BACKGROUND: Idiopathic scoliosis (IS) is a structural spinal deformity that can affect the position of the head. One of the etiological hypotheses is that it can be caused by dysfunction of the vestibular system, which can cause abnormal perception of subjective visual vertical (SVV). OBJECTIVE: This study aimed to evaluate the differences in head position and its possible correlation with the perception of SVV in children with IS. METHODS: We examined 37 patients with IS and 37 healthy individuals. The position of the head was evaluated from digital photographs, where we compared the coronal head tilt and the coronal shoulder angle. Measurement of SVV perception was performed using the Bucket method. RESULTS: Coronal head tilt values were significantly different between the groups (median 2.3° [interquartile range 1.8-4.2] vs 1.3° [0.9-2.3], p = 0.001; patients vs. controls). There was a significant difference in SVV between the groups (2.33° [1.40-3.25] vs 0.50° [0.41-1.10], p < 0.001; patients vs controls). There was a correlation between the side of head tilt and the side of SVV in patients with IS (χ2 = 5.6, p = 0.02). CONCLUSIONS: Patients with IS had a greater head tilt in the coronal plane and impaired SVV perception.

• Klíčová slova
• Head tilt, space orientation, spine deformity, vestibular function,
• MeSH
• dítě MeSH
• lidé MeSH
• skolióza * MeSH
• vestibulární aparát * MeSH
• vnímání prostoru MeSH
• zraková percepce MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem 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.

• Klíčová slova
• ELF test, Fontan procedure, liver fibrosis, liver nodules, magnetic resonance imaging, procollagen III amino-terminal peptide,
• 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.

• Klíčová slova
• Fontan procedure, fenestration, survival, total cavopulmonary connection,
• 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

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.

• Klíčová slova
• Cardiopulmonary exercise testing, Exercise capacity, Fontan circulation, Oxygen consumption, Total cavopulmonary connection,
• 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