Showing papers by "Aleš Linhart published in 2019"

Heart failure in cardiomyopathies: a position paper from the Heart Failure Association of the European Society of Cardiology.

Abstract: Cardiomyopathies are a heterogeneous group of heart muscle diseases and an important cause of heart failure (HF). Current knowledge on incidence, pathophysiology and natural history of HF in cardiomyopathies is limited, and distinct features of their therapeutic responses have not been systematically addressed. Therefore, this position paper focuses on epidemiology, pathophysiology, natural history and latest developments in treatment of HF in patients with dilated (DCM), hypertrophic (HCM) and restrictive (RCM) cardiomyopathies. In DCM, HF with reduced ejection fraction (HFrEF) has high incidence and prevalence and represents the most frequent cause of death, despite improvements in treatment. In addition, advanced HF in DCM is one of the leading indications for heart transplantation. In HCM, HF with preserved ejection (HFpEF) affects most patients with obstructive, and ∼10% of patients with non-obstructive HCM. A timely treatment is important, since development of advanced HF, although rare in HCM, portends a poor prognosis. In RCM, HFpEF is common, while HFrEF occurs later and more frequently in amyloidosis or iron overload/haemochromatosis. Irrespective of RCM aetiology, HF is a harbinger of a poor outcome. Recent advances in our understanding of the mechanisms underlying the development of HF in cardiomyopathies have significant implications for therapeutic decision-making. In addition, new aetiology-specific treatment options (e.g. enzyme replacement therapy, transthyretin stabilizers, immunoadsorption, immunotherapy, etc.) have shown a potential to improve outcomes. Still, causative therapies of many cardiomyopathies are lacking, highlighting the need for the development of effective strategies to prevent and treat HF in cardiomyopathies.

The effect of enzyme replacement therapy on clinical outcomes in male patients with Fabry disease: A systematic literature review by a European panel of experts.

Abstract: Background Enzyme replacement therapy (ERT) with recombinant human α-galactosidase has been available for the treatment of Fabry disease since 2001 in Europe and 2003 in the USA. Treatment outcomes with ERT are dependent on baseline patient characteristics, and published data are derived from heterogeneous study populations. Methods We conducted a comprehensive systematic literature review of all original articles on ERT in the treatment of Fabry disease published up until January 2017. This article presents the findings in adult male patients. Results Clinical evidence for the efficacy of ERT in adult male patients was available from 166 publications including 36 clinical trial publications. ERT significantly decreases globotriaosylceramide levels in plasma, urine, and in different kidney, heart, and skin cell types, slows the decline in estimated glomerular filtration rate, and reduces/stabilizes left ventricular mass and cardiac wall thickness. ERT also improves nervous system, gastrointestinal, pain, and quality of life outcomes. Conclusions ERT is a disease-specific treatment for patients with Fabry disease that may provide clinical benefits on several outcomes and organ systems. Better outcomes may be observed when treatment is started at an early age prior to the development of organ damage such as chronic kidney disease or cardiac fibrosis. Consolidated evidence suggests a dose effect. Data described in male patients, together with female and paediatric data, informs clinical practice and therapeutic goals for individualized treatment.

The Role of Cardiac Imaging in the Diagnosis and Management of Anderson-Fabry Disease.

Abstract: Anderson-Fabry disease (AFD) is a rare X-linked inherited metabolic disorder which results in a deficiency or absence of the enzyme α-galactosidase A, leading to the accumulation of glycosphingolipids in various cells and organs including the heart. Cardiac involvement is common and results in increased myocardial inflammation, left ventricular hypertrophy (LVH), and myocardial fibrosis. Echocardiography and cardiovascular magnetic resonance (CMR) offer distinctive and often complementary use to assist in the diagnosis and monitoring pharmacologic therapy in AFD, including detection of the AFD cardiac phenotype, differentiation from other forms of LVH, and patient selection for therapeutic intervention. Advanced cardiac imaging holds promise in subclinical detection of AFD-related abnormalities as well as disease staging and prognostication.

Plasma osteopontin levels in patients with dilated and hypertrophic cardiomyopathy

Abstract: Osteopontin (OPN) is an extracellular matrix glycoprotein that plays a role in a variety of cellular activities associated with inflammatory and fibrotic responses. Increased OPN levels in myocardium and plasma have been demonstrated in patients with dilated cardiomyopathy (DCM). However, nothing is known about OPN levels in patients with hypertrophic cardiomyopathy (HCM). Therefore, the aim of our study was to compare plasma OPN levels in patients with these two most common cardiomyopathies. We examined plasma OPN as well as creatinine, C‑reactive protein (CRP), brain-type natriuretic peptide (BNP), and troponin I levels in 64 patients with DCM, 43 patients with HCM, and 75 control subjects. Transthoracic echocardiography was also performed on all cardiomyopathy patients. Plasma OPN levels were significantly elevated in patients with DCM compared with HCM patients (95 ± 43 vs. 57 ± 21 ng/ml; p < 0.001) and control subjects (54 ± 19 ng/ml; p < 0.001); however, there was no difference between HCM patients and control subjects. New York Heart Association (NYHA) class III or IV disease was more frequently present in DCM patients than in HCM subjects (44 % vs. 2 %, p < 0.0001). In multivariate analysis, BNP and CRP levels together with NYHA class were found to be significant predictors of plasma OPN levels in DCM patients (p = 0.002, p = 0.029, and p < 0.001 for BNP, CRP, and NYHA, respectively). Plasma OPN levels were associated with overall heart failure severity rather than with specific cardiomyopathy subtype in patients suffering from DCM or HCM, respectively.

Hyperuricemia treatment in acute heart failure patients does not improve their long-term prognosis: A propensity score matched analysis from the AHEAD registry

Abstract: Background Hyperuricemia is associated with a poorer prognosis in heart failure (HF) patients. Benefits of hyperuricemia treatment with allopurinol have not yet been confirmed in clinical practice. The aim of our work was to assess the benefit of allopurinol treatment in a large cohort of HF patients. Methods The prospective acute heart failure registry (AHEAD) was used to select 3160 hospitalized patients with a known level of uric acid (UA) who were discharged in a stable condition. Hyperuricemia was defined as UA >= 500 mu moL/L and/or allopurinol treatment at admission. The patients were classified into three groups: without hyperuricemia, with treated hyperuricemia, and with untreated hyperuricemia at discharge. Two- and five-year all-cause mortality were defined as endpoints. Patients without hyperuricemia, unlike those with hyperuricemia, had a higher left ventricular ejection fraction, a better renal function, and higher hemoglobin levels, had less frequently diabetes mellitus and atrial fibrillation, and showed better tolerance to treatment with angiotensin-converting enzyme inhibitors/angiotensin receptor blockers and/or beta-blockers. Results In a primary analysis, the patients without hyperuricemia had the highest survival rate. After using the propensity score to set up comparable groups, the patients without hyperuricemia had a similar 5-year survival rate as those with untreated hyperuricemia (42.0% vs 39.7%, P = 0.362) whereas those with treated hyperuricemia had a poorer prognosis (32.4% survival rate, P = 0.006 vs non-hyperuricemia group and P = 0.073 vs untreated group). Conclusion Hyperuricemia was associated with an unfavorable cardiovascular risk profile in HF patients. Treatment with low doses of allopurinol did not improve the prognosis of HF patients.

The left atrial substrate plays a significant role in the development of complex atrial tachycardia in patients with precapillary pulmonary hypertension.

Abstract: Atrial fibrillation (AF) and related atrial tachyarrhythmias (AT), including type I atrial flutter (AFL) are frequently observed in patients with pulmonary hypertension (PH). Their relationship to hemodynamic changes, atrial size, and ventricular function are still not fully verified. We retrospectively studied hemodynamic data, echocardiographic findings and arrhythmia incidence in 814 patients with invasively diagnosed precapillary PH (aged 59 ± 14 years; 46% males). Patients with combined or post-capillary PH were excluded. AF / AT were identified in 225 (28%) of all the study population. Compared to the subgroup without arrhythmia, patients with AF / AT had elevated right atrial pressure (11 ± 5 vs. 9 ± 5 mmHg), wedge pressure (11 ± 3 vs. 10 ± 3), a more enlarged right atrium (50 ± 12 vs. 47 ± 11 mm) and an increased left atrial diameter in the parasternal long axis projection, p < 0.05 for all comparisons. In the multivariate model, the left atrial size, patient age, arterial hypertension, diabetes and type of PH were associated with AF / AT occurrence, p < 0.05. Patients with type I AFL were more frequently male (39 (80%) vs. 62 (42%)), were younger (61 ± 11 vs. 67 ± 10 years), had increased pulmonary artery mean pressure (50 ± 12 vs. 45 ± 12 mmHg), less advanced left atrial dilatation (38 ± 10 vs. 42 ± 7 mm), and a more enlarged right atrium (56 ± 12 vs. 48 ± 11) as compared to subjects with AF or other AT, p < 0.05. The evidence of elevated wedge pressure and the enlargement of the left atrium especially in patients with AF suggest a parallel involvement of the left atrial substrate in arrhythmia formation despite invasively confirmed evidence of purely isolated precapillary PH. Substantial differences were noticed between patients with type I AFL and the remaining patients with other arrhythmia types.

Conundrum of implantable electric devices in Anderson-Fabry cardiomyopathy.

Abstract: Clinical management of arrhythmias is often guided by recommendations relating to the specific type of rhythm disturbance and/or underlying diagnosis. In their Heart publication, Vijapurapu and colleagues are analysing the use of cardiac implantable electric devices (CIEDs) in patients with Anderson-Fabry disease (AFD). This multicentre UK study analyses data from a large cohort of patients with AFD and compares individuals with CIEDs to those without devices. The paper brings new insights to the complex issue of arrhythmias related to AFD cardiomyopathy.1 To appreciate the relevance of presented data, we should understand the pathophysiology of this type of cardiomyopathy which differs from sarcomeric hypertrophic cardiomyopathy (HCM). Fabry cardiomyopathy is usually characterised by progressive diffuse left ventricular hypertrophy (LVH) with low frequency of LV outflow tract obstruction (LVOTO). Later stages are associated with inflammation, accelerated apoptosis and development of progressive myocardial replacement fibrosis affecting typically mid-wall layer of posterolateral basal LV segments (figure 1) and interstitial fibrosis, potentially also within the conduction system.2 The cardiomyopathy develops in adult patients usually from third or fourth decade of life regardless whether they suffer from the multisystemic ‘classical’ AFD or ‘later onset’ variants (usually characterised by cardiomyopathy and absent or minimal renal and neurological manifestations). The disease severity in female patients depends on the genetic variant and X-chromosome inactivation. In average, manifestations in women are milder and onset of signs and symptoms delayed. The study by Vijapurapu et al confirms that arrhythmias represent a frequent cause of morbidity and mortality in AFD, particularly in males in their fifth decade of life.1 Figure 1 Cardiac MRI showing scars (green arrows) by late gadolinium enhancement in patients with Anderson-Fabry disease (AFD). Patient A is a 54-year-old man classically affected with no history of palpitations or syncope. Patient B is a 52-year-old man with later-onset variant scanned …

30-year trends in blood pressure, prevalence, awareness, treatment and control of hypertension in the czech republic. the czech monica and czech post-monica studies

Abstract: Objective:Unlike most former East Bloc countries, the Czech Republic has seen a decrease in total, and cardiovascular (CV) mortality; the decrease, since 1985, was most pronounced in cerebrovascular mortality (77%).Objective: To assess trends in BP levels, prevalence, awareness, treatment and contro

Společné stanovisko European Association of Preventive Cardiology a European Association of Cardiovascular Imaging k indikaci a interpretaci kardiovaskulárních zobrazovacích metod při vyšetřování atletického srdce.

Abstract: Autoři originalniho textu EAPC a EACVI v plnem zněni: Antonio Pelliccia, Stefano Caselli jmenem European Association of Preventive Cardiology. Tato publikace zahrnuje přeložený souhrn oficialni plne verze European Association of Preventive Cardiology (EAPC) and European Association of Cardiovascular Imaging (EACVI) joint position statement: recommendations for the indication and interpretation of cardiovascular imaging in the evaluation of the athlete’s heart, jež byla původně publikovana v anglictině v European Heart Journal 2018; 39 (21)1949–1969, doi:10.1093/eurheartj/ehx532 vydavatelstvim Oxford University Press v licenci European Society of Cardiology, ESC. www.escardio.org/Guidelines/Consensus-and-Position-Papers/EAPC-Publications. © The European Society of Cardiology 2019

Normative reference ranges for echocardiographic chamber dimensions in a healthy Central European population: results from the Czech post-MONICA survey

Abstract: Normative reference values for echocardiographic chamber quantification are of great importance; however, this can be challenging. Our aim was to derive these values including degrees of abnormality from a random Central European population sample with a homogeneous subset of healthy subjects. We analysed echocardiograms obtained in a randomly selected population sample during the Czech post-MONICA survey in 2007/2008. Overall, 1850 out of 2273 persons of the whole sample of three districts had adequate echocardiograms (81.4%). A healthy subgroup defined by the absence of known cardiovascular disease was used to define normal reference range limits (n = 575, median age 42 years [IQR 34–52], 57% females). The whole population sample with predefined percentile cut-offs was used to define degrees of abnormality. Left ventricular (LV) size tended to decrease with age, while LV mass increased with age in both males and females and in both the healthy and general populations. LV dimensions were larger in males, except for body surface area-indexed LV diameter. M-mode derived LV measurements were larger and LV mass higher compared to 2D measurements. Right ventricle basal dimension was larger in males. Our study provides reference ranges for echocardiographic measurements obtained in a healthy subgroup derived from an epidemiological study of a Central European population. Where feasible, degrees of abnormality are provided based on the whole population sample including patients with disease. Our data show that age, gender and measurement method significantly affect cardiac dimensions and function and should be always taken into account.

Vzácná příčina opakované systémové embolie u ženy středního věku s nediagnostikovanou plicní AV malformací

Abstract: Uvadime připad opakovane systemove embolie u dosud zdrave 48lete ženy, kdy postupný klinický vývoj odhalil kombinaci vzacných anatomických a patologických nalezů. Prvnim projevem onemocněni u pacientky byla opakujici se systemova embolizace do leve paže vyžadujici opakovanou chirurgickou embolektomii ve velmi kratkem casovem sledu. Při souběžnem patrani po možnem zdroji byla postupně diagnostikovana klinicky něma plicni embolie, patentni foramen ovale a taktež významna cevni arteriovenozni (AV) malformace v leve plici. V dalsim průběhu doslo k necekanemu zvratu v podobě vzniku akutniho masivniho infarktu myokardu, opět embolizacniho původu, zdroj embolizace do leve koronarni tepny byl překvapivě objeven v ascendentni aortě. Nasledujici text podrobně popisuje průběh onemocněni teto pacientky. Připad poukazuje mimo jine na to, že i přes maximalni a vcasne diagnosticke a terapeuticke možnosti moderni kardiologie a interdisciplinarni spoluprace stale existuji vzacne a překvapive připady pacientů s hyperkoagulacnimi stavy, u kterých přicinu nemocnosti neumime zcela spolehlivě vysvětlit a i přes maximalni usili a terapii může být velmi obtižne odvratit připadne dalsi komplikace takoveho stavu. © 2019, CKS.

Third degree atrioventricular block as a rare complication of Graves' thyrotoxicosis

Abstract: Kompletni atrioventrikularni (AV) blokada představuje vzacnou, avsak život ohrožujici komplikaci tyreotoxikozy. V literatuře bylo dosud popsano pouze několik připadů s nejistou kauzalni souvislosti s hypertyroidnim stavem. Vzhledem k nedostatku udajů nepanuje absolutni shoda ohledně reverzibility teto abnormality AV převodu, a tedy i nutnosti implantace trvaleho kardiostimulatoru. V nasi kazuistice popisujeme připad 35lete pacientky, u ktere se nově diagnostikovana Gravesova-Basedowova tyreotoxikoza poprve manifestovala synkopou při kompletni AV blokadě. V uvodu hospitalizace doslo ke spontanni obnově AV převodu v poměru 1 : 1, což vedlo k výrazne sinusove tachykardii s frekvenci 150/min, ktera byla symptomaticka bolestmi na hrudi, prekolapsovým stavem a vedla k rozvoji srdecniho selhani v důsledku hyperkineticke cirkulace. Dalsi komplikaci byla epizoda asystolie při recidivě uplne AV blokady bez nahradniho rytmu s nutnosti dvouminutove kardiopulmonalni resuscitace a emergentni docasne transvenozni kardiostimulace. Při lecbě tyreostatiky a kortikosteroidy se po 48 hodinach stabilizoval srdecni rytmus se sklonem k sinusove tachykardii. Dalsi průběh byl již nekomplikovaný, vcetně dobre tolerance lecby beta-blokatorem. Komplexni vysetřeni (vcetně magneticke rezonance srdce) neprokazalo žadnou jinou přicinu vysvětlujici kompletni AV blokadu. Zvažovanou implantaci trvaleho kardiostimulatoru pacientka odmitla. V nasledujicich třech měsicich sledovani již nebyla recidiva AV blokady zaznamenana. © 2019, CKS.

Importance of endomyocardial biopsy in distinguishing between cardiac sarcoidosis and giant cell myocarditis : Need to distinguish between sarcoidosis and giant cell myocarditis.

Abstract: Cardiac sarcoidosis (CS) and giant cell myocarditis (GCM) represent rare types of myocardial inflammation frequently associated with ventricular tachycardia and advanced types of atrioventricular block (AVB) [1, 2]. The majority of patients with GCM either die or undergo heart transplantation unless combined immunosuppressive therapy is administered early in the course of the disease [3]. The case presented demonstrates that even complex multimodality imaging approach was not capable of distinguishing between CS and GCM and endomyocardial biopsy was essential for establishing the correct diagnosis. This

Alcohol Use in the Czech Republic – Joint Statement of the Society for Addiction Medicine of the J. E. Purkyně Czech Medical Association and the Czech Society of Cardiology

Abstract: Michal Miovský1, Miloš Táborský2, Petr Popov1, Aleš Linhart2, Tomáš Zima3 1Czech Society for Addiction Medicine, Czech Medical Association J. E. Purkyně, Prague, Czech Republic 2Czech Society of Cardiology, Brno, Czech Republic 3Institute of Medical Biochemistry and Laboratory Diagnostic, First Faculty of Medicine, Charles University and General University Hospital in Prague, Prague, Czech Republic