. Inherited arrhythmias show marked genetic heterogeneity having multiple types and sites of mutation. Genetic testing is particularly useful as support of the clinical data and in the identification of mutation-carriers that are asymptomatic but may be at risk. We have sought to identify the genetic mutations associated with inherited arrhythmia disorders in a Croatian group of patients and in their relatives in order to identify pathogenic mutations and possible asymptomatic carriers. Patients and Methods:We have analyzed the DNA from 36 unrelated Croatian patients (50% female, 41 ± 16 years) and 15 close relatives with arrhythmogenic right ventricular cardiomyopathy (ARVC), hypertrophic cardiomyopathy (HCM), long-QT-syndrome (LQTS) or Brugada syndrome (BrS). Only definite clinical diagnoses were accepted. Arrhythmia-related genes were analyzed by polymerase chain reaction (PCR) followed by direct sequencing in the exons and exon-intron boundaries. The control population included 200 healthy Croatian individuals. Results: In our study we found a high incidence of positive results (24 of 36 probands, 66%) with a great number of

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SAŽETAK: Posljednih godina dolazi do znatnog porasta broja implantiranih elektrostimulatora srca.Posljedično tomu raste i broj mogućih komplikacija te potreba za njihovom ekstrakcijom. Najčešća indikacija za ekstrakciju elektrostimulatora jest lokalizirana ili sustavna infekcija. S obzirom na to da je riječ o najkompleksnijim i najrizičnijim zahvatima iz područja kardiologije, iz godine u godinu razvijaju se nove tehnike i alati koji znatno olakšavaju ekstrakciju i smanjuju rizik od nastanka mogućih, pokatkad i vrlo teških komplikacija. S obzirom na navedeno, potrebno je organizirati dovoljan broj adekvatnih centara u kojima bi djelovao specijalizirani multidisciplinarni tim educiran za provođenje navedenih zahvata.Od početka 2013. godine na Odjelu za aritmije i elektrostimulaciju Zavoda za kardiovaskularne bolesti Kliničkog bolničkog centra Rijeka započeo je program ekstrakcija elektroda. U razdoblju od dvije i pol godine učinjeno je ukupno 27 zahvata te je uklonjena ukupno 51 elektroda, od čega su dvije bile defibrilatorske. Glavni uzrok ekstrakcije elektroda bila je lokalizirana infekcija / dekubitus lože, dok je sustavna infekcija bila mnogo rjeđa. U postupku ekstrakcije prevladava tehnika trakcije i "locking" stileta. Najznačajnija je komplikacija razvoj simptomatskoga perikardijalnog izljeva. Smrtnih ishoda nije bilo.SUMMARY: During recent years there has been a significant increase in pacemaker implantation. Consequently, the number of possible complications and the need for pacemaker lead extraction has grown as well. The most common indication for pacemaker lead extraction is localized or systemic infection. Since lead extraction is among the most complex and dangerous cardiologic procedures, new techniques and tools are being developed on a yearly basis that significantly facilitate extraction and reduce the risk of possible, often very severe, complications. Considering the above, it is necessary to organize enough appropriate centers with specialized multidisciplinary teams trained for the performance of these procedures.Since early 2013, a pacemaker lead extraction program was started at the Department for Arrhythmia and Electrical Stimulation at the University Hospital Centre Rijeka. Over a period of two and a half years, a total of 27 procedures have been performed and 51 pacemaker leads were extracted, of which two were defibrillator leads. The main cause of lead extraction was localized infection/pocket decubitus, while the incidence of systemic infection was much lower. Extraction techniques used were predominantly traction and locking stylet extractions. The most significant complication was the development of symptomatic pericardial effusion. There were no fatal outcomes.KljUčnE RijEči: elektrostimulator, elektroda, infekcija, ekstrakcija, komplikacije.

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