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To quantify heterogeneous tissue at the periphery of areas of fibrosis (gray zone) in patients with hypertrophic cardiomyopathy (HCM) with the use of two different techniques. Cardiac magnetic resonance with late gadolinium enhancement analysis was performed in 33 patients with HCM. Gray zone was evaluated with the use of two different techniques previously described in patients after myocardial infarction. LGE was present in 25 (78%) patients. There was no significant difference in total LGE mass at two different cut-off values [53.8 g (interquartile range, IQR 43.5-77.8 g) vs. 53.8 g (IQR 37.8-64.5 g), respectively, P = 0.49]. Significant difference in gray zone mass assessed with the use of two techniques was demonstrated (19.1 +/- 7.3 g vs. 50.8 +/- 47.8 g; P = 0.003). There was a strong correlation between total LGE and gray zone mass (r = 0.789, P = 0.0001 for first method and r = 0.951, P < 0.0001 for the second one, respectively). However, significant variability of gray zone mass (and extent expressed as % of left ventricular mass) in patients with similar LGE size/extent was observed. Moreover, LGE mass varied greatly in patients with similar gray zone size. Neither left ventricular mass, nor with maximal wall thickness correlated with extent of gray zone assessed with both methods. The studied techniques provided similar results with regard to total LGE but significant differences were observed in gray zone mass. Two patients may have similar extent (or absolute mass) of LGE, but strikingly discrepant gray zone size. [ABSTRACT FROM AUTHOR]

Background: Resolution of ST segment elevation corresponds with myocardial tissue reperfusion and correlates with clinical outcome after ST elevation myocardial infarction. Simpler method evaluating the extent of maximal deviation persisting in a single ECG lead was an even stronger mortality predictor. Our aim was to evaluate and compare prognostic accuracy of different methods of ST segment elevation resolution analysis after primary percutaneous coronary intervention (PCI) in a real-life setting.Methods: Paired 12-lead ECGs were analyzed in 324 consecutive and unselected patients treated routinely with primary PCI in a single high-volume center. ST segment resolution was quantified and categorized into complete, partial, or none, upon the (1) sum of multilead ST elevations (sumSTE) and (2) sum of ST elevations plus reciprocal depressions (sumSTE+D); or into the low-, medium-, and high-risk groups by (3) the single-lead extent of maximal postprocedural ST deviation (maxSTE).Results: Complete, partial, and nonresolution groups by sumSTE constituted 39%, 40%, and 21% of patients, respective groups by sumSTE+D comprised 40%, 39%, and 21%. The low-, medium-, and high-risk groups constituted 43%, 32%, and 25%. One-year mortality rates for rising risk groups by sumSTE were 4.7%, 10.2%, and 14.5% (P = 0.049), for sumSTE+D 3.8%, 9.6%, and 17.6% (P = 0.004) and for maxSTE 5.1%, 6.7%, and 18.5% (P = 0.001), respectively. After adjustment for multiple covariates only maxSTE (high vs low-risk, odds ratio [OR] 3.10; 95% confidence interval [CI] 1.11-8.63; P = 0.030) and age (OR 1.07; 95% CI 1.02-1.11; P = 0.002) remained independent predictors of mortality.Conclusions: In unselected population risk stratifications based on the postprocedural ST resolution analysis correlate with 1-year mortality after primary PCI. However, only the single-lead ST deviation analysis allows an independent mortality prediction. [ABSTRACT FROM AUTHOR]

Introduction: The most efficient risk stratification algorithms are expected to deliver robust and indefectible identification of high-risk children with hypertrophic cardiomyopathy (HCM). Here we compare algorithms for risk stratification in primary prevention in HCM children and investigate whether novel indices of biatrial performance improve these algorithms., Methods and Results: The endpoints were defined as sudden cardiac death, resuscitated cardiac arrest, or appropriate implantable cardioverter-defibrillator discharge. We examined the prognostic utility of classic American College of Cardiology/American Heart Association (ACC/AHA) risk factors, the novel HCM Risk-Kids score and the combination of these with indices of biatrial dynamics. The study consisted of 55 HCM children (mean age 12.5 ± 4.6 years, 69.1% males); seven had endpoints (four deaths, three appropriate ICD discharges). A strong trend (DeLong p = 0.08) was observed towards better endpoint identification performance of the HCM Risk-Kids Model compared to the ACC/AHA strategy. Adding the atrial conduit function component significantly improved the prediction capabilities of the AHA/ACC Model (DeLong p = 0.01) and HCM Risk-Kids algorithm (DeLong p = 0.04)., Conclusions: The new HCM Risk-Kids individualised algorithm and score was capable of identifying high-risk children with very good accuracy. The inclusion of one of the atrial dynamic indices improved both risk stratification strategies.

Objectives: To investigate biatrial mechanics and their relation with left ventricular outflow tract (LVOT) obstruction (LVOTO), the degree of hypertrophy, indices of ventricular diastolic function and fibrosis in children with hypertrophic cardiomyopathy (HCM)., Methods: Fifty-five consecutive, prospectively recruited children with HCM (mean age 12.5 ± 4.6 years, 69.1% male), 19 (34.5%) of whom had LVOTO, underwent cardiac magnetic resonance and echocardiography with quantification of phasic components of biatrial function, biventricular diastolic function and fibrosis. Twenty healthy, sex-matched subjects served as controls., Results: We found a significant increase of left atrial (LA) and right atrial (RA) volumes and reduction in the majority of indices of contractile function, strains and strain rates (p < 0.05) in children with HCM compared with controls. Nearly all of the LA dynamics markers attained a significant association with the LVOT gradient (p < 0.05), the RA volumes and contractile functions were affected by LV fibrosis and mass (p < 0.05), and the RA mechanical components were related to the degree of LVOTO (p < 0.05). The minority of biatrial dynamics markers were associated with indices of ventricular diastolic function., Conclusions: The majority of biatrial volumetric and functional indices were severely compromised in children with HCM compared with controls. The degree of LVOTO appears to trigger LA volumetric and LA and RA mechanical malfunction. On the other hand, the deterioration of RA volumetric components was linked to LV fibrosis and mass., Key Points: • Biatrial function was severely compromised in children with HCM. • Left atrial malfunction was associated with the degree of LVOTO. • Fibrosis and LV mass were related to RA volumetric and contractile dysfunction. • The degree of LVOTO was linked to right atrial mechanical abnormalities.

Objectives: To assess the magnitude of myocardial displacement abnormalities and their alterations with the fibrosis, left-ventricular (LV) outflow tract obstruction (LVOTO) and hypertrophy in juveniles with hypertrophic cardiomyopathy (HCM)., Study Design: Fifty-five children [age 12,5±4.6years, 38 (69,1%) males, 19 (34,5%) with LVOTO] with HCM and 20 controls underwent cardiovascular magnetic resonance. The LV feature tracking (FT) derived strain and strain rates were quantified. Results of FT analysis were compared between HCM subjects and controls and between children with and without LVOTO., Results: Children with HCM exhibited decreased strain in both hypertrophied and nonhypertrophied segments versus controls. LV global longitudinal strain (LVGLS) rate (-0.69±0.04 vs -0.91±0.05, p=0,04), LV circumferential strain (LVCR) rate (-0.98±0.09 vs -1.27±0.06, p=0,02), LV radial strain (LVR) (18,5±1.9 vs 27,4±1.4, p<0,01) and LVR rate (0,98±0.1 vs 1,53±0.08, p<0,01) were substantially compromised in subjects with LVOTO vs without. In multivariable regression all LV myocardial dynamics markers, except for LVCR, exhibited a significant association with the degree of LVOTO. LVCR rate (β=0,31, p=0,02) and LVR (β=-0.24, p=0,04) were related to LV mass and only LVCR rate (β=0,15, p=0,03) was associated with the amount of LV fibrosis., Conclusions: The reduction of all indices of LV myocardial mechanics in juvenile HCM patients was global but particularly pronounced in hypertrophied segments of the LV. The majority of the LV strains and strain rates were substantially compromised in subjects with LVOTO compared to patients without the obstruction. Myocardial mechanics indices seemed to be related to the degree of LVOTO rather than either to mass or the amount of fibrosis., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Purpose: The purpose of this study was to compare the ability of various parameters of myocardial mechanics to predict large amounts of biventricular fibrosis assessed via T1 mapping in patients with dilated cardiomyopathy (DCM)., Material: Cardiovascular magnetic resonance feature tracking analysis and T1 mapping were performed in 26 patients with DCM [mean age: 34.4±9.1years, 15 (57.6%) males]. The values of various parameters of myocardial mechanics at predicting advanced left-ventricle (LV) and right-ventricle (RV) fibrosis were compared using logistic regression analysis and receiver operating characteristic curve (ROC) analysis., Results: There were 7 (26.9%) patients with a large amount of LV fibrosis and 9 (34.6%) patients with severe RV fibrosis. ROC curve analysis revealed that the model of combined LV strain rates (AUC=0.902) offered superb ability at predicting large amounts of LV fibrosis. The models including RV strain rates (AUC=0.974), a combination of RV strains, strain rates and clinical parameters (AUC=0.993) as well as the RV radial strain rate alone model (AUC=0.961) yielded outstanding performance in discriminating large and small amounts of RV fibrosis. In multivariate analysis, the LV circumferential strain (LVCR) and RV radial (RVR) strain rate were the only independent predictors of large amounts of LV and RV fibrosis, respectively., Conclusions: Indices of myocardial deformation, especially combined with clinical features, offered a superlative ability to differentiate high from low degrees of fibrosis in DCM patients. Among all analyzed parameters of myocardial mechanics, LVCR and RVR rate alone were the independent predictors of high degrees of LV and RV fibrosis, respectively., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Introduction: In children with hypertrophic cardiomyopathy (HCM) there often occurs a non-ischemic pattern of myocardial fibrosis, which could be the cause of impaired left ventricular (LV) diastolic function assessed by tissue Doppler imaging (TDI). The aim of the study was to determine the prevalence of myocardial fibrosis in children with HCM, and to evaluate its relationship with echocardiographic parameters including LV diastolic dysfunction., Material and Methods: Sixty-three children with HCM, mean age 12.2 ±4.5 years, underwent magnetic resonance imaging (MRI) and echocardiographic study from January 2010 to April 2014. The results of MRI, echocardiography, and TDI velocities were analyzed and compared between children with and without myocardial fibrosis. Moreover, correlations between the results of echocardiography and MRI were assessed., Results: Our results showed a significant correlation between magnetic resonance and echocardiographic measurements of septal wall thickness, posterior wall thickness, LV mass and left atrial dimension. Children with myocardial fibrosis (60%) had a significantly thicker interventricular septum (21.3 vs. 1.8 mm; p < 0.0001) and larger left atrial dimension (36.7 vs. 27.8 mm; p = 0.0004) and volume index (42.0 vs. 26.6 ml/m²; p = 0.0011). Tissue Doppler imaging demonstrated significantly decreased lateral E' (9.02 vs. 13.53 cm/s; p < 0.0001) and septal E' (7.05 vs. 9.36 cm/s; p = 0.0082) velocities and a significantly increased transmitral lateral (10.34 vs. 6.68; p = 0.0091) and septal (13.1 vs. 9.8; p = 0.046) E/E' ratio in children with myocardial fibrosis., Conclusions: Myocardial fibrosis in children with hypertrophic cardiomyopathy was associated with markers for disease severity such as larger septum thickness, enlargement of the left atrium as well as impairment of left ventricular diastolic function. Tissue Doppler imaging is a helpful tool to detect the presence of left ventricular diastolic dysfunction in children with hypertrophic cardiomyopathy and myocardial fibrosis., Competing Interests: The authors declare no conflict of interest.

Background and Aim: We sought to search for factors associated with the magnitude of trabeculation by cardiac magnetic resonance, and evaluate the impact of trabeculations on outcomes in patients with dilated cardiomyopathy (DCM)., Methods: We evaluated clinical profiles and outcomes of 276 subjects with DCM (age: 33.2 ± 13.3 years, 160 males). Trabeculation was quantified as trabeculated/total myocardial mass ratio (TM/M). Subjects were stratified into three subgroups (A, B, and C) according to the tertiles of rising TM/M values (33% ranges). A group of 30 healthy subjects served as controls. Patients were prospectively followed-up in search of major adverse cardiovascular events for 2.4 years on average (range 0.2-3.9 years)., Results: Dilated cardiomyopathy patients had more trabeculation than controls (27.1 ± 16.9% vs. 17.3 ± 8.1, p < 0.01). Group C subjects had lowest N-terminal pro-B-type natriuretic peptide (NT-proBNP) (1445 [984-3843] vs. 873 [440-2633] vs. 529 [206-1221] pg/mL, p < 0.01), higher ejection fraction (23.9 ± 10.4 vs. 25.0 ± 9.2 vs. 32.4 ± 2.7%, p = 0.03), and lower left ventricular mass index (LVMI) (91.3 ± 21.5 vs. 74.3 ± 31.1 vs. 55.7 ± 23.2 g/m2, p < 0.01). They also had fewer areas of late gadolinium enhancement (69 [46.3%] vs. 31 [38.2%] vs. 15 [32.6%], p = 0.01). Male sex (b = 0.21, SE = 0.13; p = 0.01), LVMI (b = -0.32, SE = 0.08, p < 0.01) and NT-proBNP (b = -0.05, SE = 0.02, p = 0.02) were independently related to TM/M. The magnitude of trabeculation was not a predictor of major adverse cardiovascular events. Prognosis was impacted by left ventricular end-diastolic volume index only (HR 2.538, 95% CI -1.734-3.218, p < 0.01)., Conclusions: Trabeculation patterns relate to cardiac function and neurohormonal activation but not to survival.

Objectives: This study explored whether cardiac magnetic resonance (CMR) could help select patients who could benefit from revascularization by identifying inducible myocardial ischemia and viability in the perfusion territory of the artery with chronic total occlusion (CTO)., Background: The benefit of revascularization using percutaneous coronary intervention (PCI) in CTO is controversial. CMR offers incomparable left ventricular (LV) systolic function assessment in addition to potent ischemic burden quantification and reliable myocardial viability analysis. Whether CMR guided CTO revascularization would be helpful to such patients has not yet been explored fully., Methods: A prospective study of 50 consecutive CTO patients was conducted. Of 50 patients undergoing baseline stress CMR, 32 (64%) were selected for recanalization based on the presence of significant inducible perfusion deficit and myocardial viability within the CTO arterial territory. Patients were rescanned 3 months after successful CTO recanalization., Results: At baseline, myocardial perfusion reserve (MPR) in the CTO territory was significantly reduced compared with the remote region (1.8 ± 0.72 vs. 2.2 ± 0.7; p = 0.01). MPR in the CTO region improved significantly after PCI (to 2.3 ± 0.9; p = 0.02 vs. baseline) with complete or near-complete resolution of CTO related perfusion defect in 90% of patients. Remote territory MPR was unchanged after PCI (2.5 ± 1.2; p = NS vs. baseline). The LV ejection fraction increased from 63 ± 13% to 67 ± 12% (p < 0.0001) and end-systolic volume decreased from 65 ± 38 to 56 ± 38 ml (p < 0.001) 3 months after CTO PCI. Importantly, despite minimal post-procedural infarction due to distal embolization and side branch occlusion in 8 of 32 patients (25%), the total Seattle Angina Questionnaire score improved from a median of 54 (range 45 to 74) at baseline to 89 (range 77 to 98) after CTO recanalization (p < 0.0001)., Conclusions: In this small group of patients showing CMR evidence of significant myocardial inducible perfusion defect and viability, CTO recanalization reduces ischemic burden, favors reverse remodeling, and ameliorates quality of life., (Copyright © 2016 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

To date limited data are available to predict the progression to end-stage heart failure (HF) with subsequent death (non-SCD), need for heart transplantation, or sudden cardiac death (SCD) in children with hypertrophic cardiomyopathy (HCM). We aimed to determine predictors of long-term outcome in children with HCM. A total of 112 children (median 14.1, IQR 7.8-16.6 years) were followed up for the median of 6.5 years for the development of morbidity and mortality, including arrhythmic and HF-related secondary end points. HF end point included HF-related death or heart transplant, and arrhythmic end point included resuscitated cardiac arrest, appropriate ICD discharge, or SCD. Overall, 23 (21 %) patients reached the pre-defined composite primary end point. At 10-year follow-up, the event-free survival rate was 76 %. Thirteen patients (12 %) reached the secondary arrhythmic end point, and 10 patients (9 %) reached the secondary HF end point. In multivariate model, prior cardiac arrest (r = 0.658), QTc dispersion (r = 0.262), and NSVT (r = 0.217) were independent predictors of the arrhythmic secondary end point, while HF (r = 0.440), LV posterior wall thickness (r = 0.258), LA size (r = 0.389), and decreased early transmitral flow velocity (r = 0.202) were all independent predictors of the secondary HF end point. There are differences in the risk factors for SCD and for HF-related death in childhood HCM. Only prior cardiac arrest, QTc dispersion, and NSVT predicted arrhythmic outcome in patients aged <18 years. LA size, LV posterior wall thickness, and decreased early transmitral flow velocity were strong independent predictors of HF-related events.

Background: Impaired right ventricular (RV) mechanics is a common problem in patients after repair of tetralogy of Fallot (TOF). Moreover, also impaired left ventricular (LV) systolic function has been demonstrated in this population. There are no studies evaluating patients after TOF repair with impaired both RV and LV ejection fractions (RVEF, LVEF)., Aim: We hypothesized that a considerable group of patients with repaired TOF would demonstrate both RV and LV systolic function impairment. Accordingly, the purpose of our study was to characterize patients with biventricular dysfunction after TOF repair., Methods: Consecutive patients with repaired TOF undergoing cardiac magnetic resonance (n=146, mean age 26.4±8.2 years, age range: 13.6-51.3 years, 60.3% males, 54 patients [37.0%] with early correction and 92 [63.0%] with late correction)., Results: There were 31 patients (21.2% of the study population; 90.3% males) with biventricular dysfunction. Normal both RVEF and LVEF were observed in 65 individuals (44.5%). Neither the presence nor the extent of late gadolinium enhancement differed between patients with normal both RVEF and LVEF vs low both RVEF and LVEF. There were no differences in pulmonary regurgitation (PR) fraction, peak right ventricular outflow tract (RVOT) gradient, and the incidences of significant PR and RVOT obstruction between these groups (P=NS for all comparisons). Multivariate logistic regression revealed that the male sex and RVOT aneurysm/akinesia (only in patients repaired early) were associated with the presence of biventricular dysfunction., Conclusions: In patients with repaired TOF, : Male sex and RVOT aneurysm/akinesia were independently associated with biventricular dysfunction. Impaired both RVEF and LVEF were common in patients with repaired TOF, with the vast majority of males.

Background: Crypts or clefts in the left ventricular inferobasal myocardium have been detected by cardiovascular magnetic resonance (CMR), but the extent to which they represent prephenotypic markers of hypertrophic cardiomyopathy (HCM) or incidental structural variants remains controversial., Methods and Results: We examined retrospectively the routine vertical long-axis cines in 686 consecutive patients (48±20 years, 55% men) referred for CMR. Crypts were identified in 46 (6.7%), 17 being among patients (8.7% of 196) with otherwise normal CMR findings and without a known family history of HCM. Higher percentages were found in patients with HCM (16%), myocarditis (15%), and hypertension (14%) but without reaching statistical significance (P=0.12). Only 1 (5%) of 20 phenotype-negative HCM family members had a visible crypt. Relative to those without, patients with crypts had lower indexed left ventricular end-systolic volumes (P=0.042) and higher indexed left and right ventricular stroke volumes (P=0.007 and P=0.015) and ejection fractions (P=0.003 and P=0.021). Crypts tended to narrow in systole, varying slightly in size, shape- and number, without obvious group-related features., Conclusions: Single or paired inferobasal myocardial crypts were an occasional and by no means rare finding among patients referred for CMR without a pretest suspicion of HCM. This, together with similar previous findings in a cohort of healthy volunteers, supports their being regarded, in such individuals, as incidental variants of local myocardial structure, unlikely to require further investigation. However, a larger registry-type study may be justified to investigate the clinical implications of multiple crypts, especially if associated with HCM family history.

Introduction: Computed tomography coronary angiography (CTCA) is a diagnostic method used for exclusion of coronary artery disease. However, lower accuracy of CTCA in assessment of calcified lesions is a significant factor impeding applicability of CTCA for assessment of coronary atherosclerosis., Aim: To provide insight into lumen and calcium characteristics assessed with CTCA, we compared these parameters to the reference of intravascular ultrasound (IVUS)., Material and Methods: Two hundred and fifty-two calcified lesions within 97 arteries of 60 patients (19 women, age 63 ±10 years) underwent assessment with both 2 × 64 slice CT (Somatom Definition, Siemens) and IVUS (s5, Volcano Corp.). Coronary lumen and calcium dimensions within calcified lesions were assessed with CTCA and compared to the reference measurements made with IVUS., Results: On average CTCA underestimated mean lumen diameter (2.8 ±0.7 mm vs. 2.9 ±0.8 mm for IVUS), lumen area (6.4 ±3.4 mm(2) vs. 7.0 ±3.7 mm(2) for IVUS, p < 0.001) and total calcium arc (52 ±35° vs. 83 ±54°). However, analysis of tertiles of the examined parameters revealed that the mean lumen diameter, lumen area and calcium arc did not significantly differ between CTCA and IVUS within the smallest lumens (1(st) tertile of mean lumen diameter at 2.1 mm, and 1(st) tertile of lumen area at 3.7 mm(2)) and lowest calcium arc (mean of 40°)., Conclusions: Although, on average, CTCA underestimates lumen diameter and area as well as calcium arc within calcified lesions, the differences are not significant within the smallest vessels and calcium arcs. The low diagnostic accuracy of CTCA within calcified lesions may be attributed to high variance and not to systematic error of measurements.

Aim: This prospective study was conducted to evaluate the incidence and predictors of coronary artery disease (CAD) in relation to the low coronary artery calcium (CAC) score among patients with intermediate probability of CAD., Material and Methods: A total of 1132 consecutive patients were included in the analysis (58.7 ±10.9 years, 46.7% males). Coronary computed tomography (CCT) angiography was performed in a multi-detector computed tomography scanner. Coronary artery calcium score was calculated by the Agatston method. Obstructive CAD was defined as the presence of coronary artery stenosis ≥ 50% on CCT angiography., Results: Coronary artery disease was diagnosed in nearly one-fourth of patients (n = 272, 24%). In the receiver operating characteristics (ROC) curve analysis a CAC score of 10 was used as an optimal cut-off point for discriminating obstructive CAD (sensitivity: 0.79, specificity: 0.75, p < 0.0001) whereas for a CAC score of 100 the sensitivity and specificity were 0.48 and 0.92, respectively. On multivariate analysis after adjustment for age, gender, hypertension, hyperlipidemia, creatinine levels, only in patients with CAC score ≤ 10 age (OR = 1.05, 95% CI: 1.02-1.08, p = 0.0005, OR = 1.05, 95% CI: 1.03-1.08, p < 0.0001) and male gender (OR = 3.45, 95% CI: 1.92-6.22, p < 0.0001), likewise in group with CAC score ≤ 100 age (OR = 1.05, 95% CI: 1.03-1.08, p < 0.0001) and male gender (OR = 3.31, 95% CI: 1.88-5.81, p < 0.0001) were independent predictors of obstructive CAD., Conclusions: The cut-off point of 10 for CAC score determined patients with CAD with the best sensitivity and specificity. Therefore, a total CAC score < 10 should be classified as "low". In patients with a low CAC score obstructive high risk plaques prone to rupture are presented and are associated with increasing age and male gender.

Introduction: Current risk assessment concepts in ST-elevation myocardial infarction (STEMI) are suboptimal for guiding clinical management., Aim: To elaborate a composite risk management concept for STEMI, enhancing clinical decision making., Material and Methods: 1995 unselected, registry patients with STEMI treated with primary percutaneous coronary intervention (pPCI) (mean age 60.1 years, 72.1% men) were included in the study. The independent risk markers were grouped by means of factor analysis, and the appropriate hazards were identified., Results: In-hospital death was the primary outcome, observed in 95 (4.7%) patients. Independent predictors of mortality included age, leukocytosis, hyperglycemia, tachycardia, low blood pressure, impaired renal function, Killip > 1, anemia, and history of coronary disease. The factor analysis identified two significant clusters of risk markers: 1. age-anemia- impaired renal function, interpreted as the patient-related hazard; and 2. tachycardia-Killip > 1-hyperglycemia-leukocytosis, interpreted as the event-related (hemodynamic) hazard. The hazard levels (from low to high) were defined based on the number of respective risk markers. Patient-related hazard determined outcomes most significantly within the low hemodynamic hazard group., Conclusions: The dissection of the global risk into the combination of patient- and event-related (hemodynamic) hazards allows comprehensive assessment and management of several, often contradictory sources of risk in STEMI. The cohort of high-risk STEMI patients despite hemodynamically trivial infarction face the most suboptimal outcomes under the current invasive management strategy.

Introduction: There are inconsistent data regarding the factors affecting left ventricular ejection fraction (LVEF) and right ventricular ejection fraction (RVEF) in patients after tetralogy of Fallot (TOF) repair., Objectives: The aim of the study was to assess the determinants of LVEF and RVEF in a large cohort of patients with repaired TOF., Patients and Methods: The study comprised 122 patients with repaired TOF (median age, 24.2 years; interquartile range, 20.3-30.9; men, 60.6%) who had undergone cardiac magnetic resonance imaging study. Predictors of LVEF, RVEF, and RVEF corrected for shunting or regurgitations (cRVEF) were identified with the use of linear regression analyses., Results: There was a weak correlation between RVEF and LVEF (r = 0.39, P <0.0001). A multiple regression analysis revealed the following independent predictors of LVEF: positive predictor - RVEF (P = 0.0002); negative predictors - pulmonary regurgitation fraction (PRF, P = 0.01) and male sex (P = 0.001). RVEF was predicted independently by positive predictors such as LVEF (P <0.0001) and LV end‑diastolic volume (LVEDV, P = 0.04) and negative predictors such as right ventricular mass (P <0.0001) and number of previous cardiothoracic surgery interventions (P = 0.005). In the model predicting cRVEF, only left ventricular mass was a positive predictor of cRVEF (P <0.0001), while right ventricular mass (P <0.0001), PRF (P <0.0001), male sex (P <0.0001), and RV late gadolinium enhancement score (P = 0.008) were negative predictors of cRVEF., Conclusions: Because PRF was inversely and independently correlated with LVEF, and LVEDV showed a positive and independent correlation with RVEF, left ventricular disease (low LVEF and LVEDV due to left ventricular compression) may be used as a marker of the severity of right ventricular disease (pulmonary regurgitation severity and its consequences). Further studies are needed to evaluate the role of LVEF and LVEDV in supporting patient selection for pulmonary valve replacement.

Background: Previous studies have advocated quantifying pulmonary regurgitation (PR) by using PR volume (PRV) instead of commonly used PR fraction (PRF). However, physicians are not familiar with the use of PRV in clinical practice. The ratio of right ventricle (RV) volume to left ventricle volume (RV/LV) may better reflect the impact of PR on the heart than RV end-diastolic volume (RVEDV) alone. We aimed to compare the impact of PRV and PRF on RV size expressed as either the RV/LV ratio or RVEDV (mL/m(2))., Methods: Consecutive patients with repaired tetralogy of Fallot were included (n=53). PRV, PRF and ventricular volumes were measured with the use of cardiac magnetic resonance., Results: RVEDV was more closely correlated with PRV when compared with PRF (r=0.686, p<0.0001, and r=0.430, p=0.0014, respectively). On the other hand, both PRV and PRF showed a good correlation with the RV/LV ratio (r=0.691, p<0.0001, and r=0.685, p<0.0001, respectively). Receiver operating characteristic analysis showed that both measures of PR had similar ability to predict severe RV dilatation when the RV/LV ratio-based criterion was used, namely the RV/LV ratio>2.0 [area under the curve (AUC)(PRV)=0.770 vs AUC(PRF)=0.777, p=0.86]. Conversely, with the use of the RVEDV-based criterion (>170mL/m(2)), PRV proved to be superior over PRF (AUC(PRV)=0.770 vs AUC(PRF)=0.656, p=0.0028]., Conclusions: PRV and PRF have similar significance as measures of PR when the RV/LV ratio is used instead of RVEDV. The RV/LV ratio is a universal marker of RV dilatation independent of the method of PR quantification applied (PRF vs PRV)., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)

Purpose: To compare indexed right ventricular (RV) end-diastolic volume (RVEDVi) and the ratio of RV volume to left ventricular (LV) volume (RV/LV ratio) in prediction of significant pulmonary regurgitation (PR) after tetralogy of Fallot (TOF) repair and to assess sex differences in the RV/LV ratio., Materials and Methods: The ethics committee approved this retrospective single-center study, and patients or their parents or guardians signed written informed consent. RVEDVi, RV/LV ratio, and PR were measured with the use of magnetic resonance imaging in 155 consecutive patients with repaired TOF (mean age, 29.2 years±10.9 [standard deviation]; 98 [63.2%] male and 57 [36.8%] female patients). PR fraction of 20% or greater was considered significant. The capability of the RVEDVi and that of the RV/LV ratio for prediction of significant PR were compared by using logistic regression analysis and receiver operating characteristic curve analysis., Results: RVEDVi was significantly higher in male (162.8 mL/m2±50.4) than in female (138.2 mL/m2±37.5) patients (P=.001). Conversely, the RV/LV ratio was similar in both sexes (1.82±0.56 [male] vs 1.69±0.46 [female], P=.13) both in the entire cohort and after excluding patients with significant (≥30 mm Hg) RV outflow tract gradient and/or other residual hemodynamic abnormalities (P=.63). Receiver operating characteristic analysis revealed better discrimination of significant (≥20%) from insignificant (<20%) PR with the use of the RV/LV ratio than with RVEDVi (area under the receiver operating characteristic curve, 0.937 [model 4] vs 0.849 [model 1], P=.01). In multivariate analysis, the only independent predictor of PR fraction was the RV/LV ratio., Conclusion: The RV/LV ratio is more accurate than the RVEDVi in differentiation of significant from insignificant PR. After TOF repair, female and male patients have similar RV/LV ratios despite significant differences in RVEDVi between the sexes., (© RSNA, 2012.)

Purpose: To assess the value of dyssynchrony and myocardial viability assessment by cardiac magnetic resonance (CMR) in prediction of response to cardiac resynchronization therapy (CRT) in patients with heart failure (HF) of both ischaemic and non-ischaemic etiologies., Materials and Methods: Patients scheduled for CRT in NYHA class II-IV, left ventricular ejection fraction <35%, QRS ≥ 120 ms were included. Tagged cine and late gadolinium enhancement (LGE) images were performed. Dyssynchrony was assessed with inTag toolbox and LGE was quantified using cutoff value at half of maximal signal in the scar. Cardiopulmonary exercise test, echocardiography and blood testing for NT-proBNP levels were done at baseline and 6 months after CRT., Results: 52 patients (age 60.3 ± 13 years) were included. 26 patients (50%) met response criteria. The ischaemic etiology of HF was more frequent (69% vs. 31%, p=0.002), the percent of LGE was higher (7.7% [0-13.5%] vs. 19.0% (0-31.9%], p=0.013), regional vector of circumferential strain variance (RVV) was lower (0.27 ± 0.08 vs. 0.34 ± 0.09, p=0.009) and uniformity of radial strain was higher (0.72 ± 0.25 vs. 0.56 ± 0.29, p=0.046) in non-responders vs. responders. Multivariate logistic regression showed that RVV predicted response to CRT (HR 2.3, 95% CI 1.02-5.02, p=0.0430) independently of LGE and the etiology of heart failure. In the subgroup of patients with ischaemic HF the extend of transmural scar within myocardium was higher in non-responders vs. responders (26.3% vs. 15.0% respectively, p=0.01) and was a predictor of response to CRT in univariable analysis (HR 0.87, 95% CI 0.77-0.98, p=0.025) providing the sensitivity of 76% and specificity of 75% at the cutoff point of 18% in the prediction of poor response to CRT. In patients with non-ischaemic HF QRS was wider (162 ms vs. 140 ms, p=0.04), regional vector of strain variance (RVV) was higher (0.39 vs. 0.25, p=0.002) and uniformity of radial strain was lower (0.52 vs. 0.80, p=0.049) in non-responders vs. responders. Univariable logistic regression showed that RVV was a predictor of response to CRT (HR 1.50, 95% CI 1.06-2.13, p=0.022), providing the sensitivity of 94% and specificity of 85% at the cutoff point of 0.31., Conclusions: CMR derived parameters of dyssynchrony such as RVV may provide an additive value in prediction of response to CRT, especially in patients with non-ischaemic etiology of heart failure. In patients with ischaemic HF the transmurality of LGE is an important predictor of lack of response to CRT., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

Background: Left ventricular hypertrophy (LVH) predisposes to larger infarct size, which may be underestimated by the left ventricular ejection fraction (LVEF) due to supranormal systolic performance often present in patients with LVH. The aim of the study was to compare infarct size and LVEF in patients with ST-segment elevation myocardial infarction (STEMI) and increased left ventricular mass on cardiac magnetic resonance (CMR)., Methods: The study included unselected group of 52 patients (61±11 years, 69% male) with first STEMI who had CMR after median 5 days from the onset of the event. Left ventricular hypertrophy (LVH) was defined as left ventricular mass index exceeding 95th percentile of references values for age and gender. Infarct size was assessed with means of late gadolinium enhancement (LGE)., Results: LVH was found in 16 patients (31%). In comparison to the rest of the group, patients with LVH had higher absolute and relative infarct mass (p=0.002 and p=0.02, respectively). LVH was related to higher prevalence of microvascular obstruction and myocardial haemorrhage and higher number of LV segments with transmural necrosis (p=0.02, p=0.01 and p=0.01, respectively). Despite marked difference in the infarct size between both studied subgroups there was no difference in LVEF and mean number of dysfunctional LV segments., Conclusions: Patients with LVH undergoing STEMI have larger infarct size underestimated by the LV systolic performance in comparison to patients without LVH., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

Background: There are concerns whether there is a difference in clinical utility of pulmonary regurgitation (PR) fraction (PRF) and PR volume (PRV) in subgroups of patients with isolated PR and individuals with combined PR and right ventricular outflow tract obstruction (RVOTO). The aim of the study was to compare PRF and PRV in patients with or without RVOTO., Methods and Results: 82 consecutive patients after repair of tetralogy of Fallot (TOF) who underwent cardiovascular magnetic resonance and echocardiography were studied. There was no difference in PRF between patients with moderate and severe right ventricular (RV) dilatation (32±13% vs. 37±12%; p=0.18). Significant difference in PRV was observed between these groups (23±10 ml/m2 vs. 31±12 ml/m2, respectively; p=0.02). PRV had better ability than PRF in identification of severe RV dilatation, both in group with RVOTO [area under the curve (AUC) 0.82 vs. 0.72, p=0.005] and in patients without RVOTO (AUC 0.83 vs. 0.77, p=0.04). A strong correlation was seen between PRF and PRV both in patients with and without RVOTO [r=0.93, p<0.0001 and r=0.92, p<0.0001, respectively]. In both subgroups high variability of PRF was found in subjects with similar degree of PRV., Conclusions: PRV shows better ability than PRF in evaluating influence of PR on RV in patients after TOF repair, both in population with and without concomitant RVOTO., (Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.)

Purpose: To compare right ventricular (RV) size and function between patients with combined pulmonary regurgitation (PR) plus RV outflow tract (RVOT) obstruction (RVOTO) and patients with isolated PR., Materials and Methods: Consecutive individuals with significant PR (PR fraction ≥ 20%) after tetralogy of Fallot (TOF) repair who underwent cardiovascular magnetic resonance (CMR) were included. Patients with additional hemodynamic abnormalities (residual ventricular septal defect, extracardiac shunt, and/or more than mild regurgitation at a valve other than the pulmonary valve) were excluded. Significant RVOTO was defined as peak gradient across RVOT ≥ 30 mmHg., Results: Significant differences between patients with combined PR+RVOTO (n = 9) and isolated PR (n = 33) were observed in RV end-diastolic volume (138.6 ± 25.1 vs. 167.0 ± 34.6 mL/m(2) , P = 0.02, respectively), RV end-systolic volume (65.0 ± 9.6 vs. 92.7 ± 26.2 mL/m(2) , P = 0.003), and RV ejection fraction (RVEF) (52.8 ± 3.7 vs. 45.0 ± 6.4%, P = 0.001). Both PR and peak RVOT gradient were independent predictors of RV size., Conclusion: Patients with combined PR+RVOTO had smaller RV volumes and higher RVEF when compared with patients with isolated PR. The confounding effect of RVOTO on RV size and function needs to be considered in CMR studies evaluating patients after TOF repair., (Copyright © 2011 Wiley-Liss, Inc.)

Fabry's disease (FD) is a rare hereditary disorder caused by the loss of alpha galactosidase A activity leading to accumulation of glycosphingolipids in various organs including hypertrophy of the heart. Most reports on cardiac involvement in FD focus on the left ventricular hypertrophy (LVH) and its relation to diastolic function. However, recent studies demonstrated large subset of patients with FD and right ventricle (RV) hypertophy. The accurate depiction of RV volumes, function and mass is possible with cardiovascular magnetic resonance (CMR). The CMR study can be also used to identify typically localised regions of intramyocardial fibrosis (infero-lateral segments of the LV), which have been shown to be a marker of inefficacious response to enzyme replacement therapy. We present series of 8 patients with genetically confirmed FD who underwent CMR study. We demonstrated a typical concentric and diffuse pattern of LVH with RV involvement in patients with the most severe LVH without significant impact on RV function and volumes. We showed that myocardial fibrosis can be observed not only in LV but also in RV. In 2 patients FD coexisted with symptomatic coronary artery disease with evidence of subendocardial myocardial fibrosis typical for ischaemic origin in one patient. The CMR confirmation of the presence of FD in one patient at an early stage of the disease, before the onset of advanced hypertrophy or failure of other organs, supports the value of this imaging technique in differential diagnosis of concentric and diffuse LVH.

Background: Cardiovascular magnetic resonance enables accurate and reproducible assessment of left ventricular (LV) dimensions and function, free of geometric assumptions and limitations related to an inadequate acoustic window. In patients with hypertrophic cardiomyopathy (HCM), LV mass (LVM) and maximal LV wall thickness (MLVWT) have prognostic significance., Aim: To compare MLVWT and LVM in patients with HCM., Methods: The study population included 33 patients with HCM (17 males, mean age 48.5 +/- 16.5 years). Subjects after alcohol septal ablation or surgical myectomy were excluded from the study. The MLVWT and LVM were measured with the use of cardiac magnetic resonance. The MLVWT was determined with the use of the dedicated software in short axis slices after manual definition of endocardial and epicardial contours. The LVM was indexed for body surface area and expressed in g/m(2). Cut-off values for normal, mildly increased and markedly increased LVM were based on previously published studies., Results: Mean LVM in the whole study group was 107.4 +/- 30.9 g/m(2) (range 57.0-163.4 g/m(2)) and was higher in males than females (120.2 +/- 30.8 g/m(2) vs 93.8 +/- 25.3 g/m(2), respectively; p = 0.01). Mean MLVWT was 23.4 +/- 4.8 mm (range 16-36 mm). There was only a weak trend toward higher MLVWT in men when compared to women (24.8 +/- 5.4 mm vs 21.9 +/- 3.7 mm, respectively; p = 0.09). There was no correlation between LVM and MLVWT (r = 0.24; p = 0.17). A significant variability in LVM was observed in subjects with similar MLVWT; a greater than two-fold difference was noted in extreme cases. In three patients (9%; one female, two male) LVM was within the normal range and in another one female (3%) patient LVM was mildly increased. In the remaining patients (n = 29; 88%) markedly increased LVM was observed., Conclusions: The MLVWT does not reflect the degree of LV hypertrophy in patients with HCM. Patients with similar MLVWT may have substantial differences in LVM. A substantial group of patients with HCM is characterised by normal, or only mildly increased LVM, despite significant LV wall hypertrophy measured as MLVWT.

Aim: There is no consensus regarding the technique of quantification of late gadolinium enhancement (LGE). The aim of the study was to compare different methods of LGE quantification in patients with hypertrophic cardiomyopathy (HCM)., Methods: Cardiac magnetic resonance was performed in 33 patients with HCM. First, LGE was quantified by visual assessment by the team of experienced readers and compared with different thresholding techniques: from 1SD to 6SD above mean signal intensity (SI) of remote myocardium, above 50% of maximal SI of the enhanced area (full-width at half maximum, FWHM) and above peak SI of remote myocardium., Results: LGE was present in 25 (78%) of patients. The median mass of LGE varied greatly depending on the quantification method used and was highest with the utilization of 1SD threshold [75.5 g, interquartile range (IQR): 63.3-112.3g] and lowest for FWHM method (8.4 g, IQR: 4.3-13.3g). There was no difference in mass of LGE as assessed with 6SD threshold and FWHM when compared to visual assessment (p=0.19 and p=0.1, respectively); all other thresholding techniques provided significant differences in the median LGE size when compared to visual analysis. Results for all thresholds, except FWHM were significantly correlated with visual assessment with the strongest correlation for 6SD (rho=0.956, p<0.0001)., Conclusions: LGE quantification with the use of a threshold of 6SD above the mean SI of the remote myocardium provided the best agreement with visual assessment in patients with HCM., (Copyright (c) 2009 Elsevier Ireland Ltd. All rights reserved.)

Objective: To examine the incidence and inter-relationships between admission hyperglycemia, anemia and impaired renal function and its impact on clinical outcomes in patients with ST-elevation myocardial infarction (STEMI) treated with primary PCI., Methods: The study group comprised 1880 patients with STEMI treated with primary PCI, enrolled in a prospective registry., Results: The primary endpoint of in-hospital death occurred in 88 (4.7%) patients. Hyperglycemia (glucose >11.1mmol/L) was present in 352(18.7%), anemia (hematocrit <36% women, <39% men) in 396(21.1%), and increased serum creatinine (> or =1.2mg/dL women, > or =1.3mg/dL men) in 423(22.5%) patients. 1026(54.6%) subjects had none of the triad risk factors. Two overlapping conditions were observed in 207(11%) and 3 in 40(2.1%) patients. Compared to the expected distribution, an increased prevalence was observed in patients with zero, two or three risk factors, and decreased prevalence was present in patients with one risk factor (p<0.001). In multivariable model including important baseline risk factors and the whole triad risk factors, hyperglycemia, anemia, and increased serum creatinine were independently associated with the primary outcome (hazard ratio (HR); 95% confidence interval (CI): 2.67; 1.56-4.55, and 2.03; 1.19-3.46, and 1.72;1.01-2.93, respectively). Adjusted HR (95% CI) for the incidence of the primary outcome associated with 1, 2 and 3 examined risk factors as compared to 0 of the risk factors was 2.7(1.4-5.4), 5.4(2.6-8.3) and 8.3(3.0-23.2), respectively., Conclusions: Hyperglycemia, anemia, and impaired renal function are independently of each other related to in-hospital death in patients with STEMI treated with primary PCI. The triad risk factors cluster and accumulation of these risk factors is related to stepwise, additive increase of risk of in-hospital mortality., (Copyright 2009 Elsevier Ireland Ltd. All rights reserved.)

Background: Hemoglobin (Hb) levels may interact with inflammatory activation, but it is unknown whether the interaction has any impact on clinical outcomes in acute coronary syndromes. The aim of this study was to assess the relationship between admission Hb levels, leukocytosis and clinical outcomes of ST-elevation myocardial infarction (STEMI) treated with primary angioplasty. Methods and Results The study group comprised 1,904 (1,380 men) patients with STEMI treated with primary percutaneous coronary intervention, enrolled in a prospective registry. The primary endpoint of in-hospital death occurred in 90 (4.7%) patients. According to univariate analysis, extreme values of Hb (for 1(st) and 5(th) vs mid quintiles respectively: hazard ratio (HR) =7.1, P<0.001 and HR =3.2, P=0.024) and leukocytosis above median (HR =2.09, P=0.001) significantly correlated with in-hospital death. After dividing patients into high and low white blood cell (WBC) count groups, a U-shaped relationship of Hb levels and mortality was observed for patients with higher leukocytosis (1(st) and 5(th) vs mid quintiles respectively: HR =8.1, P=0.001 and HR =4.4, P=0.022), whereas in patients with lower WBC count higher mortality was related solely to the lowest Hb quintile (HR =6.9, P=0.010 vs mid quintile). Conclusion Higher mortality associated with higher Hb levels in STEMI patients treated with primary angioplasty is limited to patients with increased leukocytosis.

Background: TIMI scale is commonly used for angiographic assessment of reperfusion effectiveness and early risk stratification in patients treated with primary angioplasty for ST-elevation myocardial infarction (STEMI). Since ST-resolution analysis allows a noninvasive insight into the reperfusion status at the myocardial tissue level, it may be a better predictor of outcome after primary angioplasty., Aim: To compare the prognostic value of the reperfusion effectiveness evaluation based on either the epicardial blood flow assessment according to the TIMI scale, or ST-segment resolution analysis in patients treated with primary coronary angioplasty for STEMI., Methods: 324 consecutive patients treated within 12 hours from the pain onset were studied. Based on the analysis of maximal ST-segment elevation/depression identified in a single ECG lead recorded after the procedure (maxSTE), patients were classified into groups of high versus medium/low risk. Independently, distinguished were groups with restored normal (TIMI 3) and abnormal (TIMI 0-2) final blood flow in infarct related artery., Results: The 30-day and one-year mortality rates were higher in the high-risk maxSTE group (25% of all patients) than in the other patients (14.8% vs. 2.5%, p<0.001 and 18.5% vs. 5.4%, p<0.001 respectively). In subjects (82%) with restored TIMI grade 3 blood flow, mortality at one-month and one-year was lower than in the group with abnormal final blood flow (3.1% vs. 15.6%, p=0.001 and 6.2% vs. 18.8%, p=0.005). Comparison in multivariate analysis revealed that maxSTE stratification but not final TIMI grade assessment remained an independent predictor of both, 30-day and one-year mortality (high vs. medium/low-risk category; OR 5.3, 95% CI 1.6-16.7, p=0.005, and OR 3.3, 95% CI 1.4-7.8, p=0.007, respectively). Furthermore, maxSTE proved to stratify the risk of death even in subgroup of patients with restored normal blood flow (OR 6.2, 95% CI 1.4-27.8, p=0.016, and OR 3.0, 95% CI 1.1-8.7, p=0.039, respectively)., Conclusions: Analysis of extent of maximal ST-segment elevation or depression identified in a single ECG lead after primary coronary angioplasty allows better prognosis of subsequent 30-day and one-year mortality than the assessment of final epicardial blood flow, stratifying risk of death even in a subgroup of patients with restored normal blood flow.

A collection of seven new 23,24-dihydrodiscodermolide analogues have been synthesized with modifications to the lactone ring, some of which show antiproliferative activities similar to discodermolide.

A series of 7-deoxy discodermolide analogues in which the lactone fragment 'C' was replaced by aryl substituents were designed, synthesized, and evaluated for cytotoxicity.