BACKGROUND: Heart transplantation (HTx) is an established therapeutic option for children with end-stage heart failure. Comprehensive pediatric nationwide HTx program was introduced in 2014 in the Czech Republic. The aim of this study was to evaluate its mid-term characteristics and outcomes and to compare them with international data. METHODS: Retrospective observational study, including all patients who underwent HTx from June 2014 till December 2022. Data from the institutional database were used for descriptive statistics and survival analyses. RESULTS: A total of 30 HTx were performed in 29 patients with congenital heart disease (CHD, N = 15, single ventricular physiology in 10 patients) and cardiomyopathy (CMP, N = 14). Ten patients were bridged to HTx by durable left ventricular assist devices (LVADs) for a mean duration of 104 (SD 89) days. There was one early and one late death during median follow-up of 3.3 (IQR 1.3-6.1) years. Survival probability at 5 years after HTx was 93%. Two patients underwent re-transplantation (one of them in an adult center). Significant rejection-free survival at 1, 3, and 6 years after HTx was 76%, 63%, and 63%, respectively. CONCLUSIONS: The introduced pediatric HTx program reflects the complexity of the treated population, with half of the patients having complex CHD and one-third being bridged to HTx by LVADs. Mid-term results are comparable to worldwide data. The data confirm the possibility of establishing a successful nationwide pediatric HTx program in a relatively small population country with well-developed pediatric cardiovascular care and other transplantation programs.

• MeSH
• dítě MeSH
• dospělí MeSH
• kardiomyopatie * MeSH
• lidé MeSH
• podpůrné srdeční systémy * MeSH
• retrospektivní studie MeSH
• srdeční selhání * chirurgie   MeSH
• transplantace srdce * MeSH
• vrozené srdeční vady * MeSH
• výsledek terapie MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• pozorovací studie MeSH
• Geografické názvy
• Česká republika MeSH

Vrozené srdeční vady jsou jednou z nejčastějších příčin úmrtí u novorozenců ve všech váhových kategoriích. Novorozence nízké porodní hmotnosti definujeme jako novorozence s porodní hmotností pod 2500 g, může se jednat o donošeného hypotrofického novorozence nebo nedonošeného novorozence bez ohledu na jeho trofiku. Příspěvek se věnuje managementu péče o novorozence a také prezentuje výsledky našeho pracoviště v dané oblasti.

Congenital heart defects are among the most common causes of death in newborns in all weight categories. We define a low birth weight newborn as a newborn with a birth weight below 2500 g, it can be a full-term hypotrophic newborn or a premature newborn, regardless of its trophic status. This article addresses the care management of newborns and also presents the results of our hospital in this field.

• MeSH
• intenzivní péče o novorozence metody   MeSH
• lidé MeSH
• novorozenec s nízkou porodní hmotností * MeSH
• novorozenec MeSH
• statistika jako téma MeSH
• vrozené srdeční vady * chirurgie   epidemiologie   mortalita   terapie   MeSH
• Check Tag
• lidé MeSH
• novorozenec MeSH
• ženské pohlaví MeSH
• Publikační typ
• kazuistiky MeSH

Background The goal of this study was to evaluate long-term results of percutaneous balloon valvuloplasty (BVPL) used exclusively for initial management of congenital aortic stenosis in children. Methods and Results A total of 409 consecutive pediatric patients (134 newborns, 275 older patients) who underwent BVPL as initial treatment of aortic stenosis in a single nationwide pediatric center were subjected to a retrospective follow-up study. The resulting follow-up time reached a median of 18.5 (interquartile range, 12.2-25.1) years. Successful BVPL was defined by residual Doppler gradient <70/40 (systolic/mean) mm Hg. The primary end point was death; secondary end points included any valve reintervention, balloon revalvuloplasty, any aortic valve surgery, and aortic valve replacement, respectively. BVPL effectively reduced the peak and mean gradient both immediately and at the latest follow-up (P<0.001). There was significant procedure-related progression of aortic insufficiency (P<0.001). Higher aortic annulus z score was predictive for severe aortic regurgitation (P<0.05) and lower z score for insufficient gradient reduction (P<0.05). The actuarial probability of survival/survival free from any valve reintervention was 89.9%/59.9%, 85.9%/35.2%, and 82.0%/26.7% at 10, 20, and 30 years after first BVPL, respectively. Left ventricular dysfunction or arterial duct dependency as the indication for BVPL was predictive of both worse survival and survival free from any reintervention (P<0.001). Lower aortic annulus z score and lower balloon-to-annulus ratio were predictive of a need for revalvuloplasty (P<0.001). Conclusions Percutaneous BVPL provides good initial palliation. In patients with hypoplastic annuli and left ventricular or mitral valve comorbidity, the results are less favorable.

• MeSH
• aortální chlopeň diagnostické zobrazování   chirurgie   MeSH
• aortální insuficience * chirurgie   MeSH
• aortální stenóza * diagnostické zobrazování   chirurgie   vrozené   MeSH
• balónková valvuloplastika * škodlivé účinky   MeSH
• dítě MeSH
• kojenec MeSH
• lidé MeSH
• následné studie MeSH
• novorozenec MeSH
• retrospektivní studie MeSH
• vrozené srdeční vady * MeSH
• výsledek terapie MeSH
• Check Tag
• dítě MeSH
• kojenec MeSH
• lidé MeSH
• novorozenec MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH

• Publikační typ
• abstrakt z konference MeSH

AIMS: Fontan palliation is a surgical strategy for patients with complex congenital heart disease, in whom biventricular circulation cannot be achieved. Long-term survival is negatively affected by the absence of sub-pulmonary ventricle and increased systemic venous pressure. Exercise capacity is a known predictor of overall survival and quality of life in congenital heart defects. We aim to track individual trends of peak oxygen uptake (V̇O2 peak) after total cavopulmonary connection (TCPC), identify predictors of deterioration, and derive a disease-specific reference V̇O2 peak dataset. METHODS AND RESULTS: A retrospective study of serial cardiopulmonary exercise testing (CPET) data, gathered from all patients who underwent TCPC in the Czech Republic between 1992 and 2016. Of 354 consecutive patients with TCPC, 288 (81.4%) patients underwent one or more CPETs yielding 786 unique V̇O2 peak values used as a reference dataset. Longitudinal data were available in 206 (58.2%) patients, who underwent a median (inter-quartile range) of 3.0 (2.0-5.0) CPETs over a mean (standard deviation) of 8.9 (5.5) years. The decline of exercise capacity with age was linear and not faster than in healthy peers (P = 0.47), but relative values of V̇O2 peak in TCPC patients were 12.6 mL/min/kg lower. Single ventricular morphology and pulmonary artery size had no significant influence on the exercise capacity dynamics. V̇O2 peak decline correlated negatively with the trend of body mass index z-score (P = 0.006) and was faster in women than men (P = 0.008). CONCLUSIONS: Total cavopulmonary connection patients have significantly reduced exercise capacity. The age-related decline paralleled the healthy population and correlated negatively with the body mass index trend. The presented V̇O2 peak reference dataset may help the clinicians to grade the severity of exercise capacity impairment in individual TCPC patients.

• MeSH
• bypass pravého srdce metody   MeSH
• dítě MeSH
• dospělí MeSH
• Fontanova operace * metody   MeSH
• kvalita života MeSH
• lidé MeSH
• longitudinální studie MeSH
• retrospektivní studie MeSH
• spotřeba kyslíku MeSH
• tolerance zátěže * fyziologie   MeSH
• vrozené srdeční vady * patofyziologie   rehabilitace   chirurgie   MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• klinická studie MeSH
• práce podpořená grantem MeSH

Úvod: Menší část vrozených srdečních vad je tvořena skupinou, kterou souborně označujeme jako vady s funkčně jedinou komorou. Jediným řešením pro tyto pacienty je série paliativních kardiochirurgických zákroků, jejichž cílem je vytvoření tzv. Fontanovské cirkulace. Cílovou operací je v současné době úplné kavopulmonální spojení (Total Cavopulmonary Connection, TCPC), při kterém je napojena horní dutá žíla na větev plicnice přímo a dolní dutá žíla prostřednictvím intrakardiálního nebo extrakardiálního konduitu bez chlopně. Srdce je z plicního oběhu vyřazeno a funkčně jediná srdeční komora je zapojena pouze do systémového oběhu. Soubor a metodika: Cílem práce byla analýza počtu nutných operačních a katetrizačních zákroků a jejich typů v populaci pacientů s TCPC (N = 407) z jediného celostátního centra pro léčbu vrozených srdečních vad v dětském věku. Výsledky: Pravděpodobnost 10 a 20letého přežití pacientů v souboru byla 96 a 92 %. V souboru bylo během sledování provedeno 1 128 kardiochirurgických operací, 299 intervenčních katetrizací a 738 diagnostických katetrizací. Závěr: Celkové dlouhodobé přežití je v populaci pacientů s TCPC v České republice velmi dobré. Tito pacienti jsou však zatíženi velkým počtem nezbytných kardiochirurgických i katetrizačních zákroků.

Background: A small proportion of congenital heart disease cases are united under the diagnosis of a functionally single ventricle. The only management strategy for this group of patients is a series of palliative surgical procedures creating a unique situation of the so-called Fontan circulation. Currently, total cavopulmonary connection (TCPC) is the target intervention in which the superior vena cava is connected directly to the pulmonary artery branch and the inferior vena cava via a valveless intracardiac or extracardiac conduit. The heart is excluded from the pulmonary circulation and the functionally single ventricle is connected only to the systemic circulation. Patients and methods: We aimed to analyse the number of cardiac surgical and catheterisation procedures in a population of patients with TCPC (N = 407) treated in a single paediatric centre covering the whole region of the Czech Republic. Results: The 10-year and 20-year survival probability rates in our study group were 96% and 92%, respectively. A total of 1,128 cardiac surgical procedures, 299 catheterisation procedures, and 738 diagnostic catheterisations were performed. Conclusion: Overall, long-term survival in the population of TCPC patients in the Czech Republic is favourable. However, this group of patients requires a high number of repeated cardiac interventions.

• Klíčová slova
• funkčně jediná komora, totální kavopulmonální spojení,
• MeSH
• analýza přežití MeSH
• lidé MeSH
• retrospektivní studie MeSH
• srdeční katetrizace metody   MeSH
• vrozené srdeční vady * chirurgie   MeSH
• Check Tag
• lidé MeSH

BACKGROUND: The aim of this study was to analyze the relation between the hepatic fibrosis markers, liver morphology and hemodynamics assessed by magnetic resonance imaging (MRI) after total cavopulmonary connection (TCPC). MATERIALS AND METHODS: Adult patients after TCPC performed in childhood between 1993 and 2003 are the subjects of this observational study. The follow-up protocol consisted of clinical and echocardiographic examination, liver elastography, cardiopulmonary exercise test, MRI hemodynamics and liver morphology assessment and direct enhanced liver fibrosis (ELF) test. RESULTS: The cohort consisted of 39 patients (46% female) with a median age at study 26 (IQR 23-28) years and interval from TCPC 21 (IQR 20-23) years. There was no correlation between ELF test and any MRI variables, but procollagen III amino-terminal peptide (PIIINP), a single component of ELF test, correlated significantly with ventricular end-diastolic volume (r = 0.33; p = 0.042) and inferior vena cava flow (r = 0.47; p = 0.003). Fifteen (38%) patients with liver nodules had compared to other 24 patients higher end-diastolic volume (ml/m2) 102.8 ± 20.0 vs. 88.2 ± 17.7; p = 0.023, respectively. PIIINP correlated significantly with inferior vena cava flow (r = 0.56; p = 0.030) and with end-diastolic volume (r = 0.53; p = 0.043), but only in patients with liver nodules. CONCLUSION: Gradual progression of liver fibrosis, particularly hepatic arterialization caused by liver nodules formation, increases inferior vena cava flow and subsequent ventricular volume overload may further compromise single ventricle functional reserve in adult patients after TCPC.

• Publikační typ
• časopisecké články MeSH

INTRODUCTION: Fenestration in the total cavopulmonary connection system may improve the outcome of patients with significant risk factors for Fontan haemodynamics. Our study aims to analyse the difference in long-term survival between non-fenestrated and fenestrated patients. METHODS: All consecutive patients (n = 351) who underwent total cavopulmonary connection between 1992 and 2016 were identified. Six early deaths were excluded resulting in a group of 345 patients. Median (interquartile range,) length of follow-up was 14.4 (7.1-19.7) years. Freedom from the composite endpoint of death, total cavopulmonary connection take-down or indication for a heart transplant was analysed. RESULTS: Fenestration was absent in 237 patients (68.7%, Group 1), was created and closed later in 79 patients (22.9%, Group 2), and remained open in 29 patients (8.4%, Group 3). Mean survival probability until composite endpoint was 97.1 and 92.9% at 10 and 20 years, respectively. Patients with patent fenestration had worse survival (p < 0.001) as compared to both the non-fenestrated and fenestration closure groups. Despite a similar outcome, exercise capacity was lower in Group 2 than 1 (p = 0.013). In 58 patients with interventional fenestration closure, Nakata index was lower at the time of closure than pre-operatively, and both the pressure in the circuit and oxygen saturation in the aorta increased significantly (p < 0.001). CONCLUSIONS: Patients with persisting risk factors preventing fenestration closure are at higher risk of reaching the composite endpoint. Patients after fenestration closure have the worse functional outcome; their survival is, however, not different from the non-fenestrated group.

• MeSH
• analýza přežití MeSH
• arteria pulmonalis * chirurgie   MeSH
• Fontanova operace * škodlivé účinky   MeSH
• hemodynamika MeSH
• lidé MeSH
• rizikové faktory MeSH
• výsledek terapie MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH

• MeSH
• anomálie koronárních cév * diagnóza   chirurgie   MeSH
• cévní píštěle * diagnóza   chirurgie   MeSH
• lidé MeSH
• nemoci koronárních tepen * diagnóza   chirurgie   MeSH
• píštěle * MeSH
• septální okluder * MeSH
• srdeční katetrizace MeSH
• vrozené srdeční vady * MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH

• MeSH
• diferenciální diagnóza MeSH
• dítě MeSH
• gastrointestinální nemoci klasifikace   patologie   MeSH
• genetická onemocnění kůže diagnóza   klasifikace   patologie   terapie   MeSH
• kožní nemoci diagnóza   klasifikace   patologie   terapie   MeSH
• lidé MeSH
• muskuloskeletální nemoci klasifikace   patologie   MeSH
• nemoci endokrinního systému diagnóza   etiologie   klasifikace   patofyziologie   patologie   terapie   MeSH
• neurokutánní syndromy diagnóza   klasifikace   patologie   MeSH
• oční infekce diagnóza   farmakoterapie   klasifikace   patologie   MeSH
• oční symptomy * MeSH
• poruchy pigmentace diagnóza   genetika   klasifikace   patologie   terapie   MeSH
• revmatické nemoci farmakoterapie   klasifikace   patologie   MeSH
• vrozené poruchy metabolismu klasifikace   patologie   terapie   MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• Publikační typ
• přehledy MeSH