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Introduction We aimed to study the various cardiac manifestations of the two core neuroacanthocytosis (NA) syndromes, namely chorea-acanthocytosis (ChAc) and McLeod syndrome (MLS). So far, cardiac involvement has been described as specific feature only for MLS. Methods We studied six patients with ChAc (mean age 44.5 years, five men, one woman) and six patients with MLS (mean age 57.1 years, all men). Cardiac evaluation included echocardiography and/or cardiac magnetic resonance imaging (cardiac MRI), 24-h ECG-recording and examination of cardiac biomarkers. Results Cardiac involvement of ChAc was found in four of six patients. Two patients showed mildly reduced left ventricular ejection fraction (LVEF), two other patients mild to moderate left ventricular (LV) dilatation. Neither an increase in ventricular ectopic beats nor ventricular tachycardia were evident in ChAc. Four of five MLS patients showed left ventricle dilatation and reduced left ventricular ejection fraction (LVEF). Two of these, in addition, had critical ventricular tachycardia. High sensitive troponin T was elevated in all patients, for whom data were available (n = 10). In contrast, elevation of high sensitive troponin I was found in one of six ChAc and one of two MLS patients. Conclusion For the first time, we reveal cardiac involvement in a cohort of six ChAc patients, while the risk to develop heart failure seems lower than in MLS. Our study confirms the malignant nature of MLS in terms of ventricular arrhythmias and progression to advanced heart failure. Herein, we define disease-specific recommendations for cardiac assessment in both conditions.

Background Severe heart rhythm disturbances (SHRDs) occur regularly in cardiogenic shock (CS). Percutaneous left ventricular assist devices (pLVADs) can actively unload the left ventricle (LV), decreasing left ventricular end-diastolic pressure and wall tension, which are suspected parameters for the induction and maintenance of arrhythmias. The aim of this study was to describe effects of LV unloading on SHRD. Method In the Dresden Impella Registry, 97 patients received an Impella CP in refractory CS. Of them, 19 had SHRDs, which were not stopped by common therapeutic strategies such as electrical defibrillation or antiarrhythmic drugs. They were only stopped after implantation of a micro-axial heart pump. This phenomenon was referred to as heart rhythm stabilisation (HRS). Clinical outcome and laboratory parameters were assessed and risk factors for the occurrence of HRS were identified. Results All 19 patients with refractory SHRD terminated immediately into a stable heart rhythm after insertion of the micro-axial heart pump. In 37% no additional defibrillation was needed. Of the patients with HRS, CS was mostly caused by myocardial infarction (68%). Resuscitation before pLVAD was performed in 89% for more than 30 minutes. Patients with HRS were resuscitated more frequently and for a longer duration than patients without HRS. After HRS, the serum lactate and norepinephrine dosage decreased in the first 12 hours, whereas left ventricular ejection fraction increased by 95%. Conclusions Left ventricular unloading in patients with CS seems to be an option for treating patients with sustained life-threatening tachycardia, who are refractory to common treatment.

Background Uninterrupted direct oral anticoagulation (DOAC) in AF‐ablation is recommended, proven by randomized trials. The outcome and the periinterventional differences between DOACs and VKA in the real world clinical practice are discussed controversial. Hypothesis To investigate efficiency and safety of uninterrupted DOAC therapy compared to VKA during AF‐Ablation in real world setting with a focus on periinterventional heparin dosage.

Background Doppler microembolic signals (MES) occur during atrial fibrillation ablation despite of permanent flushed transseptal sheaths, frequent controls of periprocedural coagulation status and the use of irrigated ablation catheters Purpose To investigate the number and type of MES depending on the procedure time, prespecified procedure steps, the activated clotting time (ACT) during the ablation procedure and the catheter contact force. Methods In a prospective trial, 53 consecutive atrial fibrillation patients underwent pulmonary vein isolation by super-irrigated “point-by-point” ablation. All patients underwent a periinterventional, continuous transcranial Doppler examination (TCD) of the bilateral middle cerebral arteries during the complete ablation procedure. Results An average of 686±226 microembolic signals were detected by permanent transcranial Doppler. Thereby, 569±208 signals were differentiated as gaseous and 117±31 as solid MES. The number of MES with regard to defined procedure steps were as follows: gaseous: [transseptal puncture, 26 ± 28; sheath flushing, 24±12; catheter change, 21±11; angiography, 101±28; mapping, 9±9; ablation, 439±192; protamine administration, 0±0]; solid: [transseptal puncture, 8±8; sheath flushing, 9±5; catheter replacement, 6±6; angiography, not measurable; mapping, 2±5; ablation, 41±22; protamine administration, 0±0]. Significantly less MES occurred with shorter procedure time, higher ACT and the use of tissue contact force monitoring. Conclusion The current study demonstrates that during atrial fibrillation ablation using irrigated, “point-by-point” RF ablation, masses of microembolic signals are detected in transcranial ultrasound especially in the period of RF current application. The number of MES depends on the total procedure time and the reached ACT during ablation. The use of contact force monitoring might reduce MES during RF ablation.

(1) Background: Wilson&rsquo, s disease (WD) is an inherited autosomal recessive disorder with the excessive deposition of copper into different organs, including the heart. Previous studies showed structural cardiac changes even in patients with no signs of heart failure. The aim of this study was to perform cardiac magnetic resonance-based strain analysis in WD patients, as it is a powerful independent predictor of mortality. (2) Methods: We conducted a prospective cardiac magnetic resonance study that included 61 patients and 61 age and sex-matched controls, and performed strain analysis of the left and right ventricle. (3) Results: Left ventricular global longitudinal strain (GLS) as a prognostic marker of increased mortality was not altered (control &minus, 22.8 (4.8) % vs. WD patients &minus, 21.8 (5.1) %, p = 0.124). However, 4 of the 61 patients had a markedly reduced GLS. Global circumferential strain did not significantly differ between the groups either (p = 0.534). WD patients had significantly reduced global radial strain (p = 0.002). Right ventricular GLS was also significantly reduced in WD patients (p = 0.01). (4) Conclusions: Strain analysis revealed functional impairment of the left and right ventricle in a small number of patients as a potential early sign of cardiac manifestation in asymptomatic WD patients.

Background In the first three months after Transcatheter aortic valve implantation (TAVI), a remarkable number of patients have an unfavorable outcome. An inflammatory response after TAVI is suspected to have negative effects. The exact mechanisms remain unclear. We examined the influence of monocyte subpopulations on the clinical outcome, along with the degree of monocyte activation and further parameters of inflammation and platelet activation. Methods Flow-cytometric quantification analyses of peripheral blood were done in 120 consecutive patients who underwent TAVI (one day before TAVI and on day 1 and 7 after TAVI). Monocyte-subsets were defined by their CD14 and CD16 expression, monocyte-platelet-aggregates (MPA) by CD14/CD41 co-expression. The extent of monocyte activation was determined by quantification of CD11b-expression (activation epitope). Additionally, pro-inflammatory cytokines such as interleukin (IL)-6, IL-8, C-reactive protein were measured with the cytometric bead array method or standard laboratory tests. Results Elevated Mon2 (CD14++CD16+) - monocytes (38 vs. 62 cells/μl, p Conclusions Elevated Mon2-monocytes and a high level of monocyte activation before TAVI are associated with early mortality after TAVI. Chronic inflammation in aging patients seems to be an important risk factor after TAVI.

Background: Following myocardial infarction (MI), apoptosis occurs early in the remote myocardium and contributes to the processes of myocardial remodelling. Increased nitrosative stress is a well-known and potent inductor of myocardial apopto¬sis. Excess activation of endothelial nitric oxide synthase (eNOS) increases its uncoupling potential and results in nitrosative stress via formation of peroxynitrite. However, the pathophysiological role of eNOS signalling in the remote myocardium after MI is as yet undefined. Aim: The impact of eNOS activation on pro- and anti-apoptotic signalling in the remote myocardium and the influence of pretreatment with the eNOS cofactor tetrahydrobiopterin (BH4) on eNOS activation, nitrosative stress level, and apoptosis induction and execution were studied in a rat MI model in vivo. Results: Twenty-four hours after anterior MI, eNOS activity in animals treated with left anterior descending coronary artery ligation (LIG) significantly increased in the posterior left ventricular (LV) myocardium as did protein nitrosylation when com¬pared to sham treatment. This was paralleled by induction of apoptosis via the extrinsic and intrinsic pathways. Moreover, anti-apoptotic signalling via protein kinase B/Akt and glycogen synthase-kinase 3 beta was suppressed. Notably, pretreatment with the eNOS cofactor BH4 reduced eNOS activation, prevented excess protein nitrosylation, blunted apoptosis induction, facilitated anti-apoptotic signalling, and eventually prevented apoptosis execution. Conclusions: Here we showed that 24 h after experimental MI in rats in vivo, apoptosis was induced in the posterior non-in¬farcted LV wall. Evidence is presented that pretreatment with the eNOS cofactor BH4 resulted in less nitrosative stress and weakened apoptotic processes, although the stabilisers contained did participate in this phenomenon. Because apoptosis is a crucial component of myocardial remodelling, influencing eNOS signalling might be an interesting pharmacological target for the development of novel anti-remodelling therapies.

Chromogranin B (CGB) regulates B-type natriuretic peptide (BNP) production. Circulating CGB levels are elevated in heart failure (HF) animal models and HF patients, but also increase in healthy individuals in response to physical activity. Therefore, CGB seems to integrate information from myocardial stress and systemic neuro-endocrine activation. Substantial gaps remain in our understanding of CGB regulation in HF.We conducted a retrospective registry study including 372 patients. CGB and N-terminal pro-BNP (NT-proBNP) plasma levels were assessed in acute HF and chronic valvular HF patients and controls. CGB levels were significantly increased in acute HF and chronic valvular HF, but significantly higher in the latter. Patients in chronic valvular HF with severe mitral regurgitation (cHF-MR) showed significantly higher CGB levels than patients in chronic valvular HF with severe aortic stenosis. CGB levels progressively increased with worsening NYHA functional status and were moderately correlated to NT-proBNP, but independent of left ventricular (LV) ejection fraction (LVEF), LV mass, age and body weight. Finally, cHF-MR patients showed significant reductions of CGB levels after interventional mitral valve repair.CGB is a promising emerging biomarker in HF patients with unique potential to integrate information from myocardial stress and neuro-endocrine activation.

BACKGROUND Interventional mitral valve (MV) repair of severe symptomatic mitral regurgitation (MR) is a therapeutic option in high-risk surgical or inoperable patients. Assessment of the MV remains a crucial part of pre-interventional screening. Three-dimensional transoesophageal echocardiography (3D-TOE) may compensate for well-known pitfalls that occur in 2D-TOE. PURPOSE We investigated whether the functional length of the central segments of the posterior and anterior MV leaflets (PML-P2 and AML-A2) is more reliably determined by 3D-TOE full volume datasets (3D-MPR) or orthogonal biplane-imaging (Xplane) when compared to 2D-TOE. METHODS AND RESULTS Between February 2014 and August 2015, 265 consecutive patients with moderate to severe symptomatic MR were screened. Seventy patients were judged suitable for interventional MV repair by the in-house Heart-Team. Eventually, 59 patients remained for data analysis. Inter-observer variability was lowest in 3D-MPR followed by Xplane (r = 0.92 and 0.90, p

Platelet transfusion is an effective option to reverse platelet inhibition in thienopyridine-treated patients suffering from bleedings or requiring urgent surgery. However, in ticagrelor-treated patients, the previous studies revealed significant clinical effects to platelet rich plasma (PRP) but poor response to pooled platelets (PP) as used in clinical routine. The aim of this study was to elucidate a potential pathomechanism to explain the poor response of ticagrelor to PP. From 79 whole blood samples of patients treated with ticagrelor, prasugrel, or clopidogrel, the PRI-VASP was determined before and after in vitro platelet supplementation of PP or PRP at increasing concentrations. Compared to prasugrel- and clopidogrel-treated patients, the PRI-VASP of ticagrelor-treated patients showed no significant increase after in vitro administration of PP. PRI-VASP was performed in ticagrelor-treated samples after in vitro addition of 1: centrifuged PRP platelets resuspended in PP buffer, 2: PP with human serum, 3: human serum alone. Surprisingly, PP with human serum or human serum alone were able to significantly increase PRI-VASP in samples of ticagrelor-treated patients (11.7 ± 10.9 → 61.3 ± 10.9%, p = 0.006; 11.7 ± 10.9 → 54.1 ± 2.7%, p

Kontext: Starsi studie prokazaly, že se objem, hmota a funkce leve (LK) a prave komory (PK) srdecni významně lisi podle toho, zda se při výpoctu těchto parametrů zahrnou svalove tramce a papilarni svaly (trabeculae papillary and papillary muscle, TPM).Metody: V kohortě 101 pacientů bylo provedeno vysetřeni srdce magnetickou rezonanci (cardiac magnetic resonance, CMR). Kohorta zahrnovala celkem 26 pacientů bez patologických nalezů na CMR (referencni skupina), pacienty s ischemickou kardiomyopatii (IKMP), s dilatacni kardiomyopatii (DKMP) a pacienty s hypertrofii leve komory (vždy po 25 pacientech). Parametry LK a PK se stanovovaly pomoci dřive zavedených metod: 1. metoda zahrnujici TPM a 2. metoda nezahrnujici TPM do objemu přislusneho srdecniho oddilu.Výsledky: Ve srovnani s výpoctem při zahrnuti TPM znamenalo vylouceni TPM z celkoveho objemu LK a PK významně mensi objem na konci diastoly a systoly (end-diastolic and end-systolic volume, EDV, ESV) a hmotu myokardu, a vyssi hodnoty tepoveho objemu (stroke volume, SV) a ejekcni frakce (EF) (p = 0.001). Podil TPM na hmotě LK a PK byl větsi u DKMP (18,4 ± 3,8 %) a IKMP (17,8 ± 3,2 %) než u referencni skupiny (15,2 ± 2,5 %; oboji p < 0,05); výsledkem byl statisticky významně větsi rozdil mezi oběma použitým metodami (1. metoda - 2. metoda) při výpoctu ESV, EDV, SV, EF a hmoty myokardu u nemocných s DKMP a IKMP oproti referencni skupině.Zavěr: Hodnoty parametrů LK při vysetřeni srdce MR významně ovlivňuje to, zda jsou TPM považovany za soucast krevniho poolu LK nebo za soucast hmoty LK. Aby se zabranilo chybne interpretaci výsledků vysetřeni a chybným rozhodnutim, může být při dlouhodobem sledovani pacientů naprosto zasadni systematicke použivani jedne z obou metod delineace LK.

Background: Peri-interventional myocardial injury occurs frequently during transcatheter aortic valve implantation (TAVI). We assessed the effect of remote ischemic preconditioning (RIPC) on myocardial injury, acute kidney injury (AKIN) and 6-month mortality in patients undergoing TAVI. Methods: We performed a prospective single-center controlled trial. Sixty-six patients treated with RIPC prior to TAVI were enrolled in the study and were matched to a control group by propensity-score. RIPC was applied to the upper extremity using a conventional tourniquet. Myocardial injury was assessed using high-sensitive troponin-T (hsTnT), and kidney injury was assessed using serum creatinine levels. Data were compared with the Wilcoxon-Rank and McNemar tests. Mortality was analysed with the log-rank test. Results: TAVI led to a significant rise of hsTnT across all patients (p &lt, 0.001). No significant inter-group difference in maximum troponin release or areas-under-the-curve was detected. Medtronic CoreValve and Edwards Sapien valves showed similar peri-interventional troponin kinetics and patients receiving neither valve did benefit from RIPC. AKIN occurred in one RIPC patient and four non-RIPC patients (p = 0.250). No significant difference in 6-month mortality was observed. No adverse events related to RIPC were recorded. Conclusion: Our data do not show a beneficial role of RIPC in TAVI patients for cardio- or renoprotection, or improved survival.

Background Cardiogenic shock (CS) is often associated with severe heart rhythm disturbances (SHRD). Percutaneous left ventricular assist devices (pLVAD) can actively unload the left ventricle (LV) using a micro-axial pump and resulting in a decreased end-diastolic pressure and wall tension. These parameters are suspected to induce and maintain rhythmological instability. Purpose In the current study, we firstly describe the termination of SHRD immediately (less than 5 minutes) after LV-unloading in CS patients with previous unsuccessful antiarrhythmic treatment. Methods The Dresden Impella Registry is an ongoing single center registry. Since 2014, a total of 97 patients were included. Each of whom had received a micro-axial heart pump in refractory CS supplying a circulatory support of 3.5 l/min. We investigated the subgroup of patients which initially exhibited SHRD like ventricular tachycardia or ventricular fibrillation, and showed an immediately stabilization of heart rhythm directly after insertion of pLVAD (HRS). This subgroup was compared with the other patients of the registry (NHRS). Therefore, clinical laboratory and hemodynamic parameters were measured and analyzed. Results In 19 patients of the registry a HRS was observed. Among these patients, a CPR before pLVAD was performed in 89.5% with a mean duration of 30.7min, whereby 52.6% sustained an in-hospital cardiac arrest and 36.9% an out-of-hospital cardiac arrest respectively. In the NHRS subgroup (n=78), a CPR was performed less frequently (39.7%; p The mortality showed no differences between the HRS and NHRS cohort at 30 days (68.4% vs. 58.1%; p=0.413) and 90 days (78.9% vs. 66.7%; p=0.306), despite a more frequent and longer CPR with a higher ratio of out-of-hospital cardiac arrests among the HRS patients. There was also no difference in mortality between patients, who received an in-hospital CPR. However, HRS patients with in-hospital CPR showed a significantly lower serum lactate and NA dosage compared to the NHRS cohort (Figure A & B). Furthermore, NA recovery, defined as 50% decrease as compared to the initial NA dosage, occurred more frequently in the HRS group (HRS 42.9% vs. NHRS 7.1%; p=0.049). The LVEF nearly double in the HRS subgroup after LV-unloading, whereas it did not change in the NHRS subgroup (relative LVEF increase: HRS 95% vs. NHRS 15%). Figure A & B Conclusion The termination of SHRD due to LV-unloading occurred in around 20% of CS patients in Dresden Impella Registry and was associated with a lower serum lactate and NA dosage as well as an improved LVEF among patients with in-hospital CPR. Acknowledgement/Funding None

Background Wilson's disease (WD) is an inherited autosomal recessive disorder resulting from abnormal copper metabolism. Relatively little is known about the effects of copper accumulation on the heart. Objective We aimed to determine if patients with Wilson's disease show signs of cardiac involvement and structural heart disease. Methods In this prospective trial, we studied 61 patients with Wilson's disease and compared them to 61 age- and gender-matched healthy controls. Results While left ventricular function assessed by global longitudinal and global radial strain did not differ significantly between the groups, Wilson's disease patients had significantly reduced global radial strain (table 1). Wilson's disease patients demonstrated significantly more late gadolinium enhancement than the control patients (4.9±1.4 vs. 1.1±0.2% p The severity of Wilson's disease, based on the Unified Wilson's Disease Rating Scale, was significantly correlated with the extent of late gadolinium enhancement (r=0.53, P=0.001), cardiac troponin (r=0.56, P=0.001), the number of premature ventricular contraction (r=0.66, P=0.001). Table 1. Myocardial strain and CMR characteristics of patients and controls Parameter Patients (n=61) Controls (n=61) p value Left ventricular parameters GLS, % −22.8 (4.8) −21.8 (5.1) 0.124 GRS, % 43.2 (13.2) 51.6 (13.8) 0.002 GCS, % −29.2 (5.2) −28.6 (4.7) 0.534 Late gadolinium enhancement LGE, %* 4.9 (1.4) 1.1 (0.2) 0.003 LGE at RVIP, n (%) 58 (95) 3 (5) Conclusion Our data demonstrate that cardiac involvement in Wilson's disease is possible and those patients who are severely affected by the disease carry a higher risk of developing structural heart disease.

Chromogranin B and inositol 1,4,5-trisphosphate-associated calcium signaling leading to increased natriuretic peptide production has been described in cardiac hypertrophy. Here, we performed left anterior descending coronary artery ligation in rats as a model for systolic heart failure and examined protein and gene expression clusters in the infarcted and noninfarcted myocardium and moreover under treatment with metoprolol. We found that atrial natriuretic peptide gene transcription was significantly more elevated in the infarcted compared with the noninfarcted myocardium. Chromogranin B, which facilitates calcium release from internal stores through the inositol 1,4,5-trisphosphate receptor, was upregulated in both areas. Interestingly, angiotensin II receptor type 1 gene transcription was significantly upregulated in the infarcted and unchanged in the noninfarcted myocardium. Nuclear factor ĸappa B as a calcium-dependent transcription factor showed increased activity in the infarction zone. The β-adrenergic axis does not seem to be involved, as metoprolol treatment did not have a significant impact on any of these results. We conclude that region-specific upregulation of angiotensin II receptor type 1 is a major factor for increased atrial natriuretic peptide production in the infarcted anterior wall. This effect is most likely achieved through inositol 1,4,5-trisphosphate-mediated cytosolic calcium increase and subsequent nuclear factor ĸappa B activation, which is a known transcription factor for natriuretic peptides.

Serine proteases and G-protein-coupled receptors have been studied extensively as effectors of cell death. However, their roles in myocardial infarction have not been determined. In this study, we investigated the influence of the plasminogen activator system involving urokinase and urokinase receptor on necrosis after acute myocardial infarction. Myocardial infarction and reperfusion were induced in mouse hearts using the in vitro Langendorff model. DNA fragmentation and cleaved caspase-3 activity in urokinase- (uPA(-/-)) and urokinase receptor-knockout mice (uPAR(-/-)) were determined and compared with those in wild-type mice using in situ nick-end DNA labeling (TUNEL) and enzyme-linked immunosorbent assays, respectively. Infarct sizes were determined using propidium iodide and fluorescent microspheres. Following regional ischemia and reperfusion, a significant increase in the number of TUNEL-positive nuclei was observed in the ischemic zone in mouse hearts and to a lesser degree in regions remote from the ischemic area in wild-type, uPAR(-/-), and uPA(-/-) groups compared with those in directly removed hearts. No significant differences were observed between uPAR(-/-) and wild-type mice. Conversely, a significant reduction in DNA fragmentation was observed in ischemic and nonischemic regions after acute myocardial infarction in uPA(-/-) mice when compared with that in wild-type and uPAR(-/-) groups. The resulting infarct sizes were significantly smaller in uPA(-/-) mice than in uPAR(-/-) and wild-type mice. These data demonstrated the involvement of uPA, but not uPAR, in protecting against necrosis during acute myocardial infarction.

Aims It is hypothesized that inflammation could promote structural and electrical remodelling processes in atrial fibrillation (AF). Atrial infiltration of monocytes and granulocytes has been shown to be dependent on CD11b expression. The aim of this study was to investigate whether treatment of AF by pulmonary vein isolation (PVI) may lead to reduced inflammation, as indicated by a decrease of CD11b expression on monocytes and granulocytes. Methods and results Flow-cytometric quantification analysis and determination of systemic inflammatory markers of peripheral blood were performed in 75 patients undergoing PVI 1 day before and 6 months after PVI. The extent of activation of monocytes and granulocytes was measured by quantifying the cell adhesion molecule CD11b. The mean expression of CD11b on monocytes (20.9 ± 2.5 vs. 10.2 ± 1.4; P

Background: This study evaluated the wearable cardioverter-defibrillator (WCD) for use and effectiveness in preventing sudden death caused by ventricular tachyarrhythmia or fibrillation. Methods: From April 2010 through October 2013, 6043 German WCD patients (median age, 57 years; male, 78.5%) were recruited from 404 German centers. Deidentified German patient data were used for a retrospective, nonrandomized analysis. Results: Ninety-four patients (1.6%) were treated by the WCD in response to ventricular tachyarrhythmia/fibrillation. The incidence rate was 8.4 (95% confidence interval, 6.8–10.2) per 100 patient-years. Patients with implantable cardioverter-defibrillator explantation had an incidence rate of 19.3 (95% confidence interval, 12.2–29.0) per 100 patient-years. In contrast, an incidence rate of 8.2 (95% confidence interval, 6.4–10.3) was observed in the remaining cardiac diagnosis groups, including dilated cardiomyopathy, myocarditis, and ischemic and nonischemic cardiomyopathies. Among 120 shocked patients, 112 (93%) survived 24 hours after treatment, whereas asystole was observed in 2 patients (0.03%) with 1 resulting death. ConclusionS: This large cohort represents the first nationwide evaluation of WCD use in patients outside the US healthcare system and confirms the overall value of the WCD in German treatment pathways.

Pacient s cevnim typem Ehlersova-Danlosova syndromu a s implantovanou mechanickou chlopni byl hospitalizovan pro dyspnoe a bolest na hrudi. Po vylouceni infarktu myokardu nasledovalo vysetřeni CT angiografii, ktere prokazalo spontanni rupturu jedne z mezižebernich tepen s nasledným vznikem hemothoraxu a rozvojem kompresivni atelektazy. Celý připad komplikovala soucasna antikoagulacni lecba warfarinem po implantaci mechanicke chlopně. Po neuspěsne konzervativni lecbě byl připad řesen chirurgicky. Jak ukazuje nase kasuistika, může implantace mechanicke chlopně s nezbytnou odpovidajici a dlouhodobou antikoagulaci warfarinem u těchto pacientů představovat nežadouci přidatne riziko.

Background The clinical effect of copper accumulation on the heart of patients suffering from Wilson’s disease (WD) is not completely understood. We aimed to determine if patients with WD show signs of cardiac involvement, structural heart disease or autonomic dysfunction. In this prospective trial, we studied 61 patients (mean age 44.3 ± 15.2 years, 51% males) with WD and compared them to 61 age- and gender-matched healthy controls. All subjects underwent clinical examination, blood tests, echocardiography and 24 h electrocardiographic (ECG) recording. Results Left- and right ventricular systolic function did not differ significantly between WD patients and controls. However, 5 of the 61 patients had a reduced left ventricular ejection fraction (LVEF). Furthermore, diastolic dysfunction was more prevalent in WD patients (9 of 61 vs. 0 of 61, p = 0.001). The severity of WD based on the Unified Wilson’s Disease Rating Scale was significantly correlated to NT-pro BNP (r = 0.34, P = 0.013). Patients with an exacerbation of WD in medical history had higher troponin levels compared to those without (11.3 ± 4.7 vs 4.6 ± 1.2). The autonomic function assessed by triangular index (TI) and SDNN-index was significantly reduced in WD patients compared to controls in most in almost every age category (p-value TI and SDNN: age 20–29, p

Objectives Non-ischaemic dilated cardiomyopathy (DCM) is characterised by a highly variable disease progression. Stress echocardiography and cardiopulmonary exercise testing (CPET) are beneficial in risk assessment, but are labour intensive. Repetitive squatting and standing without weights is a simple exercise (EX). The aim of this study was to investigate the prognostic role of left ventricular (LV) contractile recruitment (CR) after a simple EX of repetitive squatting through three-dimensional (3D) echocardiography. Methods Patients with DCM (LV ejection fraction (EF)4%). Patients were followed up prospectively (2 years) for cardiac death and deteriorating heart failure. Results During follow-up, 14 cardiac events occurred (21%) with six deaths and eight severe heart failure deteriorations. A poor CR after squatting EX differentiated DCM patients with cardiac events during follow-up as accurately as a reduced peak oxygen consumption (peak VO 2

SummaryA growing body of evidence suggests a pivotal role of inflammatory processes in AF in a bidirectional manner. Infiltrating leukocytes seem to promote both structural and electrical remodelling processes in patients with AF. Monocyte-platelets-aggregates (MPAs) are sensitive markers of both platelets and monocyte activation. So far it is not clear whether the content of MPAs is affected by AF. The present study examined the content of MPAs and the activation of monocytes in elderly patients with an aortic stenosis in dependence of AF. These patients are known to have a high prevalence of AF. Flow-cytometric quantification analysis demonstrated that patients with AF have an increased content of MPAs (207 ± 13 cells/μl vs 307 ± 21 cells/μl, pSupplementary Material to this article is available online at www.thrombosis-online.com.

Aims Iatrogenic pseudoaneurysms of the femoral artery lead to increased morbidity and mortality, especially when surgical treatment is necessary. Manual compression and thrombin injection are commonly used to occlude the pseudoaneurysms. However, in some cases these treatment options are inapplicable or unsuccessful. The aim of the present study was to examine the feasibility, effectiveness and safety of a novel approach with the use of suture-based closure devices to treat pseudoaneurysms. Methods and results Between January 2014 and May 2016, a total of eight iatrogenic pseudoaneurysms of the femoral artery were treated by the interventional closure technique after at least one ineffective attempt at manual compression. After puncture of the cavity, a PTCA guidewire was used to pass the neck of the pseudoaneurysm and a sheath was inserted in the femoral artery. Afterwards, a suture-based closure system (ProGlide) was used to occlude the neck. All eight pseudoaneurysms were successfully obliterated. No complications occurred during the procedure. Conclusions The new interventional technique presented in this study fills the gap in successfully treating pseudoaneurysms that cannot be obturated with conventional techniques. By implementing this new technique in clinical practice, a significant number of open surgical repairs could be prevented.

After cardiac arrest due to acute coronary syndromes (ACS) therapeutic hypothermia (HT) is the standard care to reduce neurologic damage. Additionally, the concomitant medical treatment with aspirin and a P2Y12 receptor inhibitor like clopidogrel (Cl), prasugrel (Pr) or ticagrelor (Ti) is mandatory. The platelet inhibitory effect of these drugs under hypothermia remains unclear.164 patients with ACS were prospectively enrolled in this study. 84 patients were treated with HT, 80 patients were under normothermia (NT). All patients were treated with aspirin and one of the P2Y12 receptor inhibitors Cl, Pr or Ti. 24h after the initial loading dose the platelet reactivity index (PRI/VASP-index) was determined to achieve the platelet inhibitory effect.In the HT-group the PRI/VASP-index was significantly higher compared to the NT-group (54.86%±25.1 vs. 28.98%±22.8; p0.001). In patients under HT receiving Cl, the platelet inhibition was most markedly reduced (HT vs. NT: 66.39%±19.1 vs. 33.36%±22.1; p0.001) compared to Pr (HT vs. NT: 37.6%±25.0 vs. 27.04%±25.5; p=0.143) and Ti (HT vs. NT: 41.5%±21.0 vs. 17.83%±14.5; p=0.009). The rate of non-responder defined as PRI/VASP-index50% was increased in HT compared to NT (60.7% vs. 22.5%; p0.001) with the highest rates in the group receiving Cl (CL: 82% vs. 26%, p0.001; Pr: 32% vs. 23%; n.s.; Ti: 30% vs. 8%, n.s.).The platelet inhibitory effect in patients treated with HT after cardiac arrest is significantly reduced. This effect was most marked with the use of Cl. The new P2Y12-inhibitors Pr and Ti improved platelet inhibition in HT, but could not completely prevent non-responsiveness.

For evaluation of aortic valve area (AVA), transthoracic echocardiography (TTE) is the method of choice. Cardiac magnetic resonance (CMR) at 1.5-Tesla is an alternative. The aim of the study was to check whether quantification of whole range of AVA without severe aortic stenosis is possible and reliable in higher magnetic field strength, and also including a comparison to TTE.In 3-T CMR phase contrast sequences were assessed above aortic valve and left ventricular output tract. AVA was calculated using the continuity equation. Planimetric analysis of AVA was performed in magnitude images. TTE was used as reference method for graduation of AVA.Totally 48 patients (64 ± 18 years) without severe aortic valve stenosis were prospectively enrolled. In CMR planimetric AVA was 2.5 ± 1.3 cm(2) and calculated AVA 2.4 ± 1.3 cm(2), whereas AVA in TTE was 1.9 ± 1.1 cm(2). Planimetric and calculated AVA in CMR and also AVA in CMR and TTE showed good correlation (r = 0.97, 0.92, respectively). Bland-Altman analysis demonstrated no signs of over- or underestimation. Inter- and intraobserver variabilities were low.Determination of AVA using 3-T CMR is possible using direct planimetry and continuity equation. CMR is the alternative first choice method in cases with discrepant or insufficient echocardiographic results.

Objective Hypoxia plays a pivotal role in development and progression of restenosis after vascular injury. Under hypoxic conditions the hypoxia-inducible factors (HIFs) are the most important transcription factors for the adaption to reduced oxygen supply. Therefore the aim of the study was to investigate the effect of a local HIF-inhibition and overexpression on atherosclerotic plaque development in a murine vascular injury model. Methods and results After wire-induced vascular injury in ApoE −/− mice a transient, local inhibition of HIF as well as an overexpression approach of the different HIF-subunits (HIF-1α, HIF-2α) by adenoviral infection was performed. The local inhibition of the HIF-pathway using a dominant-negative mutant dramatically reduced the extent of neointima formation. The diminished plaque size was associated with decreased expression of the well-known HIF-target genes vascular endothelial growth factor-A (VEGF-A) and its receptors Flt-1 and Flk-1. In contrast, the local overexpression of HIF-1α and HIF-2α further increased the plaque size after wire-induced vascular injury. Conclusions Local HIF-inhibition decreases and HIF-α overexpression increases the injury induced neointima formation. These findings provide new insight into the pathogenesis of atherosclerosis and may lead to new therapeutic options for the treatment of in stent restenosis.

Wilson’s disease (WD) is an inherited autosomal recessive disorder which leads to reduced copper excretion and excessive deposition of the copper into different organs. However, relatively little is known about the effects of copper accumulation on the heart. Cardiac involvement in WD has

Introduction Cardiac dysfunction occurs in several forms of limb girdle muscular dystrophy (LGMD). The aim of this study was to investigate cardiac involvement in calpainopathy (LGMD2A). Methods Cardiovascular evaluation was performed in 10 patients with genetically verified LGMD2A by echocardiography, 3 Tesla - cardiovascular magnetic resonance, 24-h electrocardiography recordings with heart rate variability (HRV) analysis, and 24-h blood pressure recordings. Results No patient with calpainopathy showed impairment of left or right ventricular function. One patient had a small amount (2% of left ventricle mass) of late gadolinium enhancement. HRV analysis revealed no significant difference compared with external reference data. Conclusions The main finding of this study is the lack of cardiac involvement in patients with calpainopathy. Cardiac involvement was not found, even in individuals with advanced age and greater disease severity. Furthermore, we did not observe an overall reduction of cardiac autonomic regulation in calpainopathy.

We present a case of a 57-year-old female patient with transient global amnesia, who later developed broken heart syndrome also known as takotsubo cardiomyopathy. The present case underlines that co-occurrence of both pathologies might still be an under-recognized clinical problem.

While impact of surgical aortic valve replacement (SAVR) on the function of the right and left ventricle (RV, LV) has been studied extensively, the objective of this study was to compare the two different transcatheter approaches, transfemoral vs. transapical aortic valve implantation (tf-AVI, ta-AVI) and SAVR with respect to postoperative recovery of RV and LV function in patients with severe aortic stenosis.Conventional echocardiographic studies were performed before and after the procedure (8 days) in 74 consecutive patients undergoing tf-AVI, 88 with ta-AVI and 63 surgically treated patients. Post procedure, a marked deterioration in the tricuspid annular plane systolic excursion (TAPSE) was seen after SAVR (23.7 +/- 4 mm vs. 15.6 +/- 2.9 mm, P0.001) and ta-AVI (21.1 +/- 4.7 mm vs. 19.1 +/- 4.7 mm, P = 0.02), while TAPSE remained unchanged in the tf-AVI group (21.7 +/- 5 mm vs. 22.1 +/- 4.9 mm, P = 0.38). Additionally, a significant drop of the visually estimated right ventricular ejection fraction in the SAVR and ta-AVI group compared to tf-AVI could be seen (P0.001, P = 0.003, respectively) Among patients with a pre-existing reduced LV-function, despite similar baseline LVEF (38.5 +/- 7.1% vs. 37.4 +/- 5.6, P = 0.8), tf-AVI patients had better recovery of LVEF compared with ta-AVI (ALVEF 7.2 +/- 8% vs. 1.6 +/- 9.3%, P = 0.038).Our study underlines the less invasive nature of tf-AVI, causing less intraoperative trauma and pericardial adhesions to the heart. Given that acute RV failure after cardiac surgery remains a major cause of morbidity and mortality, the results of the present study suggest that in high-risk patients, TAVI and in particular tf-AVI could be favoured over SAVR in regard to a RV dysfunction.

Atrial fibrillation (AF) is known to cause platelet activation. AF and its degree of thrombogenesis could be associated with monocyte-platelet aggregates (MPAs). We investigated on whether the content of MPAs or other platelet activation markers is associated with the recurrence of AF after pulmonary vein isolation (PVI). A total of 73 patients with symptomatic AF underwent PVI. After 6 months, all patients were evaluated for episodes of AF recurrence. At the same time, flow-cytometric quantification analyses were performed to determine the content of MPAs. Further platelet activation parameters were detected by using either cytometric bead arrays or quantitative immunological determination. Patients with recurrent AF (n = 20) compared to individuals without AF relapse (n = 53) were associated with an increased content of MPAs (43 ± 3% vs. 33 ± 2%, p = 0.004), as well as an increased CD41 expression on monocytes (191 ± 20 vs. 113 ± 6, p = 0.001). The level of the soluble platelet activation markers such as D-dimer, sCD40L, and sP-selectin did not differ between these groups. The content of MPAs correlated weakly with the level of sCD40L (r = 0.26, p = 0.03), but not with sP-selectin and D-dimer, whereas sP-selectin and sCD40L correlated with each other (r = 0.38, p = 0.001). Only the cellular marker of platelet activation, the content of MPAs, was increased in patients with recurrent AF after PVI. In contrast, soluble markers remained unaltered. These data indicate a distinct mechanism and level of platelet activation in AF. The clinical relevance of MPAs in identifying AF recurrence or in guiding the therapy with anticoagulants remains to be elucidated.

The soluble form of ST2 (sST2) is a novel laboratory parameter for cardiac risk prediction, and over the past years, several studies have tried to evaluate its utility, especially in the management of heart failure. We investigated whether increased serum levels of sST2 show a characteristic pathomorphologic pattern in 3-Tesla cardiac magnetic resonance imaging (CMRI).One hundred and fifty-six patients referred to 3T CMRI due to suspected coronary artery disease (CAD) or myocarditis were prospectively enrolled in the study. Ninety patients were diagnosed with CAD, 22 patients with myocarditis, and 44 patients, who constituted the reference group, showed no pathologic CMRI pattern.There was no significant difference between the sST2 values for patients in the reference group and patients with CAD or myocarditis. The sST2 concentration showed a weak correlation with the NYHA functional class (P = 0.002, r = 0.22), but correlation of sST2 and LGE, left ventricular parameters, and LVEF could not be seen. In contrast NT-proBNP was positively correlated to left ventricular parameters, LGE, and NYHA class function (P0.05). Additionally, it showed an inverse relationship to LVEF (P0.001, r = - 0.42).Soluble ST2 is not able to detect myocardial scar and should not be used alone as a parameter for detection of inflammation and myocardial scar formation.

Cardiac dysfunction occurs in several forms of limb girdle muscular dystrophy (LGMD). The aim of this study was to investigate cardiac involvement in calpainopathy (LGMD2A).Cardiovascular evaluation was performed in 10 patients with genetically verified LGMD2A by echocardiography, 3 Tesla - cardiovascular magnetic resonance, 24-h electrocardiography recordings with heart rate variability (HRV) analysis, and 24-h blood pressure recordings.No patient with calpainopathy showed impairment of left or right ventricular function. One patient had a small amount (2% of left ventricle mass) of late gadolinium enhancement. HRV analysis revealed no significant difference compared with external reference data.The main finding of this study is the lack of cardiac involvement in patients with calpainopathy. Cardiac involvement was not found, even in individuals with advanced age and greater disease severity. Furthermore, we did not observe an overall reduction of cardiac autonomic regulation in calpainopathy.