RHEUMATOLOGY IN PEDIATRIC CARDIOLOGY FROM FETAL STAGE TO ADULTHOOD. THE ROLE OF FETAL ECHOCARDIOGRAPHY

Iako su se pedijatrijska kardiologija i reumatologija unatrag deset godina i u našim uvjetima formalno razdvojile, među njima su ostale snažne poveznice. One se više ne očituju samo u reumatskoj vrućici već i u drugim reumatološkim entitetima: sistemskom eritemskom lupusu, antifosfolipidnom sindromu, sistemskoj sklerodermiji, Kawasakijevoj bolesti, nodoznom poliarteritisu, nekim oblicima granulomatoznog vaskulitisa. Posebno se osvrćemo na pojavu kompletnoga kongenitalnog atrioventrikularnog bloka (CCAVB) u trudnica koje zbog sistemskih bolesti vezivnog tkiva imaju osobita ANA-protutijela (anti-SSA/Ro i/ili anti-SSB/La), ali i vjerojatnost da istovjetno upalno zbivanje u srcu bude razlogom razvoja restrikcijske kardiomiopatije pridružene CCAVC-u. Iako je time reumatologija već odavno ušla u ranu fetalnu dob, postoje i neki problemi koji se odnose na embrionalno razdoblje (utjecaj odnosa metotreksata i folne kiseline na embrionalni razvoj srca) i na kasno fetalno razdoblje (zbog odnosa antiprostaglandinskih antifl ogistika na prematurno zatvaranje Botallova duktusa). U radu se posebno osvrćemo na najtežu komplikaciju u fetalnom razdoblju koja se zove neonatalni lupusni sindrom. Time se ostvaruje višestruka veza između kardiologije i reumatologije svih dobnih skupina, od embrionalne i fetalne do odrasle dobi. Upravo i jest cilj ovoga rada prikazati neke važne poveznice i na taj način aktualizirati novu suradnju pedijatrijske kardiologije s reumatologijom od fetalne do odrasle dobi.Even though in the last ten years pediatric cardiology and rheumatology have formally separated in Croatia as well, strong links still remain between them. Th ey are no longer manifested through rheumatic fever, but through other rheumatic entities: systemic lupus erythematosus, antiphospholipid syndrome, systemic scleroderma, Kawasaki disease, polyarteritis nodosa, and some forms of granulomatous vasculitis. We take special note of the occurrence of complete congenital atrioventricular block (CCAVB) in pregnant women who, due to systemic connective tissue diseases, develop distinctive ANA antibodies (anti-SSA/Ro and/or anti SSB/La), but who are also likely to have the same infl ammation within the heart, leading to the development of restricted cardiomyopathy associated with CCAVC. Although rheumatology has thus been involved with early fetal age for some time now, there are also some issues relating to the embryonal stage (the association between methotrexate/folic acid and heart development in embryos), as well as to the late fetal stage (antiprostaglandin anti-infl ammatory agent impact on premature ductus arteriosus closure). We gave special attention to the neonatal lupus syndrome, the most serious complication in the fetal period. Th us the multiple association between cardiology and rheumatology in all age groups, from the embryonal and fetal stage to adulthood, is being realized. Th e aim of this paper is to present some important links and thus further emphasize the new cooperation between pediatric cardiology and rheumatology from fetal stage to adulthood

FUEL INJECTION SYSTEMS OF THE DIESEL ENGINES

U ovom završnom radu opisani su i analizirani sustavi za ubrizgavanje goriva Dieselovih motora. Ponajprije je prikazan sam princip rada Diesel motora kroz četiri takta. Ujedno je i navedena glavna razlika u odnosu na Otto motor, a to je da se kod Diesel motora ubrizgava gorivo u već komprimirani zrak u cilindru, dok se kod Otto motora u cilindar ubrizgava prethodno stvorena smjesa goriva i zraka. S obzirom na to da je sustav ubrizgavanja goriva jedna od najbitnijih stavki što se tiče razvoja Diesel motora, u sljedećem poglavlju je detaljno opisan proces ubrizgavanja i izgaranja goriva u cilindru ovog tipa motora. Osnovna zadaća svakog sustava ubrizgavanja je dobaviti gorivo iz spremnika, potom ga stlačiti na određeni tlak i fino raspršiti u prostor izgaranja u cilju postizanja potpunog izgaranja smjese. Da bi se ostvarilo potpuno izgaranje, mekan i pravilan rad motora, potrebno je međusobno uskladiti rad svih komponenti sustava ubrizgavanja kako ne bi došlo do kvara samog motora. Nadalje, u radu su prikazane dvije izvedbe Diesel motora, odnosno dva tipa ubrizgavanja goriva. Razvojem visokotlačnih pumpi i brizgaljki kroz povijest ostvaruju se visoki tlakovi ubrizgavanja koji rezultiraju velikim opterećenjima pogonskog mehanizma. Zbog tog nedostatka počinju se primjenjivati motori s indirektnim ubrizgavanjem, to jest različitim izvedbama pretkomora. Motori s indirektnim ubrizgavanjem zahtijevali su ugradnju uređaja za hladan star koji je stvarao dodatne troškove i probleme. Uvođenjem predubrizgavanja goriva u sam proces ubrizgavanja, rješava se problem visokih tlakova izgaranja te se ponovno počinju primjenjivati motori s direktnim ubrizgavanjem, koji su zahvaljujući svojim karakteristikama danas postali nezamjenjivi. U zadnjem poglavlju analizirani su različiti sustavi ubrizgavanja goriva, od starih mehanički reguliranih visokotlačnih pumpi pa sve do suvremenog elektronski reguliranog Common rail sustava te rad njihovih glavnih komponenti.In this final paper, fuel injection systems of the Diesel engines are described and analysed. First and foremost, the principle of operation of Diesel engines through four strokes is displayed. It also contains the main difference compared to the Otto-engine, which is that with Diesel engines the fuel is being injected into the already compressed air in the cylinder, while discussing the Otto-engine, a previously created mixture of fuel and air is injected into the cylinder. Given that the fuel injection system is one of the most important steps regarding the development of Diesel engines, the next chapter contains a detailed process of the injection and combustion gas in the cylinder of this type of engine. The main task of each injection system is to obtain fuel from the tank, then compact it on a certain pressure and finely disperse in the combustion space in order to achieve complete combustion of the mixture. To achieve complete combustion, i. e. soft and proper operation of engine, it is necessary to synchronize the work of all components of the injection system to avoid malfunction of the motor itself. In addition, the paper presents two performances of Diesel engines, that is, two types of fuel injection. The development of high-pressure pumps and injectors throughout history helps in achieving a high injection pressures that result in large loads of the drive mechanism. Because of this disadvantage, the engines with indirect injection, that is, with different performances of the antechamber are applied. Engines with indirect injection had called for the installation of a cool start device which created additional costs and problems. By introducing the fuel pre-injection into the fuel injection process itself, a problem of high combustion pressures is fixed and the engines with direct injection are back in use, which due to its characteristics, have become irreplaceable. The last chapter analyses the different fuel injection systems, from old mechanically controlled high-pressure pumps all the way up to modern electronic regulated Common rail system, and the work of their main components

Rituximab in Treatment of Children with Refractory Vasculitis and Systemic Lupus Erythematosus – Single Center Experience in Croatia

The aim of this study was to present our experience in rituximab therapy in patients with childhood-onset systemic lupus erythematosus, lupus nephritis, and ANCA-associated vasculitis. We conducted a retrospective clinical chart review of all patients treated with rituximab in the time period from January 2009 to December 2015. Eight patients (3 boys and 5 girls) aged 8 to 15 at the onset of disease were treated with rituximab. Remission of disease was accomplished in 4 patients with childhood- onset systemic lupus erythematosus and lupus nephritis, a partial improvement was achieved in 1 patient with childhoodonset systemic lupus erythematosus and lupus nephritis as well as in 2 patients with vasculitis, while in one patient with vasculitis treatment with rituximab showed no effect and the patient died due to Candida sepsis. Reduction of corticosteroid doses was enabled by rituximab treatment. Rituximab appeared to be a safe and efficient therapeutic option in severe cases of childhood- onset systemic lupus erythematosus or ANCA-associated vasculitis that failed to respond to conventional therapy or as a rescue therapy in life-threatening conditions

DISTRIBUTION OF CONGENITAL HEART DISEASE IN CROATIA AND OUTCOME ANALYSIS A Croatian epidemiological study (2002–2007)

prikazati populacijski i hospitalni registar prirođenih srčanih grješaka za Hrvatsku. Metode: Tijekom 5 godina ispunjavali smo upitnik s pitanjima o populacijskom kretanju prirođenih srčanih grješaka (prema preporukama BWIS i EUROCAT study) i hospitalni registar kojim smo pratili liječenje i ishod liječenja u djece koja su u spomenutom razdoblju rođena u Hrvatskoj. Rezultati: Od 1. 10. 2002 do 1. 10. 2007. u Hrvatskoj je rođeno 205917-ero djece, od čega je 1480-ero djece imalo prirođenu srčanu grješku (7,2 promila). Najčešće postavljena dijagnoza je VSD (34,6%). Na kardiokirurško liječenje upućeno je 430-ero djece. Na njima je učinjeno 556 operacija, od čega 202 u Hrvatskoj, ostatak u inozemstvu. Ukupni mortalitet nakon kardiokirurškog liječenja iznosi 5%. Razlike u mortalitetu i morbiditetu nakon kardiokirurških operacija izvedenih u Hrvatskoj i onih u inozemstvu uočavaju se nakon analize ishoda liječenja prema dogovorenim metodologijama. Zaključak: Registar prirođenih srčanih grješaka čini osnovu za epidemiološka istraživanja, no i za razvoj smjernica u struci.Objective: The aim of our paper is to show results of population and hospital registry of congenital heart disease in Croatia. Methods: Information on patients born during the five-year study and with diagnosis of congenital heart defects, obtained all across the country, were collected in the population and hospital registry set up according to the EUROCAT and BWIS registries principles. Results: Between October 1, 2002, and October 1 2007, there were 205 051 live births in Croatia, of which 1480 patients were diagnosed with congenital heart defects, accounting for 0.72 % of the live-born children. The most common diagnosis was ventricular septal defect with percentage of 34.6%. Among 1480 children, 430 needed an operation. Among 553 cardiac surgeries performed, 202 were done in Croatia and others were done in institutions abroad. Mortality rate after surgery was 5%. Only after adjustment for complexity there are marked differences in mortality and occurrence of postoperative complications between Croatia and centers abroad. Conclusion: The importance of the registry of congenital heart disease relies in the field of epidemiological research but can be used as a tool for future planning of health services

DISTRIBUTION OF CONGENITAL HEART DISEASE IN CROATIA AND OUTCOME ANALYSIS A Croatian epidemiological study (2002–2007)

prikazati populacijski i hospitalni registar prirođenih srčanih grješaka za Hrvatsku. Metode: Tijekom 5 godina ispunjavali smo upitnik s pitanjima o populacijskom kretanju prirođenih srčanih grješaka (prema preporukama BWIS i EUROCAT study) i hospitalni registar kojim smo pratili liječenje i ishod liječenja u djece koja su u spomenutom razdoblju rođena u Hrvatskoj. Rezultati: Od 1. 10. 2002 do 1. 10. 2007. u Hrvatskoj je rođeno 205917-ero djece, od čega je 1480-ero djece imalo prirođenu srčanu grješku (7,2 promila). Najčešće postavljena dijagnoza je VSD (34,6%). Na kardiokirurško liječenje upućeno je 430-ero djece. Na njima je učinjeno 556 operacija, od čega 202 u Hrvatskoj, ostatak u inozemstvu. Ukupni mortalitet nakon kardiokirurškog liječenja iznosi 5%. Razlike u mortalitetu i morbiditetu nakon kardiokirurških operacija izvedenih u Hrvatskoj i onih u inozemstvu uočavaju se nakon analize ishoda liječenja prema dogovorenim metodologijama. Zaključak: Registar prirođenih srčanih grješaka čini osnovu za epidemiološka istraživanja, no i za razvoj smjernica u struci.Objective: The aim of our paper is to show results of population and hospital registry of congenital heart disease in Croatia. Methods: Information on patients born during the five-year study and with diagnosis of congenital heart defects, obtained all across the country, were collected in the population and hospital registry set up according to the EUROCAT and BWIS registries principles. Results: Between October 1, 2002, and October 1 2007, there were 205 051 live births in Croatia, of which 1480 patients were diagnosed with congenital heart defects, accounting for 0.72 % of the live-born children. The most common diagnosis was ventricular septal defect with percentage of 34.6%. Among 1480 children, 430 needed an operation. Among 553 cardiac surgeries performed, 202 were done in Croatia and others were done in institutions abroad. Mortality rate after surgery was 5%. Only after adjustment for complexity there are marked differences in mortality and occurrence of postoperative complications between Croatia and centers abroad. Conclusion: The importance of the registry of congenital heart disease relies in the field of epidemiological research but can be used as a tool for future planning of health services

DIGOXIN I AMIODARON KOD TRAJNE FETALNE SUPRAVENTRIKULARNE TAHIKARDIJE I NEIMUNOLOŠKOG FETALNOG HIDROPSA

Supraventricular tachycardia is the most common and clinically significant form of sustained fetal tachyarrhythmia in pregnancy; depending on duration and high rate variability heart failure and nonimmune hydrops may develop which are associated with a high incidence of perinatal mortality. Doppler/echo diagnosis is usually accidental during second and third trimester of pregnancy. Therapeutic goals are cardioconversion to sinus rhythm and recovery of heart failure. We present a case of fetal supraventricualr tachycardia diagnosed at 29 weeks of gestation with nonimmune hydrops. Treatment with digoxin and amiodarone was successful. The heart rate restored to sinus rhythm and nonimmune hydrops resolved within three weeks of treatment. Therapy with two drugs that act synergistically may be more efficient than monotherapy in blocking likely atrio-ventricular reentry mechanism by accessory pathway in sustained supraventricular tachycardia, thus allowing resolution of hydrops with favorable management outcome.Supraventrikularna tahikardija je najčešći i klinički najznačajniji oblik fetalne tahiaritmije u trudnoći, a ovisno o trajanju i visini srčane aktivnosti mogu se razviti zatajenje srca i neimuni fetalni hidrops, oboje povezani s lošim perinatalnim ishodom. Tijekom drugog i trećeg tromjesečja dijagnoza se često slučajno postavlja ultrazvučnim doplerskim nalazom. Terapijski cilj je konverzija u sinus ritam i oporavak srčane funkcije. Donosimo prikaz slučaja fetalne supraventrikularne tahikardije s neimunim hidropsom, dijagnosticirane u 29. tjednu trudnoće. Terapija digoksinom i amiodaronom bila je uspješna. Ponovo je uspostavljen sinusni ritam srčane frekvencije, a neimuni hidrops se povukao unutar tri tjedna liječenja. Terapija dvama lijekovima sa sinergističkim djelovanjem može biti učinkovitija od monoterapije u blokiranju vjerojatno ponovnog ulaska atrio-ventrikularnog impulsa pomoću sporednog puta u supraventrikularnoj tahikardiji, time dopuštajući povlačenje hidropsa te bolji ishod

POSTERIOR IDIOPATHIC FASCICULAR LEFT VENTRICULAR TACHYCARDIA, VERAPAMIL SENSITIVE – BELHASSEN’S TACHYCARDIA – A CASE REPORT

Uvod: Prikazujemo rijetku ventrikularnu tahikardiju nepoznata uzroka (idiopatsku) s ishodištem u blizini stražnjeg snopića (fascikula) lijeve grane Hisova snopa, koja se očituje četirima osnovnim obilježjima: široki QRS-kompleksi, blok desne grane, lijeva (superiorna) električna os i osjetljivost na verapamil. Od 1981. godine, kada je Belhassen sa suradnicima otkrio osjetljivost na verapamil, često se naziva Belhassenova tahikardija. Cilj: Osnovna svrha ovog prikaza jest podsjetiti da se već ispravnom interpretacijom EKG-a može postaviti točna dijagnoza ove potencijalno opasne aritmije. Prikaz bolesnika: Do sada zdrava djevojčica adolescentne dobi došla je u hitnu službu zbog palpitacija, dispneje i presinkopalne krize. Na 12-kanalnom EKG zapisu našli smo čak tri od četiri obilježja stražnje idiopatske fascikularne ljevoventrikularne tahikardije, a konverzijom u sinusni ritam nakon intravenske primjene verapamila zadovoljeno je četvrto obilježje i time je potvrđena elektrokardiografska sumnja. Zbog učestalih recidiva ventrikularne tahikardije uza sve češće subjektivne tegobe srčane naravi (palpitacije, prekordijalne opresije, vrtoglavice, presinkopalne krize), a usprkos sve višoj peroralnoj dozi verapamila te uzevši u obzir pozitivnu obiteljsku anamnezu na iznenadnu smrt, upućena je na elektrofiziološko ispitivanje i liječenje radiofrekventnom ablacijom. Zaključak: Temeljitom i dobrom analizom 12-kanalnog EKG-a može se postaviti točna dijagnoza vrste tahikardije, što je uvjet daljnjeg ispravnog postupanja i liječenja.Introduction: We present a rare ventricular tachycardia of unknown origin (idiopathic) which originates near the posterior fascicle of the left bundle branch of His. The tachycardia has four basic features: wide QRS complex, RBBB morphology, left (superior) axis deviation and sensitivity to verapamil. Since 1981 (when sensitivity to verapamil was discovered by Belhassen et al.) it has frequently been called Belhassen’s tachycardia. Aim: The purpose of this case report was to remind that correct diagnosis of this potentially dangerous arrhythmia can be made with detailed and correct interpretation of 12-lead ECG. Case report: Until now healthy adolescent girl came to the ER because of palpitations, dyspnoea and presyncopal attack. The 12-lead ECG was done. It showed three of four basic features of posterior idiopathic fascicular left ventricular tachycardia. After the conversion of tachycardia to sinus rhythm by intravenous application of verapamil, the fourth feature was also shown and by that the diagnosis was confirmed. Because of the frequent recurrence of ventricular tachycardia with clinical symptoms associated with cardiovascular system (palpitations, precordial oppressions, dizziness, presyncopal crisis) despite ever increasing dosage of oral verapamil and with taking into consideration a positive family history of sudden death syndrome, the adolescent girl was referred to the electrophysiology study and treatment with radiofrequency ablation. Conclusion:Detailed and correct interpretation of 12-lead ECG can correctly diagnose the type of tachycardia, which is fundamental for efficient managing and treatment