BACKGROUND: Cardiac resynchronization therapy (CRT) is a guideline-recommended therapy in patients with heart failure with mildly reduced ejection fraction (HFmrEF, 36%-50%) and left bundle branch block or indication for ventricular pacing. Conduction system pacing (CSP) using left bundle branch area pacing or His bundle pacing has been shown to be a safe and physiologic alternative to biventricular pacing (BVP). OBJECTIVE: The aim of this study was to compare the clinical outcomes between BVP and CSP for patients with HFmrEF undergoing CRT. METHODS: Consecutive patients who underwent BVP or CSP with HFmrEF between January 2018 and June 2023 at 16 international centers were included. The primary outcome was the composite end point of time to death or heart failure hospitalization (HFH). Secondary end points included change in left ventricular ejection fraction (LVEF) and individual end points of death and HFH. RESULTS: A total of 1004 patients met inclusion criteria: BVP, 178; CSP, 826 (His bundle pacing, 154; left bundle branch area pacing, 672). Mean age was 73 ± 13 years; female, 34%; and LVEF, 42% ± 5%. Paced QRS duration in CSP was significantly narrower compared with BVP (129 ± 21 ms vs 144 ± 19 ms; P < .001). LVEF improved during follow-up in both groups (49% ± 10% vs 48% ± 10%; P = .32). CSP was independently associated with significant reduction in the primary end point of time to death or HFH compared with BVP (22% vs 34%; hazard ratio, 0.64; 95% confidence interval, 0.43-0.94; P = .025). CONCLUSION: CSP was associated with improved clinical outcomes compared with BVP in this large cohort of patients with HFmrEF undergoing CRT. Randomized controlled trials comparing CSP with BVP will be necessary to confirm these results.

• Klíčová slova
• Biventricular pacing, Cardiac resynchronization therapy, Conduction system pacing, HFmrEF, Heart failure hospitalization, His bundle pacing, Left bundle branch area pacing, Mortality,
• MeSH
• blokáda Tawarova raménka terapie   patofyziologie   MeSH
• funkce levé komory srdeční * fyziologie   MeSH
• Hisův svazek patofyziologie   MeSH
• lidé MeSH
• následné studie MeSH
• převodní systém srdeční * patofyziologie   MeSH
• retrospektivní studie MeSH
• senioři MeSH
• srdeční resynchronizační terapie * metody   MeSH
• srdeční selhání * terapie   patofyziologie   MeSH
• tepový objem * fyziologie   MeSH
• výsledek terapie MeSH
• Check Tag
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH
• srovnávací studie MeSH

AIMS: Cardiac resynchronization therapy (CRT) is guideline recommended for the treatment of symptomatic heart failure (HF) with reduced left ventricular ejection fraction and prolonged QRS. However, patients with common comorbidities, such as persistent/permanent atrial fibrillation (AF), are often under-represented in clinical trials. METHODS: The Strategic Management to Optimize Response to Cardiac Resynchronization Therapy (SMART) registry (NCT03075215) was a global, multicentre, registry that enrolled de novo CRT implants, or upgrade from pacemaker or implantable cardioverter defibrillator to CRT-defibrillator (CRT-D), using a quadripolar left ventricular lead in real-world clinical practice. The primary endpoint was CRT response between baseline and 12 month follow-up defined as a clinical composite score (CCS) consisting of all-cause mortality, HF-associated hospitalization, New York Heart Association (NYHA) class and quality of life global assessment. RESULTS: The registry enrolled 2035 patients, of which 1558 had completed CCS outcomes at 12 months. The patient cohort was 33.0% female, mean age at enrolment was 67.5 ± 10.4 years and the mean left ventricular ejection fraction was 29.6 ± 7.9%. Notably, there was a high prevalence of mildly symptomatic patients (NYHA class I/II 51.3%), non-left bundle branch block (LBBB) morphology (38.0%), AF (37.2%) and diabetes mellitus (34.7%) at baseline. CCS at 12 months improved in 58.9% (n = 917) of patients; 20.1% (n = 313) of patients stabilized and 21.0% (n = 328) worsened. Several patient characteristics were associated with a lower likelihood of response to CRT including older age, ischaemic aetiology, renal dysfunction, AF, non-LBBB morphology and diabetes. Higher HF hospitalization (P < 0.001) and all-cause mortality (P < 0.001) were observed in patients with AF. These patients also had lower percentages of ventricular pacing than patients in sinus rhythm at baseline and follow-up (P < 0.001, both). A further association between AF and non-LBBB was observed with 81.4% of AF non-LBBB patients experiencing an HF hospitalization compared with 92.5% of non-AF LBBB patients (P < 0.001). Mortality between subgroups was also statistically significant (P = 0.019). CONCLUSIONS: This large, global registry enrolled a CRT-D population with higher incidence of comorbidities that have been historically underrepresented in clinical trials and provides new insight into factors influencing response to CRT. As defined by CCS, 58.9% of patients improved and 20.1% stabilized. Patients with AF had particularly worse clinical outcomes, higher HF hospitalization and mortality rates and lower percentages of ventricular pacing. High incidence of HF hospitalization in patients with AF and non-LBBB in this real-world cohort suggests that ablation may play an important role in increasing future CRT response rates.

• Klíčová slova
• atrioventricular optimization, cardiac resynchronization therapy, electrical delay, heart failure,
• MeSH
• celosvětové zdraví MeSH
• funkce levé komory srdeční * fyziologie   MeSH
• kvalita života * MeSH
• lidé středního věku MeSH
• lidé MeSH
• následné studie MeSH
• registrace * MeSH
• senioři MeSH
• srdeční resynchronizační terapie * metody   MeSH
• srdeční selhání * terapie   patofyziologie   mortalita   MeSH
• tepový objem * fyziologie   MeSH
• výsledek terapie MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH

Since cell dying in heart failure (HF) may vary based on the aetiology, we examined the main forms of regulated necrosis, such as necroptosis and pyroptosis, in the hearts damaged due to myocardial infarction (MI) or pressure overload. We also investigated the effects of a drug inhibiting RIP3, a proposed convergent point for both these necrosis-like cell death modes. In rat hearts, left ventricular function, remodelling, pro-cell death, and pro-inflammatory events were investigated, and the pharmacodynamic action of RIP3 inhibitor (GSK'872) was assessed. Regardless of the HF aetiology, the heart cells were dying due to necroptosis, albeit the upstream signals may be different. Pyroptosis was observed only in post-MI HF. The dysregulated miRNAs in post-MI hearts were accompanied by higher levels of a predicted target, HMGB1, its receptors (TLRs), as well as the exacerbation of inflammation likely originating from macrophages. The RIP3 inhibitor suppressed necroptosis, unlike pyroptosis, normalised the dysregulated miRNAs and tended to decrease collagen content and affect macrophage infiltration without affecting cardiac function or structure. The drug also mitigated the local heart inflammation and normalised the higher circulating HMGB1 in rats with post-MI HF. Elevated serum levels of HMGB1 were also detected in HF patients and positively correlated with C-reactive protein, highlighting pro-inflammatory axis. In conclusion, in MI-, but not pressure overload-induced HF, both necroptosis and pyroptosis operate and might underlie HF pathogenesis. The RIP3-targeting pharmacological intervention might protect the heart by preventing pro-death and pro-inflammatory mechanisms, however, additional strategies targeting multiple pro-death pathways may exhibit greater cardioprotection.

• Klíčová slova
• Heart failure, High mobility group box 1, Inflammation, Necroptosis, Pyroptosis, Receptor-interacting protein kinase 3,
• MeSH
• funkce levé komory srdeční účinky léků   MeSH
• inhibitory proteinkinas * farmakologie   MeSH
• kardiomyocyty * účinky léků   patologie   enzymologie   MeSH
• krysa rodu Rattus MeSH
• mikro RNA metabolismus   genetika   MeSH
• modely nemocí na zvířatech MeSH
• nekroptóza * účinky léků   MeSH
• nekróza MeSH
• potkani Sprague-Dawley MeSH
• pyroptóza * účinky léků   MeSH
• remodelace komor účinky léků   MeSH
• serin-threoninkinasy interagující s receptory * antagonisté a inhibitory   metabolismus   MeSH
• srdeční selhání * patologie   enzymologie   patofyziologie   farmakoterapie   etiologie   genetika   MeSH
• zvířata MeSH
• Check Tag
• krysa rodu Rattus MeSH
• mužské pohlaví MeSH
• zvířata MeSH
• Publikační typ
• časopisecké články MeSH
• Názvy látek
• inhibitory proteinkinas * MeSH
• mikro RNA MeSH
• Ripk3 protein, rat MeSH Prohlížeč
• serin-threoninkinasy interagující s receptory * MeSH

Malignant lymphoma survivors are at increased risk for anthracycline and/or radiotherapy-induced chronic cardiotoxicity. Proper long-term follow-up is essential for malignant lymphoma survivors after-care. This study aimed to assess TTE parameters of potential subclinical cardiotoxicity and to examine their utility in diagnosing chronic cardiotoxicity. Improvement of the diagnostic process may precede the manifestation of cardiac adverse events. Main objective of the study was to improve the identification of cancer survivors in increased risk of treatment cardiotoxicity. To achieve this goal, utility of various echocardiography parameters was examined.In this retrospective study we analysed TTE of 167 subjects with speckle tracking according to the European Society of Echocardiography guidelines during the follow-up period. 88 of them were long-term lymphoma survivors diagnosed with malignant lymphoma between the years 1994-2015. Minimum follow up period was 5 years with the median of 10 years after anti-cancer treatment cessation. TTE were performed between the years 2017-2022 at cardio-oncology outpatient office during regular follow-up period. A total of 79 volunteers with no history of chronic heart failure (CHF) or decline in LVEF, 51 (64.6%) of whom were males, with the median age of 46 (16-58) years were included in the analysis as control group. Control subjects had various indications for TTE (e.g. preoperative examination, benign palpitations, or with well controlled arterial hypertension taking two antihypertensives at most). Ischemic heart disease was ruled out by stress test. None of the control subjects had history of stroke or chronic lower limb ischemia. All control subjects were considered clinically stable with no sign of cardiac impairment caused by primary disease. Both cancer survivors and control group were divided into subgroups based on LVEF: lower normal LVEF (53-61%), and higher normal LVEF (> 61%). Survivors with lower normal LVEF (53-61%) had a statistically significant decline in GLS compared to those with higher normal LVEF (> 61%). This phenomenon was not observed in control group indicating a possible additional diagnostic value of this parameter. Inclusion of GLS assessment in follow-up TTE examination of subjects with lower normal LVEF may improve the sensitivity of detection of chronic cardiotoxicity. Patients with declined GLS and lower normal LVEF are candidates for intensified follow-up to precede manifestation of cardiac adverse events.

• Klíčová slova
• Anthracyclines, Cardiotoxicity, Echocardiography, Global longitudinal strain, Radiotherapy,
• MeSH
• antracykliny škodlivé účinky   MeSH
• dospělí MeSH
• echokardiografie metody   MeSH
• funkce levé komory srdeční * MeSH
• globální longitudinální strain MeSH
• kardiotoxicita * etiologie   diagnóza   diagnostické zobrazování   MeSH
• lidé středního věku MeSH
• lidé MeSH
• lymfom * farmakoterapie   komplikace   MeSH
• mladiství MeSH
• mladý dospělý MeSH
• následné studie MeSH
• přežívající onkologičtí pacienti * MeSH
• retrospektivní studie MeSH
• tepový objem * MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladiství MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Názvy látek
• antracykliny MeSH

INTRODUCTION: A variable proportion of non-responders to cardiac resynchronization therapy (CRT) warrants the search for new approaches to optimize the position of the left ventricular (LV) lead and the CRT device programming. CineECG is a novel ECG modality proposed for the spatial visualization and quantification of myocardial depolarization and repolarization sequences. OBJECTIVE: The present study aimed to evaluate CineECG-derived parameters in different pacing modes and to test their associations with acute hemodynamic responses in CRT patients. METHODS AND RESULTS: CineECG was used to construct the average electrical path within the cardiac anatomy from the 12-lead ECG. CineECG and LV dP/dt max were tested in 15 patients with nonischemic dilated cardiomyopathy and left bundle branch block (QRS: 170 ± 17 ms; LVEF: 26 ± 5.5%) under pacing protocols with different LV lead localizations. The CineECG-derived path directions were computed for the QRS and ST-T intervals for the anteroposterior (Xh), interventricular (Yh), and apicobasal (Zh) axes. In a multivariate linear regression analysis with adjustment for the pacing protocol type, the ST-T path direction Yh was independently associated with the increase in dP/dt max during CRT, [regression coefficient 639.4 (95% confidence interval: 187.9-1090.9), p = 0.006]. In ROC curve analysis, the ST-T path direction Yh was associated with the achievement of a 10% increase in dP/dt max (AUC: 0.779, p = 0.002) with the optimal cut-off > 0.084 (left-to-right direction) with sensitivity 0.67 and specificity 0.92. CONCLUSION: The acute hemodynamic response in CRT patients was associated with specific CineECG repolarization sequence parameters, warranting their further testing as potential predictors of clinical outcomes.

• Klíčová slova
• cardiac resynchronization therapy, heart failure, hemodynamics, multipoint pacing, multisite pacing, repolarization,
• MeSH
• akční potenciály * MeSH
• blokáda Tawarova raménka * patofyziologie   terapie   diagnóza   MeSH
• časové faktory MeSH
• dilatační kardiomyopatie * patofyziologie   terapie   diagnóza   MeSH
• elektrokardiografie * MeSH
• funkce levé komory srdeční * MeSH
• hemodynamika * MeSH
• lidé středního věku MeSH
• lidé MeSH
• prediktivní hodnota testů MeSH
• senioři MeSH
• srdeční frekvence MeSH
• srdeční resynchronizační terapie * MeSH
• výsledek terapie MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

INTRODUCTION: Atrial fibrillation (AF) can cause or aggravate heart failure (HF). Catheter ablation (CA) is an effective treatment for AF. This study focused on the feasibility and outcomes of emergent AF ablation performed during hospitalization for acute HF. METHODS AND RESULTS: We retrospectively investigated patients who underwent emergent CA for AF during hospitalization for acute HF in 2018-2024. Arrhythmia recurrence was the primary endpoint. The combination of arrhythmia recurrence, HF hospitalization, and all-cause death was the secondary endpoint. Patients were censored 1 year after the index procedure. We included 46 patients, 35% females, with median age of 67 [interquartile rage: 61, 72] years and left ventricular ejection fraction (LVEF) of 25 [23, 28]%. Thermal CA was performed in 14 patients, and pulsed field ablation (PFA) in 32 patients. Procedure time was significantly shorter with PFA compared to thermal CA (77 [57, 91] vs. 166 [142, 200] minutes, p < 0.001). Fluoroscopy time was longer with PFA (9.5 [7.6, 12.0] vs. 3.9 [2.9, 6.0] minutes, p < 0.001), with a borderline trend towards higher radiation dose (75 [53, 170] vs. 50 [30, 94] μGy.m2, p = 0.056). Extrapulmonary ablation was frequent (86% and 84% for thermal CA and PFA, p > 0.9). The estimated freedom from the primary endpoint was 79% after PFA and 64% after thermal CA (p = 0.44). The estimated freedom from the secondary endpoint was 76% after PFA and 57% after thermal CA (p = 0.43). LVEF improved by 24% ± 2% (p < 0.001) in patients with the first manifestation of HF and by 14% ± 4% (p = .004) in patients with decompensated HF diagnosed earlier. CONCLUSIONS: Emergent CA of AF during acute HF hospitalization is safe and associated with improved LVEF and good clinical outcomes. In the PFA era, the rate of these procedures is progressively increasing as they are readily available and easy to perform compared to thermal ablation.

• Klíčová slova
• acute heart failure, atrial fibrillation, catheter ablation, electroporation, posterior wall isolation, pulsed field ablation, thermal ablation,
• MeSH
• akční potenciály MeSH
• akutní nemoc MeSH
• časové faktory MeSH
• fibrilace síní * chirurgie   patofyziologie   mortalita   diagnóza   komplikace   MeSH
• funkce levé komory srdeční MeSH
• hospitalizace * MeSH
• katetrizační ablace * škodlivé účinky   mortalita   metody   MeSH
• lidé středního věku MeSH
• lidé MeSH
• obnova funkce MeSH
• příjem pacientů * MeSH
• recidiva MeSH
• retrospektivní studie MeSH
• rizikové faktory MeSH
• senioři MeSH
• srdeční frekvence MeSH
• srdeční selhání * patofyziologie   mortalita   diagnóza   terapie   MeSH
• studie proveditelnosti MeSH
• tepový objem MeSH
• výsledek terapie MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

INTRODUCTION: Left bundle branch area pacing (LBBAP) comprises pacing at the left ventricular septum (LVSP) or left bundle branch (LBBP). The aim of the present study was to investigate the differences in ventricular electrical heterogeneity between LVSP, LBBP, right ventricular pacing (RVP) and intrinsic conduction with different dyssynchrony measures using the ECG, vectorcardiograpy, ECG belt, and Ultrahigh frequency (UHF-)ECG. METHODS: Thirty-seven patients with a pacemaker indication for bradycardia or cardiac resynchronization therapy underwent LBBAP implantation. ECG, vectorcardiogram, ECG belt and UHF-ECG signals were recorded during RVP, LVSP and LBBP, and intrinsic activation. QRS duration (QRSd) was measured from the ECG, QRS area was calculated from the vectorcardiogram, LV activation time (LVAT) and standard deviation of activation time (SDAT) from ECG belt and electrical dyssynchrony (e-DYS16) from UHF-ECG. RESULTS: Both LVSP and LBBP significantly reduced ventricular electrical heterogeneity as compared to underlying LBBB and RV pacing in terms of QRS area (p < .001), SDAT (p < .001), LVAT (p < .001) and e-DYS16 (p < .001). QRSd was only reduced as compared to RV pacing(p < .001). QRS area was similar during LBBP and normal intrinsic conduction, e-DYS16 was similar during LVSP and normal intrinsic conduction, whereas SDAT was similar for LVSP, LBBP and normal intrinsic conduction. For all these variables there was no significant difference between LVSP and LBBP. CONCLUSION: Both LVSP and LBBP resulted in a more synchronous LV activation than LBBB and RVP. Especially LBBP resulted in levels of LV synchrony comparable to normal intrinsic conduction.

• Klíčová slova
• bradycardia pacing, cardiac resynchronization therapy, conduction system pacing, dyssynchrony, left bundle branch area pacing,
• MeSH
• akční potenciály * MeSH
• blokáda Tawarova raménka patofyziologie   terapie   diagnóza   MeSH
• bradykardie patofyziologie   terapie   diagnóza   MeSH
• časové faktory MeSH
• elektrofyziologické techniky kardiologické MeSH
• elektrokardiografie MeSH
• funkce levé komory srdeční * MeSH
• Hisův svazek * patofyziologie   MeSH
• kardiostimulace umělá * MeSH
• lidé středního věku MeSH
• lidé MeSH
• mezikomorová přepážka * patofyziologie   MeSH
• prediktivní hodnota testů * MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• srdeční frekvence * MeSH
• srdeční resynchronizační terapie MeSH
• vektorkardiografie * metody   MeSH
• výsledek terapie MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři nad 80 let MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• srovnávací studie MeSH

AIMS: Recent-onset dilated cardiomyopathy (RODCM) is characterized by heterogeneous aetiology and diverse clinical outcomes, with scarce data on genotype-phenotype correlates. Our aim was to correlate individual RODCM genotypes with left ventricular reverse remodelling (LVRR) and clinical outcomes. METHODS AND RESULTS: In this prospective study, a total of 386 Czech RODCM patients with symptom duration ≤6 months underwent genetic counselling and whole-exome sequencing (WES). The presence of pathogenic (class 5) or likely pathogenic (class 4) variants in a set of 72 cardiomyopathy-related genes was correlated with the occurrence of all-cause death, heart transplantation, or implantation of a ventricular assist device (primary outcome) and/or ventricular arrhythmia event (secondary outcome). LVRR was defined as an improvement of left ventricular ejection fraction to >50% or ≥10% absolute increase, with a left ventricular end-diastolic diameter ≤33 mm/m2 or ≥10% relative decrease. Median follow-up was 41 months. RODCM was familial in 98 (25%) individuals. Class 4-5 variants of interest (VOIs) were identified in 125 (32%) cases, with 69 (18%) having a single titin-truncating variant (TTNtv) and 56 (14%) having non-titin (non-TTN) VOIs. The presence of class 4-5 non-TTN VOIs, but not of TTNtv, heralded a lower probability of 12-month LVRR and proved to be an independent baseline predictor both of the primary and the secondary outcome. The negative result of genetic testing was a strong protective baseline variable against occurrence of life-threatening ventricular arrhythmias. Detection of class 4-5 VOIs in genes coding nuclear envelope proteins was another independent predictor of both study outcomes at baseline and also of life-threatening ventricular arrhythmias after 12 months. Class 4-5 VOIs of genes coding cytoskeleton were associated with an increased risk of life-threatening ventricular arrhythmias after baseline assessment. A positive family history of dilated cardiomyopathy alone only related to a lower probability of LVRR at 12 months and at the final follow-up. CONCLUSIONS: RODCM patients harbouring class 4-5 non-TTN VOIs are at higher risk of progressive heart failure and life-threatening ventricular arrhythmias. Genotyping may improve their early risk stratification at baseline assessment.

• Klíčová slova
• Genetics, Left ventricular reverse remodelling, Prognosis, Recent‐onset dilated cardiomyopathy, Whole‐exome sequencing,
• MeSH
• dilatační kardiomyopatie * genetika   patofyziologie   MeSH
• dospělí MeSH
• funkce levé komory srdeční fyziologie   MeSH
• genotyp * MeSH
• lidé středního věku MeSH
• lidé MeSH
• následné studie MeSH
• prospektivní studie MeSH
• remodelace komor * genetika   fyziologie   MeSH
• sekvenování exomu MeSH
• tepový objem fyziologie   MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Česká republika epidemiologie   MeSH

• MeSH
• blokáda Tawarova raménka etiologie   terapie   MeSH
• elektrokardiografie MeSH
• funkce levé komory srdeční MeSH
• Hisův svazek * MeSH
• kardiomyopatie * etiologie   terapie   MeSH
• kardiostimulace umělá škodlivé účinky   MeSH
• lidé MeSH
• srdeční komory MeSH
• výsledek terapie MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH

BACKGROUND: Although genetic variants in MYH7 are the most frequent cause of pediatric genetic dilated cardiomyopathy (DCM), there are no studies available describing this entity. We sought to describe clinical features, analyze variant location, and explore predictors of bad prognosis in pediatric MYH7-related DCM. METHODS AND RESULTS: We evaluated clinical records from 44 patients (24 men; median age at diagnosis, 0.54 [interquartile range, 0.01-10.8] years) with pathogenic/likely pathogenic variants in MYH7 diagnosed with DCM at pediatric age (<18 years) followed at 13 international centers. We also explored risk factors associated with a composite end point of end-stage heart failure defined as heart transplantation or heart failure-related death. Twenty-two patients (50%) were diagnosed at age <6 months, including 7 (16%) at birth. Left ventricular (LV) hypertrabeculation features were present in 15 (38%), particularly among patients with genetic variants in the head domain. After a median follow-up of 6.1 years (interquartile range, 1.9-13.4), 15 patients (36%) required a heart transplant (n=14) or died due to end-stage heart failure (n=1), 15 patients (36%) persisted with systolic dysfunction despite treatment, 12 (29%) had a significant increase in LV ejection fraction, and 2 were lost to follow-up. Overall, end-stage heart failure event rate was 25% at 5 years. New York Heart Association class III to IV (hazard ratio [HR], 7.67 [95% CI, 2.16-27.2]; P=0.002) and LV ejection fraction ≤35% (HR, 4.00 [95% CI, 1.11-14.4]; P=0.03) were the best predictors of bad prognosis. CONCLUSIONS: Pediatric MYH7-related DCM is characterized by early onset, frequent LV hypertrabeculation, and poor prognosis. Advanced New York Heart Association class and low LV ejection fraction emerged as predictors of end-stage heart failure.

• Klíčová slova
• MYH7, dilated cardiomyopathy, genetics, pediatric,
• MeSH
• dilatační kardiomyopatie * genetika   patofyziologie   diagnóza   MeSH
• dítě MeSH
• fenotyp MeSH
• funkce levé komory srdeční MeSH
• genetická predispozice k nemoci MeSH
• kojenec MeSH
• lidé MeSH
• mladiství MeSH
• mutace MeSH
• novorozenec MeSH
• předškolní dítě MeSH
• prognóza MeSH
• retrospektivní studie MeSH
• rizikové faktory MeSH
• srdeční myosiny * genetika   MeSH
• srdeční selhání genetika   patofyziologie   diagnóza   MeSH
• těžké řetězce myosinu * genetika   MeSH
• transplantace srdce * 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
• Názvy látek
• MYH7 protein, human MeSH Prohlížeč
• srdeční myosiny * MeSH
• těžké řetězce myosinu * MeSH