Cilj rada: prikazati nove metode intervencijskog liječenja u Referentnom centru. Bolesnici i metode: Prikazani su slučajevi od 1. srpnja 2018. do 1. srpnja 2022. u kojih je učinjena nova procedura, ...neovisno o dobi i spolu. U analizu su uključeni svi pacijenti kojima je učinjena nova metoda intervencijskog liječenja s prikazom podležeće anatomske/hemodinamske patologije te prikazom nove metode. Rezultati: U navedenom periodu uvedeno je pet novih perkutanih metoda, učinjenih u ukupno 34 pacijenta. Učinjeno je trinaest intervencija umetanja stenta u trinaest pacijenata s koarktacijom aorte. Srednja dob pacijenata kojima je učinjen perkutani zahvat umetanja stenta u koarktaciju aorte jest 13,4 godine. U navedenom periodu u šestero pacijenata stenoza pulmonalne grane liječena je umetanjem stenta. Od toga je troje pacijenata bilo dojenačke dobi, jedno u dobi od šesnaest mjeseci, jedno u dobi od četiri godine te jedna pacijentica u dobi od petnaest godina. U tri bolesnika učinjeno je perkutano umetanje zalistka Melody (kravlja jugularna vena integrirana u prekriveni stent), srednje dobi kod zahvata 17,3 godine. Metodom dilatacije stenta, zbog razvoja restenoze, liječili smo deset pacijenata, kod čega sedam dilatacijom prethodno umetnutog stenta u rekoarktaciju aorte, dva pacijenta dilatacijom stenta umetnutog na poziciji restenoze pulmonalnih grana te jednog dilatacijom stenta u stenotičnoj gornjoj šupljoj veni. Peta metoda koje je uvedena jest zatvaranje aortopulmonalnih kolaterala u djece s univentrikulskom cirkulacijom. Učinjena je u dvoje djece, dojenčeta u dobi od četiri mjeseca i pacijentice u dobi od tri i pol godine, a koji boluju od sindroma hipoplastičnoga lijevog srca. Svi su zahvati učinjeni uz povoljan hemodinamski rezultat, bez akutnih uz zahvat vezanih komplikacija. Zaključak: Uvođenjem novih metoda stekli smo nova znanja, poboljšali razinu dostupne skrbi u Republici Hrvatskoj (RH) te u konačnici i utjecali na smanjenje slanja složenih bolesnika u inozemstvo te tako reducirali troškove.
Cilj je ovog izvješća u proteklom trogodišnjem razdoblju (2019–2022) prikazati: 1) aktivnosti Referentnog centra, 2) postignute stručne rezultate i primjenu novih metoda, postupaka i unaprjeđenje ...struke, i 3) znanstvenu i stručnu suradnju s inozemnim ustanovama visoke razine. Rezultati: Referentni je centar jedino mjesto u Republici Hrvatskoj koje kontinuirano zbrinjava populaciju najugroženijih i najtežih bolesnika pedijatrijske dobi sa srčanom patologijom. Ima kontinuirano, 24 sata dostupnu kardiološku, kardiokiruršku, anesteziološku, neonatalnu i intenzivnu skrb za djecu sa složenim prirođenim i stečenim srčanim bolestima. Također su dostupne metode nadomještanja funkcije organa u zatajivanju (ECMO potpora, LVAD-BiVAD, Berlin-Heart pumpa, hemodijaliza, program transplantacije srca / drugih organa), a sve zahvaljujući timskom radu i suradnji tima nekoliko Zavoda. Izvode se složene kardiokirurške operacije u djece s prirođenim srčanim greškama uz jasan trend povećanja broja i složenosti operacija, te uz i dalje prihvatljivo nisku smrtnost. Danas smo u mogućnosti samostalno liječiti gotovo sve srčane bolesti u djece. Godišnje se izvede oko 200 kateterizacija srca u djece. Više od 40% čine intervencijske procedure, a više od 50% tih intervencija izvodi se u dojenačkom periodu. Tijekom protekle tri godine uvedeno je pet novih perkutanih intervencijskih metoda: liječenje nativne koarktacije i rekoarktacije umetanjem stenta, liječenje stenoze pulmonalnih grana umetanjem stenta, perkutano umetanje valvule na pulmonalnu poziciju, dilatacija postojećeg stenta te zatvaranje aortopulmonalnih kolaterala u djece s univentrikulskim srcem. Navedeni iskoraci učinjeni su kontinuiranim zalaganjem članova tima uz potporu i mentorstvo, odnosno kontinuiranu suradnju s inozemnim stručnjacima iz triju inozemnih ustanova (DeutschesHerzZentrum Muenchen, KinderherzZentrum Linz, Kids Heart Center Budapest). Zaključak: Naš centar stoji uz bok rijetkih centara u Europi koji su u mogućnosti izvesti navedene procedure. Navedene su aktivnosti rezultirale unaprjeđenjem kvalitete skrbi na razini RH i temelj su za daljnji planirani rast i razvoj struke u okvirima naše zemlje.
IntroductionAlagille syndrome is an autosomal dominant disorder that primarily affects the liver and heart. The genetic basis for the development of the syndrome is a mutation in the JAG1 gene (in ...most cases) or the NOTCH2 receptor. The syndrome is clinically characterized by chronic cholestasis, congenital heart defect (most often pulmonary stenosis), abnormalities in the skeletal system (‘butterfly’ vertebrae), changes on the eyes (posterior embryotoxon) and characteristic phenotype. The prevalence of the syndrome is estimated at 1:30 000 live births.Case ReportWe will present a male infant aged 10 months with a diagnosis of Alagille syndrome. The boy was born from his mother’s first, regularly controlled pregnancy. The boy was vital at birth with mild neonatal jaundice. On the third day of life, a systolic murmur was observed over the precordium, louder along the left edge of the sternum, and a diagnosis of tetralogy of Fallot was made by echocardiography. During the next examination, at the age of one month, the classic form of tetralogy of Fallot was verified by ultrasound with infundibular and valvular stenosis of the pulmonary artery with ultrasound pressure gradient over pulmonary stenosis of about 50 mmHg. The infant, then at the age of 4 months, underwent invasive cardiac procedure during the next hospitalization, showing pulmonary branches: the initial part of the right branch was 5 mm, followed by a fully hypoplastic right branch (3 mm) with middle segment stenosis up to 2 mm; the left branch is even more hypoplastic, entirely 2.2 mm with 2 mm segments. According to the finding, surgery was performed to establish an aortic-pulmonary anastomosis in the form of an mBT (modified Blalock Taussig) compound. In addition to cardiac processing, elevated levels of liver transaminases, primarily GGT enzymes, were observed in the findings. Due to the justified suspicion of Alagille’s syndrome (tetralogy of Fallot with elevated liver transaminases), an X-ray of the spine was performed, where the fusion of the bodies of the Th6 and Th8 vertebrae was found, the so-called ‘butterfly’ vertebrae. Among other things, an ophthalmological examination was performed where the posterior embryotoxon of both eyes was observed. Complete processing met the higher diagnostic criteria for Alagille’s syndrome, and therefore genetic analysis was requested, which confirmed the heterozygous variant in the JAG1 gene and diagnosed Alagille’s syndrome. During the last hospitalization, cardiac catheterization was performed, which still showed hypoplastic pulmonary branches with a narrow mBT joint.Discussion/ConclusionAlthough pulmonary stenosis is the most common heart defect within Alagille syndrome, tetralogy of Fallot with hypoplasia of the pulmonary branches may also be one of the clinical manifestations. Since the pulmonary branches are still hypoplastic in our patient after aorthopulmonary anastomosis, in the idea of better pulmonary blood supply, dilatation of the pulmonary valve with a balloon was performed for better anterograde flow until a decision on further treatment modality.
Background: The prevalence of heart failure is constantly increasing in both children and adults. End-stage heart failure in children unresponsive to medical therapy has limited treatment options. ...Surgical options include heart transplantation or implantation of durable ventricular assist devices (VADs). To start the VAD program, it was necessary to train core team members, invite experienced proctors and adjust the organizational approach. Methods: We present our first seven pediatric patients who underwent a VAD implantation with primary indication end-stage dilated cardiomyopathy. Results: The median age on implant was four and a half years and the median duration of VAD support was 39 days with long term survival achieved in three patients. The causes of death were multiorgan failure, thromboembolic events, sepsis, and low cardiac output syndrome. Ischemic stroke was the reason for successful neurointervention during VAD support in two patients. Conclusions: To establish a VAD program, numerous specialties must be included with adequate training and learning for all team members.
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