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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]

Introduction: In patients with repaired tetralogy of Fallot (TOF), various pathologies of the vascular system (both arterial and venous) may be present as a result of the previous therapeutic procedures or due to the congenital disease itself. Because of the limited diagnostic capabilities in the past, lacking surgical reports of patients operated on several decades ago and/or a long time since a corrective procedure, some of these pathologies/anomalies may remain unknown., Aim: To identify selected vascular pathologies with the use of cardiac magnetic resonance in patients after TOF repair., Material and Methods: We included 208 consecutive patients (median age 24.9 years, interquartile range 20.5-36.7; 126 (60.6%) males) with repaired TOF undergoing cardiac magnetic resonance (CMR) imaging., Results: Significant unexpected vascular pathologies/anomalies were found in 30 patients (14.4%) and included: uni- or bilateral occlusion of the subclavian artery (n = 20), persistent left superior vena cava (n = 7, in 1 case draining into the left atrium), occluded subclavian vein (n = 1), and interruption of the inferior vena cava (n = 2). Additionally, 1 patient with the left subclavian artery occluded had an occlusion of the brachiocephalic vein. In none of the patients was the information about the uncovered pathology/anomaly present either in the referral information or in the present medical history., Conclusions: The CMR in patients with repaired TOF may uncover some pathologies/anomalies which were unknown or forgotten at the time of patients' referral for the study, and which may have a significant impact on patient management.

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: The results of clinical trials assessing the effect of late opening of infarct-related artery (IRA) on left ventricular ejection fraction (LVEF) and size in stable patients are equivocal, which may be related to the fact that the presence of viability was not a requirement for randomization in these trials. The aim of the study was to assess the influence of late percutaneous coronary intervention (PCI) with optimal medical therapy (OMT) vs. OMT alone on cardiac function and remodeling in patients presenting infarct zone with preserved viability on cardiovascular magnetic resonance (CMR)., Methods: The analysis included pooled data of 43 patients from 3 randomized studies. All patients underwent CMR before randomization, but only in 1 previously unpublished study was preserved viability required for randomization to treatment. Follow-up CMR was performed after 6-12 months., Results: Late PCI with OMT led to improved LVEF (+5 ± 7% vs. -1 ± 6%, p = 0.005), decreased left ventricular end-systolic volume (-11 ± 19 mL vs. 12 ± 40 mL, p = 0.02) and a trend towards a decrease in end-diastolic volume (-7 ± 27 mL vs. 15 ± 47 mL, p = 0.07) in comparison to OMT alone. Increased LVEF and decreased left ventricular volumes were observed after the analysis was restricted to patients with left anterior descending artery (LAD) occlusion., Conclusions: In patients with the presence of infarct zone viability, OMT with late PCI for an occluded IRA (particularly LAD) is associated with improvement of left ventricular systolic function and size over OMT alone.

Introduction: Adrenomedullin (ADM) is a vasopeptide with multiple actions in the cardiovascular system and a potentially powerful tool in comparison to some of the well-established unimodal biomarkers of risk stratification in myocardial infarction (MI). Previous studies on ADM in acute MI were based on single assessment. Therefore the aim of the study was to examine the relation between ADM plasma concentrations assessed at different time points following MI and outcomes., Material and Methods: The study included 127 patients with acute MI treated with percutaneous coronary intervention and 60 healthy individuals as controls. Adrenomedullin concentration was assessed at baseline in all study subjects and 48 h after admission in patients with MI. The primary endpoint consisted of all-cause death, nonfatal myocardial infarction, stroke and the need of target vessel revascularization at 6-month follow-up., Results: Mean ADM plasma concentration on admission was higher in patients with MI than in controls (30.3 ±14.3 pmol/l vs. 14.6 ±4.7 pmol/l, p < 0.0001). There was no significant difference between ADM concentration after 48 h (30.6 ±12.3 pmol/l) and on admission. The primary endpoint occurred in 9.4% of patients with MI. Multivariable analysis showed that ADM concentration at 48 h after admission (OR = 2.121, 95% CI 1.180-3.810 for every increase of 10 pmol/l, p = 0.012) was the only independent predictor of the primary endpoint., Conclusions: In patients with acute MI adrenomedullin plasma concentration assessed at 48 h after admission, but not ADM concentration at baseline, is an independent predictor of major adverse cardiovascular events at mid-term follow-up.

Background: Common variants of the renin-angiotensin system (RAS) genes have been linked to a higher risk of coronary artery disease (CAD) and its complications., Aim: To determine the prognostic significance of a combination of three common polymorphisms of RAS genes (angiotensin converting enzyme - ACE Ins/Del, angiotensin receptor type 1 - AGT1R A1166C and angiotensinogen - ATG M235T) in patients with CAD., Methods: The study included 216 patients (mean age 58 ± 9 years, 74% male) prospectively followed for a mean 41 ± 17 months. The end-points were all-cause mortality, myocardial infarction, stroke or the need for coronary revascularisation., Results: An end-point occurred in 41 (19%) patients. None of the polymorphisms analysed separately was associated with the end-point. Odds ratios were calculated for different combinations of analysed alleles to determine their relation to outcomes. Based on the cut-off points of odds ratios, the study group was divided into three subgroups: 55 patients without ATG 235T allele (T- subgroup); 100 patients with ATG 235T allele alone or ATG 235T allele combined with ACE Del allele or AGT1R 1166C allele (T+ or T+1 subgroup); and 61 patients with all three variants (T+2 subgroup). Multivariate analysis showed that the only independent predictor of the endpoint was an increasing number of variant genes (HR = 2.6, 95% CI 1.4-4.9, p = 0.002)., Conclusions: Co-existing angiotensinogen M235T AGT polymorphism and two other common polymorphisms of the RAS genes are related to adverse events in patients with CAD.

Mutations in the lamin A/C gene (LMNA) are established causes of familial dilated cardiomyopathy (DCM) with atrio-ventricular block although relatively little is known about genotype-phenotype correlations. We describe a 23-year-old patient who presented with inferolateral wall thinning and akinesis with evidence of mid-myocardial fibrosis on cardiac magnetic resonance. Molecular analysis driven by clinical similarities with a previously described case harboring the p.R541C LMNA mutation revealed a novel c.1621 C > G, p.R541G substitution whose pathogenicity was confirmed by transfection of mouse myoblasts. Our results emphasize the role of LMNA mutations at position R541 in DCM cases with segmental LV wall motion akinesis/dyskinesis.

Background: Complete revascularisation (CR) by means of percutaneous coronary intervention (PCI) has been associated with better long-term prognosis than incomplete revascularisation (IR) in several clinical trials. However, in the published studies, the completeness of myocardial revascularisation has been judged mainly on an anatomical basis, while including criteria directed at functionally driven IR might lead to different results., Aim: To examine the potential value of functionally driven IR in a large cohort of patients with multivessel coronary artery disease (MVD) undergoing PCI., Methods: The study population consisted of 908 patients with MVD undergoing PCI without stenting between 1988 and 1997. Functionally driven IR was defined as dilation of all segments with >70% stenosis, with the exception of arteries supplying an area of previous transmural myocardial infarction (MI) or a small amount of myocardium. Complete revascularisation was defined as successful PCI of all coronary artery lesions with significant narrowing not fulfilling the above criteria. Patients were followed for a mean 11 years (range 8-16 years). End-points included: death, MI, re-PCI or coronary artery bypass grafting (CABG)., Results: Complete revascularisation was performed in 284 (31.3%) patients. Follow-up was obtained from 873 (96.1%) patients. There was no significant difference in the frequency of all-cause mortality, cardiovascular deaths or MI between patients who underwent CR and IR. Patients who underwent IR were more likely to require re-PCI and had a trend toward more frequent CABG., Conclusions: In comparison to CR, a strategy of functionally driven IR by means of PCI without stenting does not increase the rate of major cardiovascular outcomes, but is related to higher frequency of repeat procedures during a long-term follow-up.

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.

Objectives: To determine whether the 681 G>A (*2) polymorphism of cytochrome P450 (CYP2C19) is related to suboptimal reperfusion and mortality in patients with acute myocardial infarction (AMI) pretreated with clopidogrel., Methods: The study included 276 consecutive patients with AMI in whom percutaneous coronary intervention (PCI) with stenting was attempted. Four-year follow-up for all-cause mortality was obtained., Results: There were 15 failed procedures (5.4%). In the remaining 261 patients, suboptimal reperfusion (post-PCI TIMI flow <3) was observed in 12.6% of the cases. There were 56 carriers (50 heterozygous and 6 homozygous) of CYP2C19*2. The prevalence of carriers in patients with suboptimal flow was 39.4% in comparison to 18.9% in the other patients (p = 0.01). Independent predictors of suboptimal reperfusion were initial TIMI flow ≤1 (OR = 5.9, 95% CI 2.2-16.2, p = 0.001) and CYP2C19*2 (OR = 2.9, 95% CI 1.3-6.6, p = 0.01). Thirty patients died during follow-up (11.5%). Four-year mortality tended to be higher in carriers of CYP2C19*2 (17.9%) versus non-carriers (9.8%; p = 0.09), but the only independent predictors of death were age (HR = 2.0, 95% CI = 1.4-2.8, p = 0.0001) and suboptimal reperfusion (HR = 3.6, 95% CI 1.5-8.8, p = 0.004)., Conclusions: The CYP2C19*2 allele is an independent predictor of suboptimal reperfusion in patients with AMI undergoing PCI with stenting after pretreatment with clopidogrel and may increase the risk of all-cause mortality., (Copyright © 2010 S. Karger AG, Basel.)

Management of cardiac tumours depends mainly on their localisation and histological type. Cardiac magnetic resonance (CMR) imaging is a robust technique in diagnosis of cardiac tumors. It enables precise localisation of a mass and its tissue characteristics. We present a case of a lipomatous hypertrophy of the interatrial septum diagnosed by CMR.

Background: In patients with non-obstructive hypertrophic cardiomyopathy (HCM) and preserved left ventricular (LV) systolic function, diastolic dysfunction is one of the major factors contributing to limited exercise capacity. Cardiovascular magnetic resonance (CMR) imaging has become a useful tool in diagnosis, risk stratification and treatment monitoring in patients with HCM., Aim: To assess the relationship between simple CMR parameters of LV diastolic function at rest and exercise capacity measured by means of cardiopulmonary exercise testing on a treadmill in patients with non-obstructive HCM and preserved LV systolic function., Methods: The study included 13 patients with non-obstructive HCM and preserved LV systolic function who underwent cardiopulmonary exercise testing on a treadmill and CMR within 1 month. Analysed parameters of diastolic function included: LV mass index (LVMI), peak filling rate normalised to LV stroke volume index (PFR/LVSVI) and time from the end-systole to PFR normalised to heart rhythm (TPFR)., Results: There was a significant correlation between PFR/LVSVI at rest and peak oxygen uptake (V02peak) (r=0.64, p=0.02). Patients with V02peak below median (<30 ml/kg/min) had a significantly lower PFR/LVSVI than patients with higher V02peak [5.12 m2/s, interquartile range (IQR) 4.16-6.82 vs. 7.93 m2/s, IQR 7.49-8.21 respectively, p=0.035]. LVMI, TPFR were not related to exercise capacity. There was also no correlation between V02peak and age (r=-0.38, p=0.19), LV ejection fraction (r=-0.36, p=0.22) or normalised LV volume indices: LVEDVI (r=0.09, p=0.76), LVESVI (r=0.34, p=0.26)., Conclusions: Assessment of LV diastolic function by peak filling rate normalised to stroke volume index by means of CMR at rest in patients with non-obstructive HCM and preserved LV systolic function is a useful marker of exercise capacity.

The aim of our study was to analyze electrocadiographic changes in patients with acute myocardial infarction related to the occlusion of diagonal (DG) or marginal (MG) branch. We selected 13 cases with DG and 12 with MG occlusion on angiography and evaluated their electrocardiogram (ECG) patterns on admission obtained in emergency department (ED) of university hospital with catheterization laboratory serving everyday interventional cardiology duty for ACS. Most characteristic ECG changes in acute occlusion of DG observed in 12 patients (92.3%) included ST-segment elevation in leads V(2) and V(3) (mean, 1.2 +/- 0.5 mm; maximum, 1.7 mm) and ST-segment depression in leads II and III (mean, 0.9 +/- 0.4 mm; maximum, 1.5 mm). Most characteristic ECG changes for acute occlusion of MG were ST-segment depression in leads V(5) and V(6) (mean, 0.9 +/- 0.4 mm; maximal, 1 mm) observed in 11 (91.7%) patients, ST-segment depression in lead II (mean, 0.7 +/- 0.2 mm; maximal, 0.8 mm) in 10 (83.3%,) and in leads V(2) and V(3), and aVF in 8 (66.7%) of cases. Risk of complications including cardiogenic shock and death was high in both groups especially during acute phase of myocardial infarction. Prevalence of borderline ECG changes in patients with acute coronary occlusion confirms how important is precise ECG interpretation usually initially done by ED physician.