• 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

A 6-year-old boy, born with hypoplastic left heart syndrome, underwent total cavopulmonary connection and later presented in a significantly deteriorated condition. A CT scan revealed multiple thrombi in the extracardiac conduit, although the patient was maintained on an effective anticoagulant therapy. Further examination revealed anamnestic antibodies suggesting that the patient had gone through a clinically inapparent COVID-19 infection, which we conclude most likely contributed to his hypercoagulable state and led to the formation of significant thrombi impairing the patient's haemodynamics. The patient underwent a surgical thrombectomy; there were no post-operative thrombotic complications.

• MeSH
• antikoagulancia terapeutické užití   MeSH
• COVID-19 * MeSH
• dítě MeSH
• Fontanova operace * škodlivé účinky   MeSH
• lidé MeSH
• pooperační komplikace MeSH
• syndrom hypoplazie levého srdce * chirurgie   MeSH
• trombóza * etiologie   MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

Autoři popisují kazuistiku 5letého chlapce s infekční endokarditidou nativní aortální chlopně, která se nejprve projevila febriliemi a výraznými bolestmi kyčelního kloubu imitujícími coxitidu, které byly způsobeny septickou embolizací, s následným vznikem významného srdečního šelestu při rychlé destrukci cípu aortální chlopně. Stav si vyžádal urgentní operační řešení, Rossovu operaci. Jako etiologické agens byl zjištěn virulentní Streptococcus pneumoniae sérotyp 15B/C.

We are presenting a case report of a 5-year-old boy with infective endocarditis who presented with spiking high fevers, fierce pain in hip joint imitating coxitis caused by septic embolization. Subsequently a severe heart murmur appeared due to fast destruction of previously healthy aortic valve. Overall condition of the patient required an urgent surgical treatment, the Ross procedure was performed. An aggressive serotype 15B/C of Streptococcus pneumoniae was revealed as causing pathogen.

• MeSH
• antibakteriální látky aplikace a dávkování   terapeutické užití   MeSH
• aortální chlopeň abnormality   chirurgie   diagnostické zobrazování   MeSH
• bakteriální endokarditida * diagnóza   etiologie   terapie   MeSH
• horečka MeSH
• lidé MeSH
• pneumokokové infekce * diagnóza   farmakoterapie   patologie   MeSH
• pneumokokové vakcíny MeSH
• předškolní dítě MeSH
• výsledek terapie MeSH
• Check Tag
• lidé MeSH
• mužské pohlaví MeSH
• předškolní dítě MeSH
• Publikační typ
• kazuistiky MeSH

Background The aim of this study was to evaluate long-term survival and freedom from coronary artery reintervention after the arterial switch operation (ASO). Methods and Results This single-center nationwide retrospective study included consecutive children who underwent ASO between 1990 and 2016 (n=605). Long-term outcomes were obtained by cross-mapping individual data with the National Death Registry and the National Registry of Cardiovascular Interventions for adults. A control group was randomly retrieved at a 1:10 ratio from the National Birth and Death Registries. Early mortality was 3.3% and late mortality was 1.7% during a median follow-up of 10 (interquartile range, 5-16) years. The probability of overall survival at 20 years after ASO was 94.9% compared with 99.5% in the background population (hazard ratio [HR] 15.6; 95% CI, 8.9-27.5, P<0.001). Independent multivariable predictors of worse survival were an intramural coronary artery (HR, 5.2; 95% CI, 1.8-15.2, P=0.002) and period of ASO 1990 to 1999 (HR, 4.6; 95% CI, 1.5-13.6, P<0.001). Fourteen patients (2.3%) required 16 coronary artery reoperations. Freedom from coronary artery reintervention at 20 years after ASO was 96%. The only independent multivariable predictor associated with a higher hazard for coronary artery reintervention was an intramural coronary artery (HR, 33.9; 95% CI, 11.8-97.5, P<0.001). Conclusions Long-term survival after ASO is excellent. Coronary artery reinterventions are rare. An intramural coronary artery was an independent predictor associated with a higher risk for coronary artery reintervention and death, regardless of the surgical period.

• MeSH
• anomálie koronárních cév diagnostické zobrazování   epidemiologie   chirurgie   MeSH
• časové faktory MeSH
• dítě MeSH
• doba přežití bez progrese choroby MeSH
• dospělí MeSH
• hodnocení rizik MeSH
• kojenec MeSH
• korekce transpozice velkých arterií * škodlivé účinky   mortalita   MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• novorozenec MeSH
• pooperační komplikace mortalita   chirurgie   MeSH
• předškolní dítě MeSH
• přežívající MeSH
• registrace MeSH
• reoperace MeSH
• replantace * škodlivé účinky   mortalita   MeSH
• retrospektivní studie MeSH
• rizikové faktory MeSH
• transpozice velkých cév diagnostické zobrazování   epidemiologie   chirurgie   MeSH
• Check Tag
• dítě MeSH
• dospělí MeSH
• kojenec MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• novorozenec MeSH
• předškolní dítě MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• srovnávací studie MeSH
• Geografické názvy
• Česká republika MeSH