This article is to commemorate and celebrate achievements of Professor Milan Šamánek who passed away on 29 April, 2020. Milan was an excellent and visionary paediatric cardiologist who helped to establish paediatric cardiology as a speciality in Czechoslovakia and several other east European countries in the late 1970s. Milan was also paramount for connecting the East and West, helping in no small way to establish the Association for European Paediatric Cardiology (AEPC) as the leading learned society in Europe.

• Klíčová slova
• History, organisation, paediatric cardiology, prenatal cardiology,
• MeSH
• dítě MeSH
• kardiologie * MeSH
• lidé MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Evropa MeSH

IMPORTANCE: Sudden cardiac death (SCD) is the most common mode of death in childhood hypertrophic cardiomyopathy (HCM), but there is no validated algorithm to identify those at highest risk. OBJECTIVE: To develop and validate an SCD risk prediction model that provides individualized risk estimates. DESIGN, SETTING, AND PARTICIPANTS: A prognostic model was developed from a retrospective, multicenter, longitudinal cohort study of 1024 consecutively evaluated patients aged 16 years or younger with HCM. The study was conducted from January 1, 1970, to December 31, 2017. EXPOSURES: The model was developed using preselected predictor variables (unexplained syncope, maximal left-ventricular wall thickness, left atrial diameter, left-ventricular outflow tract gradient, and nonsustained ventricular tachycardia) identified from the literature and internally validated using bootstrapping. MAIN OUTCOMES AND MEASURES: A composite outcome of SCD or an equivalent event (aborted cardiac arrest, appropriate implantable cardioverter defibrillator therapy, or sustained ventricular tachycardia associated with hemodynamic compromise). RESULTS: Of the 1024 patients included in the study, 699 were boys (68.3%); mean (interquartile range [IQR]) age was 11 (7-14) years. Over a median follow-up of 5.3 years (IQR, 2.6-8.3; total patient years, 5984), 89 patients (8.7%) died suddenly or had an equivalent event (annual event rate, 1.49; 95% CI, 1.15-1.92). The pediatric model was developed using preselected variables to predict the risk of SCD. The model's ability to predict risk at 5 years was validated; the C statistic was 0.69 (95% CI, 0.66-0.72), and the calibration slope was 0.98 (95% CI, 0.59-1.38). For every 10 implantable cardioverter defibrillators implanted in patients with 6% or more of a 5-year SCD risk, 1 patient may potentially be saved from SCD at 5 years. CONCLUSIONS AND RELEVANCE: This new, validated risk stratification model for SCD in childhood HCM may provide individualized estimates of risk at 5 years using readily obtained clinical risk factors. External validation studies are required to demonstrate the accuracy of this model's predictions in diverse patient populations.

• MeSH
• dítě MeSH
• hodnocení rizik metody   MeSH
• hypertrofická kardiomyopatie komplikace   mortalita   MeSH
• incidence MeSH
• lidé MeSH
• míra přežití trendy   MeSH
• mladiství MeSH
• náhlá srdeční smrt epidemiologie   etiologie   MeSH
• následné studie MeSH
• prognóza MeSH
• retrospektivní studie MeSH
• rizikové faktory MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH
• práce podpořená grantem MeSH
• Geografické názvy
• Evropa epidemiologie   MeSH

BACKGROUND: We sought to report the frequency, types, and outcomes of left-sided reoperations (LSRs) after an arterial switch operation (ASO) for patients with D-transposition of the great arteries (D-TGA) and double-outlet right ventricle (DORV) TGA-type. METHODS: Seventeen centers belonging to the European Congenital Heart Surgeons Association (ECHSA) contributed to data collection. We included 111 patients who underwent LSRs after 7,951 ASOs (1.4%) between January 1975 and December 2010. Original diagnoses included D-TGA (n = 99) and DORV TGA-type (n = 12). Main indications for LSR were neoaortic valve insufficiency (n = 52 [47%]) and coronary artery problems (CAPs) (n = 21 [19%]). RESULTS: Median age at reoperation was 8.2 years (interquartile range [IQR], 2.9-14 years). Seven patients died early after LSRs (6.3%); 4 patients with D-TGA (5.9%) and 3 patients with DORV TGA-type (25%) (p = 0.02). Median age at last follow-up was 16.1 years (IQR, 9.9-21.8 years). Seventeen patients (16%) required another reoperation, which was more frequent in patients with DORV- TGA type (4 of 9 [45%]) than in patients with D-TGA (13 of 95 [14%]). Late death occurred in 4 patients (4 of 104 [3.8%]). The majority of survivors were asymptomatic at last clinical examination (84 of 100 [84%]). CONCLUSIONS: Reoperations for residual LSRs are infrequent but may become necessary late after an ASO, predominantly for neoaortic valve insufficiency and CAPs. Risk at reoperation is not negligible, and DORV TGA-type anatomy, as well as procedures on the coronary arteries, were significantly associated with a higher morbidity and a lower overall survival. Recurrent reoperations after LSRs may be required.

• MeSH
• aortální insuficience epidemiologie   etiologie   chirurgie   MeSH
• dítě MeSH
• dvojvýtoková pravá komora chirurgie   MeSH
• incidence MeSH
• kojenec MeSH
• korekce transpozice velkých arterií škodlivé účinky   MeSH
• lidé MeSH
• míra přežití trendy   MeSH
• mladiství MeSH
• následné studie MeSH
• pooperační komplikace epidemiologie   etiologie   chirurgie   MeSH
• předškolní dítě MeSH
• prognóza MeSH
• reoperace metody   MeSH
• retrospektivní studie MeSH
• rizikové faktory MeSH
• transpozice velkých cév chirurgie   MeSH
• Check Tag
• dítě MeSH
• kojenec MeSH
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• předškolní dítě MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH
• Geografické názvy
• Evropa epidemiologie   MeSH

BACKGROUND: In the era of multi-modality imaging, this study compared contemporary, pre-operative echocardiography and cardiac MRI in predicting the need for intervention on additional lesions before surgical bidirectional cavopulmonary connection. METHODS: A total of 72 patients undergoing bidirectional cavopulmonary connection for single-ventricle palliation between 2007 and 2012, who underwent pre-operative assessment using both echocardiography and MRI, were included. The pre-determined outcome measure was any additional surgical or catheter-based intervention within 6 months of bidirectional cavopulmonary connection. Indices assessed were as follows: indexed dimensions of right and left pulmonary arteries, coarctation of the aorta, adequacy of interatrial communication, and degree of atrioventricular valve regurgitation. RESULTS: Median age at bidirectional cavopulmonary connection was 160 days (interquartile range 121-284). The following MRI parameters predicted intervention: Z score for right pulmonary artery (odds ratio 1.77 (95% confidence interval 1.12-2.79, p=0.014)) and left pulmonary artery dimensions (odds ratio 1.45 (1.04-2.00, p=0.027)) and left pulmonary artery report conclusion (odds ratio 1.57 (1.06-2.33)). The magnetic resonance report predicted aortic arch intervention (odds ratio 11.5 (3.5-37.7, p=0.00006)). The need for atrioventricular valve repair was associated only with magnetic resonance regurgitation fraction score (odds ratio 22.4 (1.7-295.1, p=0.018)). Echocardiography assessment was superior to MRI for predicting intervention on interatrial septum (odds ratio 27.7 (6.3-121.6, p=0.00001)). CONCLUSION: For branch pulmonary arteries, aortic arch, and atrioventricular valve regurgitation, MRI parameters more reliably predict the need for intervention; however, echocardiography more accurately identified the adequacy of interatrial communication. Approaching bidirectional cavopulmonary connection, the diagnostic strengths of MRI and echocardiography should be acknowledged when considering intervention.

• Klíčová slova
• Bidirectional cavopulmonary connection, cardiac magnetic resonance, echocardiography,
• MeSH
• aorta diagnostické zobrazování   MeSH
• arteria pulmonalis diagnostické zobrazování   MeSH
• echokardiografie MeSH
• Fontanova operace * MeSH
• kojenec MeSH
• lidé MeSH
• logistické modely MeSH
• magnetická rezonanční tomografie MeSH
• multivariační analýza MeSH
• retrospektivní studie MeSH
• srdeční komory diagnostické zobrazování   MeSH
• vrozené srdeční vady diagnostické zobrazování   chirurgie   MeSH
• Check Tag
• kojenec MeSH
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• srovnávací studie MeSH
• Geografické názvy
• Česká republika MeSH

BACKGROUND: Permanent junctional reciprocating tachycardia (PJRT) is an uncommon form of supraventricular tachycardia in children. Treatment of this arrhythmia has been considered difficult because of a high medication failure rate and risk of cardiomyopathy. Outcomes in the current era of interventional treatment with catheter ablation have not been published. OBJECTIVE: To describe the presentation and clinical course of PJRT in children. METHODS: This is a retrospective review of 194 pediatric patients with PJRT managed at 11 institutions between January 2000 and December 2010. RESULTS: The median age at diagnosis was 3.2 months, including 110 infants (57%; aged <1 year). PJRT was incessant in 47%. The ratio of RP interval to cycle length was higher with incessant than with nonincessant tachycardia. Tachycardia-induced cardiomyopathy was observed in 18%. Antiarrhythmic medications were used for initial management in 76%, while catheter ablation was used initially in only 10%. Medications achieved complete resolution in 23% with clinical benefit in an additional 47%. Overall, 140 patients underwent 175 catheter ablation procedures with a success rate of 90%. There were complications in 9% with no major complications reported. Patients were followed for a median of 45.1 months. Regardless of treatment modality, normal sinus rhythm was present in 90% at last follow-up. Spontaneous resolution occurred in 12% of the patients. CONCLUSION: PJRT in children is frequently incessant at the time of diagnosis and may be associated with tachycardia-induced cardiomyopathy. Antiarrhythmic medications result in complete control in few patients. Catheter ablation is effective, and serious complications are rare.

• Klíčová slova
• Antiarrhythmic medications ok, Catheter ablation, Pediatrics, Permanent junctional reciprocating tachycardia,
• MeSH
• dítě MeSH
• elektrokardiografie * MeSH
• incidence MeSH
• kojenec MeSH
• lidé MeSH
• mladiství MeSH
• následné studie MeSH
• novorozenec MeSH
• předškolní dítě MeSH
• převodní systém srdeční patofyziologie   MeSH
• reciproční tachykardie epidemiologie   patofyziologie   MeSH
• retrospektivní studie MeSH
• Check Tag
• dítě MeSH
• kojenec MeSH
• lidé MeSH
• mladiství MeSH
• mužské pohlaví MeSH
• novorozenec MeSH
• předškolní dítě MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH
• práce podpořená grantem MeSH
• Geografické názvy
• Kanada epidemiologie   MeSH
• Spojené státy americké epidemiologie   MeSH

OBJECTIVES: We sought to determine the prevalence, morphology, surgical methods and results of surgery for left ventricular outflow tract obstruction (LVOTO) associated with atrioventricular septal defect (AVSD). METHODS: Correction of AVSD was performed in 615 patients. Twenty-three (3.7%) patients with LVOTO were identified. Sixteen (70%) of them had partial and 7 (30%) had complete AVSD. Surgery for AVSD was performed at a median of 0.6 years (mean 2.1 ± 3.0 years), and surgery for LVOTO at a median of 3.4 years (mean 4.7 ± 3.5 years). The point and period prevalence of LVOTO in AVSD were determined. Detailed morphological study, individualized repair of AVSD with LVOTO and long-term follow-up were performed. Early and long-term results were analysed. RESULTS: The point prevalence of LVOTO at the time of AVSD repair was 1.3%. The period prevalence of LVOTO was 3.7% in course of 8.3 ± 6.0 (0-18.4) years and 191.4 patient-years following AVSD repair. Causes of LVOTO were fibromuscular membrane (n = 17), septal hypertrophy (n = 17), abnormal atrioventricular (AV) valve (n = 9), muscular bands (n = 3), fibrous strands (n = 4) and stenotic aortic valve (n = 2). Usually, a combination of several obstructive lesions was present. LVOTO was present at the time of AVSD repair in 8 patients (35%) and developed after repair in 15 (65%) patients. Membrane excision (n = 17), myectomy (n = 17), excision of abnormal AV valvar tissue (n = 8), excision of muscular bands and fibrous strands (n = 6), AV valve replacement (n = 2) and aortic valvotomy (n = 2) were required. There was 1 (4%) early and 1 (4%) late death. Six (29%) survivors required reoperation for recurrence of LVOTO at an average interval of 6.3 ± 3.2 years after surgery. The actuarial survival at 1 and 10 years was 96 and 88%, respectively. The actuarial freedom from reoperation for LVOTO was 80, 40 and 20% at 6, 10 and 15 years after surgery, respectively. Eighteen (78%) patients remain in good condition at mean 6.0 ± 5.5 years after surgery. CONCLUSIONS: The point prevalence of LVOTO at the time of AVSD repair was 1.3%, and period prevalence 3.7%. Fibromuscular membrane, septal hypertrophy and valvar attachments represent the most common causes of LVOTO. Usually, more structures are involved. The repair must be individualized. The presence of LVOTO increases the need for reoperation.

• Klíčová slova
• Atrioventricular septal defect, Congenital heart disease, Left ventricular outflow tract obstruction, Long-term results, Reoperations, Surgical treatment,
• MeSH
• časové faktory MeSH
• defekty srdečního septa diagnóza   mortalita   chirurgie   MeSH
• dítě MeSH
• kardiochirurgické výkony * škodlivé účinky   mortalita   MeSH
• kojenec MeSH
• lidé MeSH
• novorozenec MeSH
• obstrukce výtoku ze srdeční komory diagnóza   mortalita   chirurgie   MeSH
• předškolní dítě MeSH
• prevalence MeSH
• reoperace MeSH
• retrospektivní studie MeSH
• rizikové faktory MeSH
• výsledek terapie MeSH
• Check Tag
• dítě MeSH
• kojenec MeSH
• lidé MeSH
• mužské pohlaví MeSH
• novorozenec MeSH
• předškolní dítě MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• Geografické názvy
• Česká republika epidemiologie   MeSH

PURPOSE OF REVIEW: To review and prioritize data on pediatric cardiac pacing published during the period of the last 18 months. RECENT FINDINGS: New approaches to preservation of ventricular function in pediatric pacing are based on recent publications confirming major influence of the ventricular pacing site on left ventricular (LV) function and synchrony. Current studies on epicardial vs. transvenous pacing continue to show survival superiority of endocardial leads. Long-term outcome of epicardial pacing may, however, be positively influenced by technical refinements. Recent amendments of the guidelines for cardiac resynchronization therapy (CRT) in adult idiopathic and ischemic cardiomyopathy are likely to influence CRT indications in children. Novel data give interesting insights into implantable cardioverter-defibrillator (ICD) lead survival as well as the use of ICDs in young patients with hypertrophic cardiomyopathy. SUMMARY: Pediatric cardiac pacing and ICD therapy is still a developing field likely to improve with technical refinements, proper lead placement and more specific therapy indications. The current review will give the reader information about recent developments and directions for the future.

• MeSH
• defibrilátory implantabilní MeSH
• dítě MeSH
• funkce levé komory srdeční MeSH
• kardiomyopatie komplikace   MeSH
• kardiostimulace umělá metody   MeSH
• kardiostimulátor * MeSH
• lidé MeSH
• směrnice pro lékařskou praxi jako téma MeSH
• srdeční arytmie etiologie   terapie   MeSH
• srdeční resynchronizační terapie metody   MeSH
• srdeční selhání etiologie   terapie   MeSH
• vrozené srdeční vady komplikace   MeSH
• výsledek terapie MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• přehledy MeSH