Biatrial performance in children with hypertrophic cardiomyopathy: CMR study.

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.

Predictors of Long-Term Outcome in Children with Hypertrophic Cardiomyopathy.

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.

Repaired tetralogy of Fallot: ratio of right ventricular volume to left ventricular volume as a marker of right ventricular dilatation.

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.

Right ventricular outflow tract obstruction as a confounding factor in the assessment of the impact of pulmonary regurgitation on the right ventricular size and function in patients after repair of tetralogy of Fallot.

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.

The Indices of Cardiovascular Magnetic Resonance Derived Atrial Dynamics May Improve the Contemporary Risk Stratification Algorithms in Children with Hypertrophic Cardiomyopathy.

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.

Comparison between maximal left ventricular wall thickness and left ventricular mass in patients with hypertrophic cardiomyopathy.

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.

Cardiac magnetic resonance imaging in patients with Fabry's disease.

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.

Comparison of prognostic value of epicardial blood flow and early ST-segment resolution after primary coronary angioplasty. ANIN--Myocardial Infarction Registry.

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.

Comparison of echocardiography with tissue Doppler imaging and magnetic resonance imaging with delayed enhancement in the assessment of children with hypertrophic cardiomyopathy.

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.

Clinical and prognostic relevancy of left ventricular trabeculation assessed by cardiac magnetic resonance in patients with dilated cardiomyopathy.

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.

Biventricular mechanics in prediction of severe myocardial fibrosis in patients with dilated cardiomyopathy: CMR study.

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.

Left-ventricular mechanics in children with hypertrophic cardiomyopathy. CMR study.

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 (ß=0,31, p=0,02) and LVR (ß=-0.24, p=0,04) were related to LV mass and only LVCR rate (ß=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.

CMR Guidance for Recanalization of Coronary Chronic Total Occlusion.

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.

Prevalence of inferobasal myocardial crypts among patients referred for cardiovascular magnetic resonance.

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.

Factors associated with biventricular dysfunction in patients with repaired tetralogy of Fallot.

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.

What is the optimal cut-off point for low coronary artery calcium score assessed by computed tomography? Multi-Detector Computed Tomography ANIN Registry.

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.

Determinants of left- and right­ventricular ejection fractions in patients with repaired tetralogy of Fallot: a cardiac magnetic resonance imaging study.

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 end­diastolic 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.

Risk is not flat. Comprehensive approach to multidimensional risk management in ST-elevation myocardial infarction treated with primary angioplasty (ANIN STEMI Registry).

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.

Lumen and calcium characteristics within calcified coronary lesions. Comparison of computed tomography coronary angiography versus intravascular ultrasound.

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.