INTRODUCTION AND OBJECTIVES:Stent implantation has become the gold standard for the treatment of native coarctation of the aorta (CoA) and recurrent CoA in adolescents and adults. In smaller children, however, stent implantation remains technically challenging due to anatomical constraints and growth considerations. Furthermore, data on the optimal treatment strategy in this age group are still limited. This study retrospectively analyzed clinical outcomes in young children treated with stent implantation for recurrent aortic arch stenosis. METHODS:A total of 101 patients (63 male; 62%) with a body weight of less than 15kg were treated with endovascular stent implantation for restenosis of the reconstructed aortic arch (n=94; 93%) or native CoA (n=7; 7%). The median age at the time of stent implantation was 4.8 months (IQR, 3.2-9.6 months) with a median body weight of 5.9kg (IQR, 4.7-8.4kg). The median follow-up period was 46.4 months (IQR, 11.0-76.6 months). RESULTS:All procedures were successful, and no serious complications occurred. The peak-to-peak gradient decreased significantly from a median of 32.5mmHg (IQR, 17.3-46.0mmHg) to 0.0mmHg (IQR, 0.0-2.5mmHg; P<.005). The diameter of the narrow segment increased from a median of 3.0mm (IQR, 2.0-4.0mm) to 6.9mm (IQR, 6.0-8.0mm; P<.005). During follow-up, none of the patients required reoperation. CONCLUSIONS:Stent implantation is a safe and feasible treatment option in patients with restenosis after complex aortic arch reconstruction or native CoA in whom surgical treatment would pose an elevated risk of complications. However, repeated dilatations and, ultimately, intentional stent fracture are required during follow-up.

Introducción y objetivos El implante de stent se ha convertido en el tratamiento de referencia de la coartación aórtica (CoA) nativa y la CoA recurrente en adolescentes y adultos. Sin embargo, en los niños más pequeños, el implante de stent sigue siendo un reto técnico debido a las limitaciones anatómicas y al crecimiento. Además, los datos sobre la estrategia de tratamiento óptima en este grupo de edad siguen siendo limitados. Este estudio analizó retrospectivamente los resultados clínicos en niños pequeños tratados con implante de stent para la estenosis recurrente del arco aórtico. Métodos Se trató a 101 pacientes (63 varones; 62%) con un peso corporal inferior a 15kg con implante de stent por reestenosis del arco aórtico reconstruido (n=94; 93%) o de la CoA nativa (n=7; 7%). La mediana de edad en el momento del implante fue de 4,8 meses (RIC, 3,2-9,6 meses) con una mediana de peso corporal de 5,9kg (RIC, 4,7-8,4kg). La mediana de seguimiento fue de 46,4 meses (RIC, 11,0-76,6 meses). Resultados Todos los procedimientos fueron satisfactorios y no se produjeron complicaciones graves. El gradiente pico-pico disminuyó significativamente de una mediana de 32,5mmHg (RIC, 17,3-46,0mmHg) a una mediana de 0,0mmHg (RIC, 0,0-2,5mmHg; p<0,005). El segmento estrecho se dilató de una mediana de 3,0mm (RIC, 2,0-4,0mm) a 6,9mm (RIC, 6,0-8,0mm; p<0,005). Durante el seguimiento, ningún paciente requirió reintervención. Conclusiones El implante de stent es una modalidad de tratamiento segura y factible en pacientes con reestenosis tras una reconstrucción compleja del arco aórtico o CoA nativa en los que el tratamiento quirúrgico supondría un elevado riesgo de presentar complicaciones. Sin embargo, durante el seguimiento son necesarias dilataciones repetidas y, en última instancia, la fractura intencionada de los stents.

Uvod: Cilj rada je kroz retrospektivnu analizu vlastitih podataka prikazati današnje mogućnosti intervencijskog liječenja u Republici Hrvatskoj, s posebnim osvrtom na pojedine metode. Ispitanici i metode: Retrospektivno su analizirani podatci o učinjenim kateterizacijama u Klinici za pedijatriju, Zavodu za pedijatrijsku kardiologiju, u periodu od 1. siječnja 2020 godine do 31. prosinca 2024. Učinjena je deskriptivna statistika slučajeva, izdvojen postotak kateterizacija tijekom kojih je učinjena intervencija te posebno izdvojena skupina intervencija koje predstavljaju nove metode uvedene tih godina. Rezultati: u navedenom periodu, učinjeno je ukupno 866 kateterizacija raspodjelom kako slijedi: 2020. godine 129 kateterizacija, 2021. godine 184 pacijenata, 2022. godine 189 kateterizacija, 2023. godine 215, a 2024. godine 149 kateterizacija. Od ukupnog broja od 866 kateterizacija, učinjeno je 289 intervencija (33.4 %). Neke su intervencijske metode uvedene ranije, poput balonske atrioseptostomije po Rashkindu, koja je u ovom razdoblju učinjena u 50 pacijenata, balonske dilatacije novorođenčake kritične aortne (15 pacijenata) ili kritične pulmonalne stenoze (15 pacijenata). U navedenom periodu učinjeno je 105 biopsija miokarda, poglavito u djece sa stanjem nakon transplanatcije srca. Od 2018 godine uvedene su nove metode, etablirane u opisivanom periodu od 2020-2024, izvedene kako slijedi: imlantacije Melody valvule u izlazni trakt desne klijetke u 6 pacijenata, umetanje stentova u nativnu koarktaciju, rekoarktaciju, stenozu pulmonalnih grana (20 pacijenata), dilataciju postojećih stentova (13 pacijenata), zatvaranje aortopulmonalnih kolaterala u pacijenata sa univentrikulskih srcem, zatvaranje venovenskih kolaterala u pacijanata sa univentrikuslkim srcem (8 pacijenata). Zaključak: današnje mogućnosti intervencijskog liječenja Republici Hrvatskoj na visokoj su razini, uz stalan porast broja zahvata, udjela intervencija u ukupnom broju zahvata te same kompleksnosti intervencijskih zahvata.

By anchoring different cell structures like Z-bands, mitochondria, and desmosomes to the cytoskeleton, desmin filaments are essential for cellular integrity, signal transduction and mitochondrial function. The spectrum of clinical phenotypes associated with DES gene mutations is wide and heterogeneous. The most common clinical presentations of desminopathy include cardiomyopathy, cardiac conduction disease, and progressive skeletal myopathy. We present a case of an 11-year-old girl with progressive dilated cardiomyopathy (DCM) needing ECMO treatment. ECMO treatment was complicated by the early development of intracardiac thrombi and lung necrosis. Post-mortem exome sequencing revealed the causative, previously unreported, de novo mutation of DES gene, c.365 A > C, p.Tyr122Ser, characterized with unusually progressive clinical course leading to death.

BACKGROUND:Sacubitril/valsartan, an angiotensin receptor-neprilysin inhibitor (ARNI), is an established treatment for heart failure (HF) with reduced left ventricular ejection fraction. It has not been rigorously compared with angiotensin-converting enzyme inhibitors in children. PANORAMA-HF (Prospective Trial to Assess the Angiotensin Receptor Blocker Neprilysin Inhibitor LCZ696 Versus Angiotensin-Converting Enzyme Inhibitor for the Medical Treatment of Pediatric HF) is a randomized, double-blind trial that evaluated the pharmacokinetics and pharmacodynamics (PK/PD), safety, and efficacy of sacubitril/valsartan versus enalapril in children 1 month to <18 years of age with HF attributable to systemic left ventricular systolic dysfunction (LVSD). METHODS:Children with HF attributable to LVSD were randomized to sacubitril/valsartan versus enalapril to assess the efficacy and safety of sacubitril/valsartan at 52 weeks of follow-up. The primary end point of the study was to determine whether sacubitril/valsartan was superior to enalapril for the treatment of pediatric patients with HF attributable to systemic LVSD, assessed using a primary global rank end point consisting of ranking patients from worst to best on the basis of clinical events such as death, listing for urgent heart transplant, mechanical life support requirement, worsening HF, New York Heart Association (NYHA)/Ross class, Patient Global Impression of Severity (PGIS), and Pediatric Quality of Life Inventory physical functioning domain. The change from baseline to 52 weeks in NT-proBNP (N-terminal pro-B-type natriuretic peptide) was an exploratory end point. RESULTS:A total of 375 children (mean age, 8.1 +/- 5.6 years; 52% female) were randomized to sacubitril/valsartan (N=187) or enalapril (N=188). At week 52, no significant difference was observed between the 2 treatment arms in the global rank end point (Mann-Whitney probability, 0.52 [95% CI, 0.47-0.58]; Mann-Whitney odds, 0.91 [95% CI, 0.72-1.14]; P=0.42). At week 52, clinically meaningful reductions were observed in both treatment arms in NYHA/Ross, PGIS, Patient Global Impression of Change, and NT-proBNP, without significant differences between groups. Adverse events were similar between treatment arms (incidence: sacubitril/valsartan, 88.8%; enalapril, 87.8%), and the safety profile of sacubitril/valsartan was acceptable in children. CONCLUSIONS:In this study, sacubitril/valsartan did not show superiority over enalapril in the treatment of children with HF attributable to systemic LVSD using the prespecified global rank end point. However, both treatment arms showed clinically meaningful improvements over 52 weeks.

Cardiac myxomas are the most common benign cardiac neoplasms. Echocardiography is the first-line imaging modality used to analyze cardiac masses, allowing the detection of tumor location, size, and mobility. However, additional imaging techniques are required to confirm the diagnosis, evaluate tissue characteristics of the mass, and assess potential invasion of surrounding structures. Second-line imaging includes cardiac magnetic resonance imaging (MRI) and/or computed tomography (CT) depending on availability and the patient’s characteristics and preferences. The advantages of CT include its wide availability and fast scanning, which allows good image quality even in patients who have difficulty cooperating. MRI has excellent soft-tissue resolution and is the gold standard technique for noninvasive tissue characterization. In some cases, evaluation of the tumor metabolism using 18F-fluorodeoxyglucose positron emission tomography with CT may be useful, mainly if the differential diagnosis includes primary or metastatic cardiac malignancies. A cardiac myxoma can be identified by its characteristic location within the atria, typically in the left atrium attached to the interatrial septum. The main differential diagnoses include physiological structures in the atria like crista terminalis in the right atrium and the coumadin ridge in the left atrium, intracardiac thrombi, as well as other benign and malignant cardiac tumors. In this review paper, we describe the characteristics of cardiac myxomas identified using multimodality imaging and provide tips on how to differentiate myxomas from other cardiac masses.

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.

IntroductionIn children, congenital heart defects represent the primary cause of increased serum troponin I. The elimination process of cardiac troponin I from the bloodstream and the factors influencing this process remain unknown. The objective of this study was to explore the role of troponin I as an indicator of cardiac damage in children both in serum and urine, a concept previously investigated in adults.MethodsOur prospective study involved 70 children under 24 months of age. The first group underwent ventricular septal defect repair, while the second group involved children who had undergone partial cavopulmonary anastomosis. For these groups, urine and serum troponin I were assessed on four occasions. The third group, consisting of healthy children, underwent a single measurement of urine troponin I.ResultsSerum troponin I values exhibited an expected elevation in the early postoperative period, followed by a return to lower levels. Significantly higher concentrations of serum troponin I were observed in the first group of children (p < 0.05). A positive correlation was found between troponin I in the first three measurements and cardiopulmonary bypass and aortic cross-clamping time. There was no discernible increase in urine troponin I directly related to myocardial damage; troponin I couldn't be detected in most urine samples.DiscussionThe inability to detect troponin I in urine remains unexplained. Potential explanatory factors may include the isoelectric point of troponin I, elevated urinary concentrations of salts and urea, variations in urine acidity (different pH levels), and a relatively low protein concentration in urine.

Background: The use of high-sensitive cardiac troponin T (hsTnT) in urine as a marker of cardiac damage in children has not yet been reported. Elimination of cardiac troponins is dependent on renal function; persistently increased serum hsTnT concentrations were observed among individuals with impaired renal function. The aim of this study was to investigate serum and urine hsTnT levels and its correlation in infants and children younger than 24 months of age after cardiac surgery. Methods: This study was conducted on 90 infants and children under 24 months of age who were divided into three groups. The experimental group consisted of patients with intracardiac surgery of ventricular septal defect (VSD), first control group consisted of infants with extracardiac formation of bidirectional cavopulmonary connection (BCPC), and the second control group consisted of healthy children. Troponin T values ​​were determined in serum and urine at five time points: the first sample was taken on the day before cardiac surgery (measure 0) and the other four samples were taken after the surgery; immediately after (measure 1), on the first (measure 2), third (measure 3), and fifth postoperative day (measure 5). The first morning urine was sampled for determining the troponin T in the control group of healthy infants. Results: A positive correlation between troponin T values in serum and urine was found. Urine hsTnT measured preoperatively in children undergoing BCPC surgery was higher (median 7.3 [IQR 6.6–13.3] ng/L) compared to children undergoing VSD surgery (median 6.5 [IQR 4.4–8.9] ng/L) as well as to healthy population (median 5.5 [IQR 5.1–6.7] ng/L). After logarithmic transformation, there was no statistically significant difference in urine hsTnT concentration between the groups at any point of measurement preoperatively or postoperatively. Statistically significant negative correlation was found between serum and urine hsTnT concentrations and glomerular filtration rate estimated by creatinine clearance. Patients who underwent surgical repair of VSD had significantly higher concentrations of troponin T in serum on the first three postoperative measurements compared to those who had BCPC surgery. Conclusions: According to the results of this study, renal function after cardiac surgery appears to have a major effect on the urinary hsTnT concentrations, and we cannot conclude that this is an appropriate marker for the assessment of postoperative myocardial damage in children. Nevertheless, more research is needed to reach a better understanding of the final elimination of cardiac troponins in children.

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.

INTRODUCTION:Extracorporeal membrane oxygenation (ECMO) is an important treatment option for organ support in respiratory insufficiency, cardiac failure, or as an advanced tool for cardiopulmonary resuscitation. Reports on pediatric ECMO use in our region are lacking.METHODS:This study is a retrospective review of all pediatric cases that underwent a veno-arterial (VA) or veno-venous (VV) ECMO protocol between November 2009 and August 2020 at the Department of Pediatrics, University Hospital Center Zagreb, Croatia.RESULTS:Fifty-two ECMO runs identified over the period; data were complete for 45 cases, of which 23 (51%) were female, and median age was 8 months. Thirty-eight (84%) patients were treated using the VA-and 7 (16%) using VV-ECMO. The overall survival rate was 51%. Circulatory failure was the most common indication for ECMO (N = 38, 84%), and in 17 patients ECMO was started after cardiopulmonary resuscitation (E-CPR). Among survivors, 74% had no or minor neurological sequelae. Variables associated with poor outcome were renal failure with renal replacement therapy (p < .001) and intracranial injury (p < .001).CONCLUSION:Overall survival rate in our cohort is comparable to the data published in the literature. The use of hemodialysis was shown to be associated with higher mortality. High rates of full neurological recovery among survivors are a strong case for further ECMO program development in our institution.

We report the case of a full term male child with multiple organ dysfunctions caused by mutations of the TTN gene. Our patient had cardiac abnormalities verified by fetal echocardiography at 26 weeks of gestational age, when fetal hydrops due to cardiac decompensation was shown. Due to specific appearance seen at birth – generalized oedema, light facial and sexual dimorphism with hypogenital-ism and chryptorchidism, axial hypotonia in addition to the proven congenital heart defects, a genetic disorder or syndrome with muscle involvement was suspected. Multiple comorbidities were present since birth, mainly cardiac and neurologic, some of which have been described in patients with core myopathies (atrial and ventricular septal defects, supraventricular tachycardia, hypotonia, developmental delay, myopathy).

Total Anomalous Pulmonary Venous Return (TAPVR) is an infrequent congenital heart de-fect, occurring in about 1 in 15,000 live births. The condition involves an abnormal con-nection where pulmonary veins, rather than attaching to the left atrium, drain into the right atrium or systemic venous circulation.

Objectives: Severe hypoxemia in the early postoperative period after bidirectional cavopulmonary shunt (BCPS) is a critical complication. We aimed to evaluate patients who underwent additional systemic to pulmonary shunt and septation of central pulmonary artery (partial takedown) after BCPS. Methods: The medical records of all patients who underwent BCPS between 2007 and 2020 were reviewed. Patients who underwent partial takedown were extracted and their outcomes were analyzed. Results: Of 441 BCPS patients, 27 patients (6%) required partial takedown. Most frequent diagnosis was hypoplastic left heart syndrome (n = 14; 52%). Additional complicating factors included pulmonary artery hypoplasia (n = 12) and pulmonary venous obstruction (n = 3). Thirteen patients (48%) underwent partial takedown on the same day of BCPS, and all of them survived the procedure. The remaining 14 patients (52%) underwent partial takedown between postoperative 1 to 64 days. The reasons for partial takedown were: postoperative high pulmonary vascular resistance (n = 4), early BCPS (<90 days) with PA hypoplasia (n = 3), mediastinitis/pneumonia (n = 3), pulmonary venous obstruction (n = 2), ventricular dysfunction (n = 1), and recurrent pneumothorax (n = 1). Four patients experienced hospital deaths. Six patients died after discharge, 10 achieved Fontan completion, and 6 were alive and waiting for Fontan. Overall survival after partial takedown was 54% at 3 years. The pulmonary venous obstruction (P = .041) and genetic/extracardiac anomalies (P = .085) were identified as risks for mortality after partial takedown. Conclusions: The partial takedown resulted in a 3-year survival rate of more than 50%. Of these patients, a significant number underwent successful Fontan completion who would exhibit potential early death with conservative treatment.

Cardiac tumors are neoplasms arising from or located in the heart or the pericardium. Although rare, primary cardiac tumors in children require an accurate and timely diagnosis. Most pediatric primary cardiac tumors are benign (around 90%). Echocardiography is the first imaging modality used due to its availability, noninvasiveness, inexpensiveness, and absence of ionizing radiation. Computed tomography (CT) and magnetic resonance imaging (MRI) offer better soft tissue visualization as well as better visualization of extracardiac structures. A great advantage of MRI is the possibility of measuring cardiac function and blood flow, which can be important for obstructing cardiac tumors. In this article, we will offer a brief review of clinical, echocardiographic, CT, and MRI features of cardiac rhabdomyomas, fibromas, teratomas, and lipomas providing their differential diagnosis.

We report the case of a full term male child with multiple organ dysfunctions caused by mutations of the TTN gene. Our patient had cardiac abnormalities verified by fetal echocardiography at 26 weeks of gestational age, when fetal hydrops due to cardiac decompensation was shown. Due to specific appearance seen at birth – generalized oedema, light facial and sexual dimorphism with hypogenitalism and chryptorchidism, axial hypotonia in addition to the proven congenital heart defects, a genetic disorder or syndrome with muscle involvement was suspected. Multiple comorbidities were present since birth, mainly cardiac and neurologic, some of which have been described in patients with core myopathies (atrial and ventricular septal defects, supraventricular tachycardia, hypotonia, developmental delay, myopathy). Due to the complexity of the heart defect at the age of 2 months a partial pulmonary trunk banding and ligation of the ductus Botalli (PDA) was initially carried out, and at 9 months a complete correction of heart defect was performed. Our patient also had failure to thrive as well as recurrent vomiting, wich was later on asociated with extraluminal compression of the proximal duodenum, intestinal malrotation and malfixation. Although a surgical approach somewhat increased tolerance of peroral food intake, chronic diarrhoea and failure to thrive persisted. In addition, our patient had several metabolic and endocrine disorders including hypocalcemia, hypomagnesemia, hyponatremia, repeatedly elevated levels of PTH, hypothyroidism etc. Our patient had recurrent, mainly respiratory infections with multiresistant microorganisms, and recuired intubation and long-lasting mechanical ventilation. The above mentioned multiple disorders of various organ systems, with complications caused by multiple sepses, ultimately resulted in multiorgan failure, and despite all the applied treatment at the ICU, resulted in a lethal outcome. Coresponding findings described in other patients with core myopathies included neonatal hypotonia, poor suckling, severe motor skill delay, complex heart defects and cardiomyopathy.

Hypoplastic left heart syndrome (HLHS) is rare congenital heart defect in which the left side of the heart is severely underdeveloped. It has been a lethal congenital heart anomaly until the last four decades until three palliative surgeries were established (operation by Norwood, Glenn and Fontan). The aim of this study was to evaluate the outcomes of treating patients with HLHS. The main methods were statistical, and the clinical characteristics were retrospectively reviewed. We included 132 patients in 20-year period who have been treated at University Hospital Centre Zagreb, operated in our and in foreign centers. We followed them before, in the meantime and after final operation. Of all patients, 69 survived and 63 died. The highest mortality was in period between operation by Norwood and Glenn in early infancy and accounts for almost 48% all lethal outcomes. The most common anatomic variant is the mitral atresia and aortic atresia (MA-AA) subtype and the rarest is mitral stenosis and aortic atresia (MS-AA) subtype. Apart of a three – staged operation procedures, 53 patients required one or more interventions involving implantation of the stent into the pulmonary branches, isthmus the aorta, the Sano or mBT junction, and the dilatation of the same, then coiling of the arteriovenous malformations, electrostimulator implantation and the closure of fenestra. The most common interventions are stent implantation into the pulmonary branches and dilatation of the aortic recoarctation and stenosis of the pulmonary branch stent. In twelve patients, fenestra was closed with an Amplatzer occluder. The mean follow-up age operation by Fontan (TCPC) is 7.64 years (1.1 – 16.5 years). Two patients were transferred to the GUCH population. This retrospective study included 132 patients with hypoplastic left heart syndrome in twenty-year period who have been treated at University Hospital Centre Zagreb. The overall survival of all patients is 52.2%. The highest mortality was in period between operation by Norwood and Glenn in early infancy. The most common interventions are stent implantation into the pulmonary branches and dilatation of recoarctation of the aorta. Mean follow-up age operation by Fontan (TCPC) is 7.64 years.