PERKUTANA AORTNA VALVULOPLASTIKA BALONOM U DJECE

We assessed the effectiveness of aortic balloon valvuloplasty (AoVP) in 34 children who were admitted for aortic valve balloon dilatation over 7 years (Feb 1997-Feb 2004) in two institutions (Stuttgart and Zagreb). There was a prevalence of male children (28/6; p<0.01), mean age at dilatation 35.55±55.59 months (mean ± SD, min 1 day, max 14.2 years) and mean body weight 13.1±15.9 kg (min 2640 g, max 57 kg). Patients were divided into two groups as follows: group 1 including neonates and small infants younger than 2 months at dilatation with criteria for critical aortic stenosis (n=18); and group 2 including infants older than 2 months at dilatation (n=16). Sixteen (47%) of all patients had no clinical symptoms, 12 (35.3%) were dyspneic at rest and sweating at feeding (NYHA III), and 6 (17.6%) had severe heart failure (NYHA IV). All NYHA IV patients were in group 1 (n=6) versus 0 in group 2 (p<0.05). According to ECHO estimation, left ventricular (LV) function was normal in 16 (47%), moderately limited in 12 (35.3%) and severely impaired in 6 (17.6%) patients. All patients with severely impaired LV function belonged to group 1 (n=6) versus 0 in group 2 (p<0.05). Balloon dilatation was performed retrogradely via the percutaneous femoral artery approach in all except one patient in which the balloon catheter was introduced anterogradely via the mitral valve (MV). Indexed aortic valve-annulus/body surface area (BSA) (mm/m2) was 30.97±10.02) (max 47.5, min 12.02) for overall study sample, 37.60±5.99 in group 1 and 23.03±7.86 in group 2 (group 1 vs. group 2, p<0.05). Ao/Ba ratio (mm) was 0.85±0.09 for overall study sample, 0.81±0.11 for group 1 and 0.89±0.05 for group (group 2 vs. group 1, p<0.01). Immediately after dilatation, the mean systolic pressure gradient across the aortic valve decreased from 70.62±20.78 (max 120, min 45 mm Hg) to 20.03±13.7 (max 65, min 0 mm Hg) in the whole study group (p<0.05), from 73.23±21.57 (max 120, min. 50 mm Hg) to 15.25±11.09 (max 40, min 0 mm Hg) in group 1 (p<0.05), and from 67.78±20.21 (max 111, min 45 mm Hg) to 24.81±14.71 (max 65, min 10 mm Hg) in group 2 (p<0.05) (catheter measurement). Follow-up results were studied in 31 (91%) patients at 3.5-84 months (20.91±22.19) after AoVP and revealed continuously increasing residual aortic valve gradient (31.35±12.01, max 50, min 15 mm Hg), still being significantly lower (p<0.001) than before valvuloplasty. The overall actuarial survival rate after 7 years was 91%. Freedom of three categories (any reintervention, surgical reintervention, and re-dilatation) was 77, 74, 61; 87, 84, 77; and 90, 90, 83 at 2, 4 and 7 years for the total number of patients, respectively. The actuarial freedom for the same categories in group 1 vs. group 2 was 72, 67, 56 vs. 87, 87, 75 (p<0.05); 89, 83, 78 vs. 87, 87, 75 (NS); 83, 83, 78 vs. 100, 100, 94 (p<0.05) at 2, 4 and 7 years. The degree of aortic regurgitation immediately after catheterization did not significantly increase; only 1 patient developed moderate aortic regurgitation, which was treated with surgical valve reconstruction on day 1 after intervention. At follow-up, aortic regurgitation increased to grade 3 in 3 (10%) and to grade 2 in 7 (23.3%) patients. All three patients with high grade of aortic insufficiency were from group 1 vs. 0 in group 2 (p<0.05). Eight (26%) patients required reintervention, 4 (13%) balloon valvuloplasty plus surgery, and 4 surgery only. Of 8 patients requiring surgery, 4 (13%) were operated on during a period of 27-78 months and 4 within one month after dilatation. One patient died one week after dilatation, re-dilatation and surgery due to fibroelastosis (confirmation by histology). Conclusion: Percutaneous balloon valvotomy provides an effective palliative interventional method in the treatment of infants and children with aortic valve stenosis. The majority of problems in the early and late period after dilatation appear in the group of patients with critical aortic stenosis.Procijenili smo učinkovitost aortne valvuloplastike (AoVP) kod 34 djece koja su podvrgnuta dilataciji aortne valvule balonom tijekom 7 godina (od veljače 2097. do veljače 2014.) u dvije ustanove (Stuttgart i Zagreb). Prevladavala su muška djeca (28/6; p<0,01), srednja dob (±SD) kod dilatacije bila je 35,55±55,59 mjeseci (minimum 1 dan, maksimum 14,2 godine), a tjelesna težina 13,1±15,9 kg (minimum 2640 g, maksimum 57 kg). Pacijenti su podijeljeni u dvije skupine. Skupinu 1 činila su novorođenčad i dojenčad mlađa od 2 mjeseca kod prve dilatacije s kriterijima za kritičnu aortnu stenozu (N=18), a skupinu 2 djeca starija od 2 mjeseca kod dilatacije (N=16). Šesnaest od 34 (47 %) pacijenata nije imalo kliničkih simptoma, 12 (35,3 %) je imalo dispneju pri mirovanju i pojačano se znojilo tijekom hranjenja (NYHA III), a 6 (17,6 %) pacijenata je imalo simptome teškog srčanog zatajenja (NYHA IV). Svi pacijenti s NYHA IV bili u skupini 1 (n=6) prema 0 u skupini 2 (p<0,05). Prema ehokardiografskom nalazu funkcija lijevog ventrikla (LV) bila je normalna u 16 (47 %), umjereno oslabljena u 12 (35,3 %) i teško oslabljena u 6 (17,6 %) pacijenata. Svi pacijenti s teško oštećenom funkcijom LV pripadali su skupini 1. (n=6) prema 0 u skupini 2. (p<0,05]. AoVP balonom izvedena je retrogradno nakon perkutane punkcije femoralne arterije kod svih osim jednog pacijenta kod kojega je balonski kateter uveden anterogradno putem mitralne valvule (MV). Kvocijent anulusa aortnog zalistka/u odnosu na površinu tijela (BSA) (mm/m2) bio je 30,97±10,02 (maksimum 47,5, minimum 12,02) i 37,60±5,99 u odnosu na 23,03±7,86 u skupini 1 prema skupini 2 (p<0,05). Omjer Ao/Ba (mm/mm) za cijelu skupinu bio je 0,85±0,09 te 0,81±0,11) u skupini 1 i 0,89±0,05) u skupini 2 (skupina 1 prema skupini 2, p<0,01). Odmah nakon dilatacije srednja sistolička vrijednost gradijenta tlaka na aortnoj valvuli smanjuje se sa 70,62±20,78 (maksimum 120, minimum 45 mm Hg) na 20,03±13,7 (maksimum 65, minimum 0 mm Hg.) u cijeloj skupini (p<0,05) te sa 73,23±21,57 (maksimum 120, minimum 50 mm Hg) na 15,25±11,09 (maksimum 40, minimum 0 mm Hg) u skupini 1 (p<0,05) i sa 67,78±20,21 (maksimum 111, minimum 45 mm Hg) na 24,81±14,71 (maksimum 65, minimum 10 mm Hg) u skupini 2 (skupina 1 prema skupini 2, p<0,05) (mjerenje kateterom). Rezultati termina praćenja proučavani su kod 31 (91 %) pacijenta 3,5-84 (20,91±22,19) mjeseca. Nakon AoVP nađen je kontinuirano rastući rezidualni gradijent aortne valvule (31,35±12,01; maksimum 50, minimum 15 mm Hg), ali je i dalje bio značajno niži (p<0,001) nego prije valvuloplastike. Ukupna stopa preživljavanja nakon 7 godina iznosila je 91 %. Oslobađanje od tri moguće skupine re-intervencija (bilo koja re-intervencija, kirurška re-intervencija; ponovna dilatacija) bila je 77, 74, 61; 87, 84, 77; 90, 90, 83 u 2, 4 i 7 godina za cijelu skupinu te u skupini 1 prema skupini 72, 67, 56 prema 87, 87, 75 (p<0,05); 89, 83, 78 prema 87, 87, 75 (NS); 83, 83, 78 prema 100, 100, 94 (p<0,05) u 2, 4 i 7 godina. Stupanj aortne insuficijencije neposredno nakon kateterizacije nije se značajno povećao; samo je 1 pacijent razvio aortnu insuficijenciju koja je liječena kirurškom rekonstrukcijom zalistaka na dan intervencije. Nakon praćenja aortna insuficijencija povećala se na stupanj III. u 3 (10 %) i na stupanj II. u 7 (23,3 %) pacijenata. Sve troje bolesnika s visokim stupnjem aortne insuficijencije pripadali su skupini 1 (prema 0 u skupini 2; p<0,05). Osam (26 %) pacijenata zahtijevalo je ponovnu intervenciju, a samo 4 (13 %) ponovnu AoVP plus operaciju. Od 8 pacijenata kojima je bila potrebna operacija 4 (13 %) su operirana tijekom razdoblja od 27 do 78 mjeseci, a 4 u roku od mjesec dana nakon dilatacije. Jedan pacijent umro je tjedan dana nakon dilatacije, ponovne dilatacije i kirurškog zahvata zbog fibroelastoze (potvrda histološka). Zaključak: Perkutana balonska valvuloplastika je učinkovita palijativna intervencijska metoda u liječenju novorođenčadi i djece sa stenozom aortne valvule. Većina problema u ranom i kasnom razdoblju nakon dilatacije pojavljuje se u skupini bolesnika s kritičnom aortnom stenozom u novorođenačkoj i ranoj dojenačkoj dobi

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

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