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
• prostředky srdeční resynchronizační terapie MeSH
• senioři MeSH
• srdeční frekvence MeSH
• srdeční resynchronizační terapie * MeSH
• srdeční selhání patofyziologie   terapie   diagnóza   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

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

The causes of nonischemic dilated cardiomyopathy are classified as genetic or nongenetic, but environmental factors such as metal pollutants may interact with genetic susceptibility. The presence of metal particles has been detected in the myocardium, including in those patients with dilated cardiomyopathy. It is also known that hypersensitivity reactions can induce inflammation in tissue. The present study aimed to verify if metal-induced delayed-type hypersensitivity is present in patients with nonischemic dilated cardiomyopathy. The patient group consisted of 30 patients with newly diagnosed dilated cardiomyopathy; the control group comprised 41 healthy subjects. All patients and control subjects provided blood samples for lymphocyte transformation testing (MELISA®) to assess possible hypersensitivity to seven common metals. Specific exposure to metals was based on interview data. Results showed that exposure to cadmium and lead (p = 0.0002), aluminum (p = 0.0006), nickel (p = 0.0012), and chromium (p = 0.0065) was more often reported by patients than controls. The patients also had significantly more frequent hypersensitivity reactions to mercury (26.7% vs. 7.3%, p = 0.014624), nickel (40% vs. 12.2%, p = 0.02341), and silver (20% vs. 4.8%, p = 0.025468) than the control group. Patients with dilated cardiomyopathy had greater exposure to certain metals compared with healthy controls. Hypersensitivity to metals was more frequent in patients with dilated cardiomyopathy, suggesting a possible association that warrants further investigation.

• Klíčová slova
• Delayed-type hypersensitivity, Dilated cardiomyopathy, Inflammation, Lymphocyte transformation test, Metal exposure,
• MeSH
• aktivace lymfocytů MeSH
• biopsie MeSH
• dilatační kardiomyopatie komplikace   diagnóza   patofyziologie   MeSH
• dospělí MeSH
• hodnocení rizik MeSH
• kovy škodlivé účinky   MeSH
• kultivované buňky MeSH
• lidé středního věku MeSH
• lidé MeSH
• pilotní projekty MeSH
• pozdní přecitlivělost diagnóza   etiologie   imunologie   MeSH
• rizikové faktory MeSH
• studie případů a kontrol MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• Názvy látek
• kovy MeSH

BACKGROUND: Elevated levels of the extracellular matrix glycoprotein osteopontin (OPN) may be detected in both myocardium and plasma under various pathological conditions affecting the heart. Several studies demonstrated increased plasma OPN levels in patients with heart failure due to dilated cardiomyopathy (DCM), while other studies showed high OPN expression levels in the myocardium of such patients. However, very little is known about OPN levels in both plasma and myocardium of the same individual with DCM. Therefore, we aimed to compare plasma OPN levels and levels of myocardial OPN expression in patients with recent-onset DCM (Ro-DCM). METHODS: We examined plasma OPN as well as creatinine, C‑reactive protein (CRP), brain natriuretic peptide (BNP), and troponin I levels in 25 patients with Ro-DCM. Furthermore, all subjects underwent transthoracic echocardiography, selective coronary angiography, and endomyocardial biopsy (EMB) for the assessment of myocardial OPN expression. RESULTS: No significant correlation between myocardial OPN expression and clinical, biochemical, or echocardiographic parameters was found. In log transformation analysis, plasma OPN levels correlated significantly with BNP levels (r = 0.46, p = 0.031), with CRP levels (r = 0.52, p = 0.015), and with early diastolic mitral annular velocity (r = -0.57, p = 0.009). There was a borderline association between the plasma OPN log value and New York Heart Association class (p = 0.053). CONCLUSION: Plasma OPN levels reflect heart failure severity in patients with Ro-DCM. Myocardial OPN expression is not associated with either plasma OPN levels or markers of heart failure in these individuals.

• Klíčová slova
• Biological markers, Cardiac failure, Cardiomyopathies, Extracellular matrix, Myocardium,
• MeSH
• dilatační kardiomyopatie * diagnóza   MeSH
• krevní plazma MeSH
• lidé MeSH
• myokard MeSH
• osteopontin MeSH
• srdeční selhání * diagnóza   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• Názvy látek
• osteopontin MeSH

Hypocalcaemic cardiomyopathy is a rare form of dilated cardiomyopathy. The authors here present two cases in which symptomatic dilated cardiomyopathy was the result of severe hypocalcaemia. First, we report about a 26-year-old woman with primary hypoparathyroidism and then about a 74-year-old man with secondary hypoparathyroidism following a thyroidectomy. In both cases, the left ventricular systolic function improved after calcium supplementation. In the first case, a lack of compliance led to a repeated decrease of both serum calcium level and left ventricular systolic function. The authors also present a comprehensive summary of all cases of hypocalcaemic dilated cardiomyopathy that have been described in literature to date. The mean age of the affected patients was 48.3 years, of which 62% were female patients. The most common causes of hypocalcaemic cardiomyopathy are primary hypoparathyroidism (50%) and post-thyroidectomy hypoparathyroidism (26%). In the post-thyroidectomy subgroup, the median time for the development of hypocalcaemic cardiomyopathy is 10 years (range: 1.5 months to 36 years). Hypocalcaemic cardiomyopathy leads to heart failure with reduced ejection fraction in 87% of patients. Generally, the most common complications of hypoparathyroidism and/or hypocalcaemia are cerebral calcifications, cognitive deficit, and cataracts. Once calcium supplementation is administered, the disease has a good prognosis and, in most individuals, a significant improvement (21%) or even normalization (74%) of the left ventricular systolic function occurs.

• Klíčová slova
• Calcium, Dilated cardiomyopathy, Heart failure, Hypocalcaemia, Hypoparathyroidism, Parathormone,
• MeSH
• dilatační kardiomyopatie * diagnóza   etiologie   MeSH
• dospělí MeSH
• hypokalcemie * diagnóza   etiologie   MeSH
• hypoparatyreóza * komplikace   diagnóza   MeSH
• lidé středního věku MeSH
• lidé MeSH
• senioři MeSH
• tyreoidektomie MeSH
• vápník MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• přehledy MeSH
• Názvy látek
• vápník MeSH

His bundle pacing is a relatively new method of cardiac pacing. This method is used in patients with atrioventricular block to prevent heart failure associated with right ventricular pacing, and in patients with bundle branch block and cardiomyopathy. We report a patient with cardiomyopathy and left bundle branch block with failure of cardiac resynchronization therapy. Permanent His bundle pacing was associated with clinical improvement and improvement of parameters of cardiac function.

• Klíčová slova
• His bundle pacing, cardiac resynchronization therapy, cardiomyopathy, heart failure, left bundle branch block, left ventricular ejection fraction,
• MeSH
• blokáda Tawarova raménka diagnóza   etiologie   patofyziologie   chirurgie   MeSH
• dilatační kardiomyopatie komplikace   diagnóza   patofyziologie   chirurgie   MeSH
• elektrokardiografie MeSH
• funkce levé komory srdeční fyziologie   MeSH
• Hisův svazek fyziologie   MeSH
• implantované elektrody MeSH
• kardiostimulace umělá metody   MeSH
• kardiostimulátor MeSH
• lidé MeSH
• senioři MeSH
• srdeční katetrizace přístrojové vybavení   metody   MeSH
• srdeční resynchronizační terapie škodlivé účinky   MeSH
• výsledek terapie MeSH
• Check Tag
• lidé MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

Distinct cellular level of the Ca2+-binding chaperone calreticulin (CRT) is essential for correct embryonal cardiac development and postnatal function. However, CRT is also a potential autoantigen eliciting formation of antibodies (Ab), whose role is not yet clarified. Immunization with CRT leads to cardiac injury, while overexpression of CRT in cardiomyocytes induces dilated cardiomyopathy (DCM) in animals. Hence, we analysed levels of anti-CRT Ab and calreticulin in the sera of patients with idiopatic DCM and hypertrophic cardiomyopathy (HCM). ELISA and immunoblot using human recombinant CRT and Pepscan with synthetic, overlapping decapeptides of CRT were used to detect anti-CRT Ab. Serum CRT concentration was tested by ELISA. Significantly increased levels of anti-CRT Ab of isotypes IgA (p < 0.001) and IgG (p < 0.05) were found in patients with both DCM (12/34 seropositive for IgA, 7/34 for IgG) and HCM (13/38 seropositive for IgA, 11/38 for IgG) against healthy controls (2/79 for IgA, 1/79 for IgG). Titration analysis in seropositive DCM and HCM patients documented anti-CRT Ab detected at 1/1600 dilution for IgG and 1/800 for IgA (and IgA1) and at least at 1/200 dilution for IgA2, IgG1, IgG2 and IgG3. Pepscan identified immunogenic CRT epitopes recognized by IgA and IgG Ab of these patients. Significantly increased levels of CRT relative to healthy controls were found in sera of patients with HCM (p < 0.01, 5/19). These data extend the knowledge of seroprevalence of anti-CRT Ab and CRT, and suggest possible involvement of autoimmune mechanisms directed to CRT in some forms of cardiomyopathies, which are clinically heterogeneous.

• Klíčová slova
• Anti-calreticulin antibodies, autoimmunity, calreticulin, dilated cardiomyopathy, hypertrophic cardiomyopathy,
• MeSH
• autoantigeny krev   imunologie   MeSH
• autoimunita MeSH
• autoprotilátky krev   imunologie   MeSH
• biologické markery MeSH
• dilatační kardiomyopatie krev   diagnóza   imunologie   MeSH
• dospělí MeSH
• ELISA MeSH
• hypertrofická kardiomyopatie krev   diagnóza   imunologie   MeSH
• imunoglobulin A krev   imunologie   MeSH
• imunoglobulin G krev   imunologie   MeSH
• kalretikulin krev   imunologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladý dospělý MeSH
• senioři MeSH
• studie případů a kontrol MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mladý dospělý MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• Názvy látek
• autoantigeny MeSH
• autoprotilátky MeSH
• biologické markery MeSH
• CALR protein, human MeSH Prohlížeč
• imunoglobulin A MeSH
• imunoglobulin G MeSH
• kalretikulin MeSH

In this paper the Working Group on Myocardial and Pericardial Disease proposes a revised definition of dilated cardiomyopathy (DCM) in an attempt to bridge the gap between our recent understanding of the disease spectrum and its clinical presentation in relatives, which is key for early diagnosis and the institution of potential preventative measures. We also provide practical hints to identify subsets of the DCM syndrome where aetiology directed management has great clinical relevance.

• Klíčová slova
• Dilated cardiomyopathy, Heart failure, Position statement,
• MeSH
• časná diagnóza MeSH
• diferenciální diagnóza MeSH
• dilatační kardiomyopatie diagnóza   etiologie   terapie   MeSH
• kardiomyopatie diagnóza   etiologie   terapie   MeSH
• lidé MeSH
• multimodální zobrazování metody   MeSH
• myokarditida diagnóza   MeSH
• rizikové faktory MeSH
• rodokmen MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy MeSH

BACKGROUND: Several recent small studies have suggested a causal link between Lyme disease and dilated cardiomyopathy (DCM) by demonstrating the presence of the Borrelia burgdorferi (Bb) genome in the myocardium of patients with recent-onset DCM. The aim of this study was to further investigate the effect of targeted antibiotic treatment of Bb-related recent-onset DCM in a larger cohort of patients. PATIENTS AND METHODS: We performed endomyocardial biopsy (EMB) in 110 individuals (53 ± 11 years, 34 women) with recent-onset unexplained DCM, and detected the Bb genome in 22 (20 %) subjects. Bb-positive patients were subsequently treated with intravenous ceftriaxone for 21 days in addition to conventional heart failure medication. RESULTS: At the 1-year follow-up, a significant improvement in left ventricular (LV) ejection fraction (26 ± 6 vs. 44 ± 12 %; p < 0.01) and a decrease in LV end-diastolic (69 ± 7 vs. 63 ± 11 mm; p < 0.01) and end-systolic (61 ± 9 vs. 52 ± 4 mm; p < 0.01) diameters were documented. Moreover, a significant improvement in heart failure symptoms (NYHA class 3.4 ± 0.6 vs. 1.5 ± 0.7; p < 0.01) was also observed. CONCLUSION: Targeted antibiotic treatment of Bb-related recent-onset DCM in addition to conventional heart failure therapy is associated with favorable cardiac remodeling and improvement of heart failure symptoms.

• Klíčová slova
• Antibiotics, Borrelia burgdorferi, Dilated cardiomyopathy, Endomyocardial biopsy, Lyme disease,
• MeSH
• antibakteriální látky aplikace a dávkování   MeSH
• bakteriální endokarditida diagnóza   farmakoterapie   mikrobiologie   MeSH
• Borrelia burgdorferi izolace a purifikace   MeSH
• ceftriaxon aplikace a dávkování   MeSH
• dilatační kardiomyopatie diagnóza   farmakoterapie   mikrobiologie   MeSH
• injekce intravenózní MeSH
• kardiotonika aplikace a dávkování   MeSH
• kombinovaná farmakoterapie metody   MeSH
• lidé středního věku MeSH
• lidé MeSH
• lymeská nemoc diagnóza   farmakoterapie   mikrobiologie   MeSH
• výsledek terapie MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• klinické zkoušky kontrolované MeSH
• práce podpořená grantem MeSH
• Názvy látek
• antibakteriální látky MeSH
• ceftriaxon MeSH
• kardiotonika MeSH

A case of a 44-year-old female patient is described, examined for several weeks with shortness of breath and the symptoms of heart decompensation after repeated untreated respiratory infections. Echocardiographically determined diffuse hypokinesis of dilated left ventricle with ejection fraction of 20%. Coronarographic examination without any significant finding at the coronary bed. MRI of the myocardium supported the suspicion of myocarditis, followed by the endomyocardial biopsy with electron microscopic evidence of borrelia fragments. After the causal and symptomatic treatment, the patient experienced significant clinical improvement and full normalisation of the finding.

• MeSH
• biopsie MeSH
• dilatační kardiomyopatie diagnóza   patologie   MeSH
• dospělí MeSH
• echokardiografie MeSH
• elektronová mikroskopie MeSH
• endokard patologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• lymeská nemoc diagnóza   patologie   MeSH
• myokard patologie   MeSH
• myokarditida diagnóza   patologie   MeSH
• nízký srdeční výdej diagnóza   patologie   MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH