We assessed segmental Ecc and scar using CMR with myocardial tissue tagging and late gadolinium enhancement (LGE) in patients with DMD, then compared CMR with echocardiographic velocity vector imaging (VVI) for regional Ecc based on independent observer assessments.The Participants enrolled  had median left ventricular (LV) ejection fraction of 0.52 (range 0.28-0.69), and 156 basal and mid-cavity myocardial segments from the 13 patients completing the LGE protocol were analyzed for strain and scar. Segmental CMR Ecc in the most negative quartile (quartile 4) ruled out scar in that segment, but scar was present in 46% of segments in the least negative  Ecc quartile 1, 33% of Ecc quartile 2 segments, and 15% of Ecc quartile 3 segments. Overall scar prevalence in inferior, inferolateral, and anterolateral segments was eight times higher than in inferoseptal, anteroseptal, and anterior segments (p < 0.001). This increased proportion of scar in lateral versus septal segments was consistent across CMR Ecc quartiles . Echocardiographic analysis could be performed in 12 of 14 patients with CMR exams and had to be limited to mid-cavity slices. Echo segmental Ecc in the most negative quartile made scar by CMR in that segment highly unlikely, but the correlation in segmental Ecc between CMR and echo was limited .The relationship between scar and Ecc in DMD is complex. Among myocardial segments with depressed Ecc, scar prevalence was much higher in inferior, inferolateral, and anterolateral segments, indicating a regionally dependent association between abnormal Ecc and scar, with free wall segments commonly developing dysfunction with scar and septal segments developing dysfunction without scar. Although normal echocardiographic Ecc predicted absence of scar, regional echocardiographic Ecc by VVI has only a limited association with CMR Ecc in DMD. Duchenne muscular dystrophy (DMD), the most common of the muscular dystrophies, with an incidence of 1 in 3,500 males, is an X-linked recessive disorder resulting from a disabling mutation of the gene encoding dystrophin, a sarcolemmal protein found in skeletal and cardiac muscles . There iCardiovascular magnetic resonance (CMR) has recently been investigated as a means to characterize the cardiac abnormalities of DMD -12. In aThe prevalence and distribution of regional myocardial scar relative to regional Ecc in DMD is not well understood and has important implications for our understanding of myocardial dysfunction in DMD. Ecc is a sensitive indicator of occult myocardial dysfunction in DMD , and aveThe study also sought to assess whether velocity vector imaging (VVI) assessment of Ecc would have some utility in DMD. VVI echocardiography offers far more advanced technology than that used in the original echocardiographic studies in DMD patients . VVI assThe protocol was approved by the Johns Hopkins Institutional Review Board and carried out in the General Clinical Research Center of the Johns Hopkins Hospital.All patients underwent CMR studies using a 1.5-T clinical scanner  with a phase array receiver coil on the chest. Cine images were acquired using a steady-state free precession pulse sequence with the following parameters: temporal resolution: 40 ms, echo time: 1.27 ms, flip angle: α = 50°, field of view: 36 cm, slice thickness: 8 mm, matrix size: 256 × 161, and bandwidth: 930 Hz/pixel.Myocardial tagging was performed using an electrocardiogram-triggered spoiled gradient echo pulse sequence with spatial modulation of magnetization grid-tagging. Short-axis slices were acquired at the left ventricular base, mid-cavity, and apex with the following parameters: repetition time: 3.5-7.2 ms, echo time: 2.0-4.2 ms, flip angle: α = 12°, field of view: 40 cm, slice thickness: 8 mm, matrix size: 256 × 140, bandwidth: 275 Hz/pixel, and tag spacing: 7 mm.LGE images in locations identical to the cine images were acquired 10-15 minutes after a bolus of 0.2 mmol/kg gadodiamide  or gadopentetate dimeglumine  in all patients. An inversion recovery fast gradient-echo pulse sequence was used for the acquisition with the following parameters: repetition time: 5.4 ms, echo time: 1.3 ms, flip angle: α = 20°, field of view: 36-40 cm, slice thickness: 8 mm, matrix size: 256 × 192, and inversion recovery time: 250 ms .Short axis tagged slices were analyzed in blinded fashion by the harmonic phase (HARP) method to assess strain . Segmental peak systolic strains were determined in 24 segments for basal and mid left ventricular slices. These were then grouped and averaged to provide average peak strain values for each of 6 segments in basal and mid-cavity slices . Thus, for each patient, we report the peak circumferential systolic strain in six myocardial regions at both basal and mid-cavity myocardium. Left ventricular end-systolic volume (LVESV) and left ventricular end-diastolic volume (LVEDV) were measured, and left ventricular ejection fraction (LVEF) calculated for each study patient. The strain analysis was performed by two experienced investigators , and interobserver variability for segmental strain was determined for 60 segments  acquired from 5 study participants (12 segments/participant).LGE images were analyzed, and abnormally enhanced myocardium was determined based on prior methods ,23 at si2-dimensional echocardiographic images were acquired using either GE Vivid 7 or Siemens Acuson machines and stored digitally at 30 frames per second . On a separate workstation , VVI  was used to determine peak Ecc. The endocardial border was manually traced at end-systole in the parasternal short axis view at the level of the papillary muscles, corresponding to the mid-cavity left ventricle, by a single researcher (WRT) without knowledge of the CMR data. A single cardiac cycle was selected for analysis. The software generated a vector for each frame in the cardiac cycle at 48 points along the endocardial border and calculated Lagrangian strain at each location. MATLAB  was used to find the peak (most negative) strain value for each point, and the average peak values from 8 consecutive points in each of the six segments in the mid-cavity left ventricle were calculated.All but one subject had quantitative hand-held myometry evaluation of bilateral elbow and knee flexion and extension strength within two months of the time of cardiac evaluation. Strength was determined in units of pounds as the average of these myometry measurements for muscle groups associated with the elbow and knee. An overall composite strength score of 0 indicates that patients were not capable of any movement in these muscle groups, although they may have some strength in the distal extremities.Continuous and categorical variables were reported as the median and interquartile range (IQR). Chi square tests and Fisher exacts tests were used to compare proportions among groups. We used t-tests for comparisons between continuous strain variables. Linear regression was used to determine associations between selected continuous variables, with the Pearson correlation coefficient (r) and p-values reported. The intraclass correlation coefficient (ICC) and associated 95% confidence interval were determined for the analysis of interobserver variability of segmental strain , as previously reported .16 DMD subjects were enrolled (age range 8-23), with individual clinical characteristics shown in Table Although echo studies were performed in all patients, two patients (ages 12 and 13) could not tolerate the CMR due to limited cooperation in one and contractures that prevented positioning in the MRI chamber in the second, and LGE could not be performed in another patient due to difficulty with venous access. Thus a total of 14 subjects had CMR, and 13 had LGE studies. Scar was rare in apical segments, so the analysis was performed in the basal and mid-cavity segments for Ecc and scar, based on a 12-segment model . Comparative analyses of scar and strain were performed both at the level of individual segments (n = 156) and on the basis of individual patients.For the segmental analysis, 156 segments were analyzed for scar and Ecc by CMR. Aggregate results for scar and average Ecc by patient are shown in Table The mean Ecc for segments in groups 1 and 2 were similar (p = NS). However, the relationship between segmental Ecc and scar was complex. As shown in Figure The relationship between the transmurality of scar and circumferential strain by CMR and echocardiography is shown in Figure VVI analysis for regional Ecc by echo could be performed in only 12 of the 14 patients who had CMR and only in mid-cavity rather than basal segments. As shown in Table 54% of patients had evidence of late gadolinium enhancement in at least one segment, and in all patients with scar the abnormality involved at least the lateral segments. Those with scar had a mean percent scar volume of 13% (range 6-29%). There was an inverse correlation between LVEF by CMR and the percent scar volume . Although scar was common in patients with moderately and severely reduced LVEF, some patients with only mildly reduced LVEF also were noted to have scar. Percent scar volume also correlated with average peak CMR strain in basal  and mid-cavity  segments.Analysis of the 12-lead ECG was performed for all patients. All patients had an R wave in lead V1 with amplitude ranging from 4-19 mV. Small q waves were present in lead I and aVL in most patients, but only one patient had prominent Q waves in leads I and aVL. Several ECG parameters were examined for prediction of inferior/lateral scar, including the R wave amplitude in V1, the R/S ratio in lead V1, and Q wave presence in leads I and aVL, but none demonstrated significant predictive value for scar. R/S ratios in V1 for individual patients are shown in Table The important findings of this study include the following: 1) segmental basal or mid-cavity Ecc by CMR in the highest quartile makes regional myocardial scar highly unlikely; 2) segmental scar is much more common in inferolateral, anterolateral, or lateral segments with depressed Ecc than in inferoseptal, anteroseptal, and anterior segments with depressed Ecc, consistent with a regionally dependent strength of association between depressed segmental Ecc and scar; 3) echocardiographic segmental mid-cavity Ecc based on velocity vector imaging in the highest quartile also makes scar (by CMR) in the corresponding segment unlikely, but there was only a limited correlation for mid-cavity segmental Ecc assessed using the two modalities.The importance of assessing and understanding myocardial scar and function in DMD has taken on increased clinical relevance with recent evidence that medical therapy with antifibrotic agents such as angiotensin converting enzyme inhibitors , angioteAlthough autopsy studies and CMR both provide excellent assessments of myocardial scar, CMR has the advantage of also providing quantitative regional assessment of myocardial function with Ecc. Of note, circumferential strain has been better studied in DMD than radial or longitudinal strain ,10. Ecc mdx mice [As in prior autopsy studies, we found that scar predominantly involved LV free wall segments and that septal segments were involved less frequently and only in patients with significant LV free wall involvement. In addition, the present study shows that there were similar numbers of LV septal and free wall segments with depressed Ecc; however, the septal segments with abnormal Ecc values were much less likely to have myocardial scar than free wall segments with similarly abnormal Ecc values. Although the pathophysiologic mechanism behind this curious tendency for the septal segments to become dysfunctional without scar and LV free wall segments to become dysfunctional with scar is unknown, TGF-β has emerged as the major likely effector of fibrosis in DMD. TGF-β inhibits terminal differentiation of myoblasts and is associated with the development of peripheral muscle fibrosis in DMD . Losartamdx mice . In addimdx mice . Althougmdx mice .Our findings regarding Ecc and scar complement those reported by Silva et al in a series of 10 DMD or Becker muscular dystrophy patients with regional dysfunction assessed by visual inspection rather than Ecc [Of note, we analyzed Ecc in this study using the well-validated HARP method  and demoWith respect to VVI echocardiography, adequate echocardiographic strain data could not be obtained in all DMD patients due to limited echocardiographic windows, and the analysis had to be limited to mid-cavity segments. Although normal segmental Ecc by echo predicted absence of scar in the corresponding segment by CMR, the correlation between VVI and CMR assessments of Ecc in mid-cavity segments was limited. Based on these results, although VVI echocardiographic assessment of regional Ecc in DMD was feasible, it does not appear to be an adequate surrogate in DMD for CMR with MTT, which is presently regarded as the gold standard for myocardial Ecc ,35.With respect to electrocardiography, although there may be clues in the 12-lead electrocardiogram suggestive of more advanced cardiac disease and scar, we were unable to identify a specific predictor relative to CMR findings, highlighting the importance of accurate and feasible cardiac imaging for this purpose.The relationship between scar and Ecc in DMD is complex. Among myocardial segments with depressed Ecc, scar prevalence was much higher in inferior, inferolateral, and anterolateral segments, indicating a regionally dependent association between abnormal Ecc and scar. As a result, LV septal segments are more likely to develop abnormal Ecc without scar in DMD, whereas abnormal Ecc is commonly associated with scar in LV free wall segments. With respect to echocardiography, although normal segmental Ecc by VVI is associated with absence of scar, VVI assessment of abnormal regional echocardiographic Ecc was only weakly associated with CMR Ecc in DMD.CMR:  cardiovascular magnetic resonance; DMD:  Duchenne muscular dystrophy; Ecc:  circumferential strain; ECG:  electrocardiogram; HARP:  harmonic phase; IQR:  interquartile range; LGE:  late gadolinium enhancement; LV:  left ventricle; LVEDV:  left ventricular end-diastolic volume; LVESV:  left ventricular end-systolic volume; MTT:  myocardial tissue tagging.The authors declare that they have no competing interests.KCB played an important role in the conception/design of the study, acquired the CMR data, performed the primary analysis of CMR strain and scar data, and drafted the manuscript. DP and MS also performed analysis of CMR data and revised the manuscript critically for important intellectual content. YD performed analysis of the echocardiographic data and provided a critical review of the manuscript. TC and DD were involved in the conception and design of the study, performed neurologic evaluations of the study participants, and provided a critical review of the manuscript for important intellectual content. WRT was involved in the conception and design of the study, both acquired and analyzed the echocardiographic data, and provided a critical review of the manuscript. All authors have reviewed and approved the final manuscript.


To test the hypothesis that myocardial hyper- intensities on T2 weighted imaging in patients with hypertrophic cardiomyopathy (HCM) reflect ongoing myocardial injury, the cardiac- specific biomarker troponin T was measured by a new highly sensitive assay (hs-cTnT). In 52 patients with HCM, the presence and extent of myocardial T2 abnormalities were associated with hs-cTnT levels. The number of LV segments with T2 hyper-intesity was the strongest independent predictor of hs-cTnT levels. This novel finding suggests that T2 imaging may aid in the identification of HCM patients with active and potentially progressive disease.In a sub-group of patients with hypertrophic cardiomyopathy (HCM) abnormalities on T2 weighted imaging on cardiovascular magnetic resonance imaging (CMR) have been reported. The significance of this finding is still not clear, although it has been proposed to reflect ongoing myocardial injury. The loss of cardiomyocyte cell integrity leads to the release of troponins into the circulation. Recently, a highly sensitive cardiac troponin T assay (hs-cTnT) was introduced which allows the detection and quantification of very low serum levels of cTnT with a high diagnostic accuracy.We therefore aimed to test the hypothesis that the presence and extent of myocardium with increased signal intensity on T2-weighted images in patients with HCM is associated with evidence of cardiomyocyte injury as assessed by the hs-cTnT assay.The study cohort consists of 52 patients undergoing late gadolinium enhanced CMR (LGE-CMR) on a 1.5T clinical scanner (Philips Intera Achieva®) for characterization of hypertrophic cardiomyopathy in whom hs-cTnT was measured by the ELECSYS® assay (Roche Diagnostics). Left ventricular (LV) volumes and mass were derived from SSFP images. The presence of hyper-intense areas on T2 images was assessed in the modified LV AHA-16 segment model by two blinded observers. LGE was quantified in percent of LV mass.T2 hyper-intensities were present in 31 (60%) patients (T2-pos) with a mean of 2.67± 1.6 LV-segments affected. The presence of T2 hyper-intensity was associated with increased septal wall thickness  and the presence  and extent of LGE . T2-pos patients showed markedly increased levels of hs-cTnT . Furthermore, the number of myocardial segments showing a signal hyper-intensity on T2 imaging was correlated to hs-cTnT levels . Remarkably, when entered into a multivariate logistic regression model, the number of T2 segments was the only independent predictor of hs-cTnT levels in addition to renal function (tableThe presence and the extent of myocardial T2 abnormalities in patients with HCM predict serum levels of hs-cTnT and thus seem to reflect ongoing myocardial damage. Whether this finding is associated with an increased risk for future adverse events needs to be examined in further studies.None.


Right ventricular ejection fraction (RVEF) has been shown to be an independent predictor of mortality after myocardial infarction. However, the predictive value of right ventricluar (RV) assessment in patients with severe ischemic cardiomyopathy (ICM) is unknown.In patients with severe ICM, we sought to assess the association of RVEF, RV end systolic and diastolic volumes (RV ESVi/RV ESDVi), right ventricular systolic pressure (RVSP) with outcomes in severe ICM.450 patients with > 70% stenosis in ≥1 epicardial coronary artery  22 %, median ESVi 106ml, median RV EF 45%. Median RVESVi 37.5ml) underwent delayed hyperenhancement-MRI between 2002-2007  between 2003-2007. CMR evaluation included long and short axis assessment of LV function on balanced steady state free precession images along with assessment of myocardial scar (on phase-sensitive inversion recovery DHE-CMR sequence ~ 10-20 minutes after injection of 0.2 mmol/kg of Gadolinium dimenglumine). Cox proportional hazards survival modeling, using a primary end-point of all-cause mortality), was used to risk-adjust comparisons. Echocardiograms performed within 60 days were analyzed, and RVSP was assessed as described by American Society of Echocardiogram guidelines.Results: Over a follow-up of up to 9 years[mean 5.75 years], 186 deaths occurred. Survival analysis revealed that after adjusting for left ventricular scar burden, left ventricular ESVi, subsequent revascularization, sex, age, mitral valve procedures, RVSP , RVESVi , and RVEDVi  were independent predictors of mortality. See Table Conclusions: RV volumes and RVSP, provide independent, incremental prognostic value in patients with severe ICM. RV EF did not provide independent prognostic value.None.


Type 2 diabetes (T2D) is an independent risk factor for adverse cardiac events, such as hypertension and myocardial infarction. The underlying biochemical changes associated with diabetes have been shown to increase collagen deposition and cross-linking, which may lead to microscopic alterations in the myocardial structure that precede measurable changes in cardiac function. Non-invasive imaging methods are important for risk stratification of asymptomatic patients and to gain a better understanding of the pathophysiology of diabetic heart disease. T1 mapping is a novel Cardiovascular Magnetic Resonance Imaging (CMR) technique for quantification of increased extracellular volume fraction that occurs with diffuse collagen replacement. The objective of this research is to examine post-contrast myocardial T1 values in asymptomatic T2D patients.Patients diagnosed with type 2 diabetes , and healthy, non-diabetic age-matched controls  were assessed at the Stephenson CMR Centre in Calgary using a clinical 1.5 T scanner. Medical history and ECG were reviewed to rule out ischemic heart disease. Eligible patients underwent a CMR scan including quantitative T1 mapping (SASHA) before and 15 minutes after 0.15 mmol/kg of Gd-DTPA (Magnevist) contrast injection, with subsequent evaluation of mid and basal slices for segment and global T1 values . No significant differences in functional parameters were found between patients and controls; both had normal systolic function , although T2D patients had a slightly reduced indexed LV end diastolic volumes. No regional areas of LGE were detected in either group.The observed decrease in T1 mapping times reflect an increased extracellular volume fraction, and may reflect globally increased collagen deposition in diabetic myocardium. This finding compliments previous findings by this group of preclinical changes in microvascular function in diabetes. Further studies are required to assess the pathophysiologic context and prognostic impact.none


Evaluation of a dynamic T2-preparation (dynT2prep) sequence, whereby the first dynamic is acquired with and the second without T2 preparation. T2 preparation allows suppression of tissues with short T2 relaxation times, e.g. deoxygenated blood. Tissues with long T2 relaxation times, e.g. oxygenated arterial blood, are minimally influenced. Thus both blood of the pulmonary veins and left ventricle should appear signal suppressed on subtracted dynT2prep images (T2prep OFF - T2prep ON). Subtraction of both dynamics, however, always resulted in black blood images of the great cardiac chambers and coronary arteries while the pulmonary circulation appeared bright due to a frequency offset in the lungs Figure . This mePatients with congenital heart disease (CHD) were examined with a dynamic T2prep 3D-whole heart acquisition  and compared to ten healthy subjects  in diastole. An independent samples t-test and a paired t-test were performed to compare normally distributed variables.An increase of SNR was seen in children and healthy volunteers in the subtracted images. After analyzing the B0-map, statistically significant differences were shown for the pulmonary veins (p<0.027).The brightest signal in all cases was observed in the pulmonary circulation. This effect is caused by an off-resonance phenomenon. Whilst using T2-preparations pulses, the B0-field inhomogeneity acts as a susceptibility background gradient and leads to a suppression of moving tissue and blood and consequently to a signal enhanced pulmonary circulation in subtraction images.Division of Imaging Sciences, The Rayne's InsituteKing's College London


We hypothesised that homozygosity for the α2CDel322-325 polymorphism may be a risk factor for heart failure due to idiopathic dilated cardiomyopathy (DCM) in black South Africans.A four-amino acid deletion was identified within the α2CDel322-325 polymorphism was genotyped in 37 patients with heart failure and 34 controls, all of black African ancestry. Genotyping was performed by a size-fractionation assay.The α2CDel322-325 polymorphism or in allele and genotype frequencies between patients and controls. The frequency of the allele containing the deletion was 0.54 in cases and 0.53 in controls. The genotype frequencies in the patients were consistent with those of the controls (p = 0.56).The patients studied ranged in age from 21 to 79 years with a mean age of 50 years, and 62% were male. No significant difference was observed in homozygosity for the α2CDel322-325 polymorphism is not associated with an increased risk for heart failure due to idiopathic DCM in black South Africans.Homozygosity for the α The function of α2C-AR is to regulate the release of norepinephrine from cardiac sympathetic nerves through negative feedback. The α2CDel322-325 AR variant shows decreased function, which results in increased synaptic norepinephrine release. The association between the α2CDel322-325 AR polymorphism and heart failure is biologically plausible, as sustained cardiac adrenergic stimulation has been implicated in the development and progression of heart failure.3Dilated cardiomyopathy (DCM) is a major cause of congestive heart failure in Africa.We hypothesised that homozygosity for this variant may be a risk factor for heart failure due to idiopathic dilated cardiomyopathy in black South Africans.Patients of black African ancestry, who were diagnosed with heart failure due to idiopathic DCM, were recruited from Groote Schuur Hospital in Cape Town and Dr George Mukhari Hospital in Tshwane. The diagnosis of idiopathic DCM was made after comprehensive investigation to exclude a secondary cause of heart failure; cardiac catheterisation was performed in patients with a history suggestive of coronary artery disease and/or risk factors for coronary disease.The patients provided informed consent for participating in genetic studies of cardiomyopathy, and the studies were approved by the Research Ethics Committee of the University of Cape Town. The control group, which was matched for ethnic background, was recruited from the Eye Clinic at Groote Schuur Hospital and had no history of cardiac disease, as previously reported.2CDel322-325 polymorphism was performed in 37 patients and 34 controls. The region of interest was amplified from genomic DNA using oligonucleotides 5′-TGCGTCCCCGACTACCGAAA-3′ and 5′-FAMTGACCACAGCCAGCACAAAGG-3′. Amplicons were size-fractionated on an ABI Prism® 3100 genetic analyzer (Applied Biosystems). The genotype and allele frequencies in cases and controls were compared.Genotyping for the α2 test was used to evaluate the association between heart failure and genotype. This was an exploratory study; hence it was not designed to maximise power to detect an association. However, given the frequency of the deletion variant in the study, the study had 91, 84 and 69% power to detect a significant association at a 95% significance level for a genotypic relative risk of heart failure of five, four and three, respectively, under an additive model (the reported increased risk is five-fold for the association of the polymorphism with heart failure in black subjects).The χThe 37 patients studied ranged in age from 21 to 79 years with a mean age at diagnosis of 48 (± 16) years, and 25 of the patients (68%) were male. The mean left ventricular ejection fraction was 36% (± 9), and 29 patients (78%) underwent cardiac catheterisation to exclude coronary artery disease. All patients were on standard medication for symptomatic heart failure, and two had a past history of hypertension. The HIV status of the cases was not determined in this series.p = 0.734). There was no significant association between the α2CDel322-325 polymorphism and heart failure under an additive model , a dominant model , or a recessive model ; the recessive model is equivalent to comparing deletion homozygotes to the rest.The distribution of allele frequencies was in Hardy-Weinberg equilibrium  or decreased left ventricular ejection fraction (LVEF)], no association was found in either the African-American or the white American patients.7In this study no association was found between the αThere are several reasons that may explain the discrepancy between our findings and the original report of an association, 2CDel322-325 polymorphism is unlikely to be a major risk factor for heart failure due to idiopathic DCM in our African patients.Despite these limitations, the results of our study suggest that the α


Circulatory failure due to acute arterial hypertension in the neonatal period is rare. This study was undertaken to assess the clinical and echocardiographic manifestations of circulatory failure resulting from acute neonatal hypertensive crisis.Neonatal and cardiology databases from 2007 to 2010 were reviewed. An established diagnosis of circulatory failure due to neonatal hypertension before the age of 14 days was required for inclusion. Six patients were identified.Five patients presented with circulatory failure due to an acute hypertensive crisis. The median age at presentation was 8.5 days (range: 6.0–11.0) with a median body weight of 3.58 kg (range: 0.86–4.70). Echocardiography demonstrated mild left ventricular dysfunction [median shortening fraction (SF) 25%, range 10–30) and mild aortic regurgitation in 83% (5/6) of patients. One patient with left ventricular dysfunction (SF = 17%) had a large apical thrombus. Two patients were hypotensive, and hypertension only became evident after restoration of cardiac output. Administration of intravenous milrinone was successful, with rapid improvement of the clinical condition. Left ventricular function normalised in all survivors.Early neonatal circulatory collapse due to arterial hypertension is a rare but potentially life-threatening condition. At presentation, hypotension, especially in the presence of a dysfunctional left ventricle, does not exclude a hypertensive crisis being the cause of circulatory failure. The echocardiographic presence of mild aortic regurgitation combined with left ventricular hypocontractility in a structurally normal heart should alert the physician to the presence of underlying hypertension. Circulatory failure is frequently the forerunner of a serious underlying condition in the neonate. It requires rapid detection of origin to differentiate non-cardiac  from cardiac causes. Echocardiography is requested as an early diagnostic test, primarily with the intention to exclude potentially lethal structural heart disease, for example coarctation of the aorta, critical aortic stenosis, or hypoplastic left heart. When left ventricular dysfunction is observed, the focus shifts to myocardial disease. Acute episodes of systemic hypertension are rarely, if ever, considered in the differential diagnosis.Very little is known about the incidence of acute hypertensive crisis in newborn infants. Acute systemic hypertensive episodes during this period are most likely under-recognised and underdiagnosed. It is a rare but potentially life-threatening condition. Systemic hypertension in neonates has a reported incidence varying from 0.2 to 2.6%.4Cardiomegaly, hypocontractility, overt cardiac failure and even death related to neonatal hypertension have been described but consist mostly of a few case reports.This study was undertaken to assess the clinical and echocardiographic manifestations of circulatory failure resulting from acute hypertensive events.This was a retrospective review. To be considered for inclusion, an established diagnosis of circulatory failure associated with a blood pressure more than the 95th percentile for gestational age and weight during the course of admission in the neonatal intensive care unit before the age of 14 days was essential.Standard descriptive and demographic data were obtained from patient records. Patient charts were reviewed for clinical course, laboratory results and outcome. In order to eliminate inter-observer bias, echocardiograms performed during the initial presentation were digitally reviewed by a single paediatric cardiologist and recalculated. The averages of previous and recalculated measurements were used.z-scores were calculated.Left ventricular systolic function, end-diastolic dimension and wall thickness were measured by means of standard M-mode and two-dimensional views. Using previously published normal values, Approval by the local medical ethics committee was obtained. Data were analysed using standard statistical software . Data are presented as medians with minimum and maximum values where appropriate.The median age at presentation was 8.5 days (range: 6.0–11.0) with a median body weight of 3.58 kg (range: 0.86–4.70). All infants were full term, except one 28 weeks premature infant with a birth weight of 860 g. All patients had circulatory failure and required admission to the intensive care unit for cardiopulmonary support. At initial presentation, three infants had hypertension while two patients were hypotensive and one normotensive. Hypertension in the latter only became evident after resuscitation and restoration of cardiac output. Common symptoms included lethargy, feeding intolerance or poor feeding. Patient characteristics are depicted in Table 1.Umbilical arterial lines had been inserted in two children prior to the episodes of hypertension. None of the mothers were diabetic and maternal health was good except in one where a history of herpes infection and pre-eclampsia was present. There was no history of ingestion of drugs associated with hypertension.Lactate levels were elevated in most infants on admission Systolic cardiac function was considerably impaired in three patients and mildly impaired in two, with a median SF of 25% for the group as a whole , two patients presented with hypotension and shock and one was normotensive It is of clinical significance that all our patients presented with some form of circulatory failure. Although most were hypertensive (In most of our patients, hypertrophy of the interventricular septum and/or left ventricular posterior wall was evident. This increase in left ventricular mass had also been reported by Peterson.14The differential diagnosis of neonatal hypertension has been extensively reviewed.Echocardiography is usually requested once an infant with circulatory failure is admitted to the neonatal intensive care unit. Faced with this clinical presentation, the demonstration of hypocontractility would inevitably lead the cardiologist to consider a differential diagnosis of myocarditis, cardiomyopathy, coarctation of the aorta or coronary artery anomalies. Left ventricular hypocontractility in the absence of hypertension will be misleading in this case. However, careful analysis of the above mentioned echocardiographic findings associated with mild aortic regurgitation should alert the physician to consider systemic hypertension as the probable underlying cause.Mitral regurgitation is not unexpected in the presence of left ventricular dysfunction and is frequently observed in association with cardiomyopathy. However, aortic regurgitation is very unusual in a supposedly normal heart, provided the valve is structurally normal. Although the left heart was not dilated, central aortic and mitral valve regurgitation were seen in the majority of patients. We did not measure aortic diameter in this study but Peterson and coworkers reported mild dilation in their study, which they ascribed to the increased aortic distensability in neonates.Left ventricular hypocontractility was observed in a significant number of our patients. Acute decompensation can be explained on the basis of normal neonatal cardiac physiology. The neonatal heart has, by way of its structure, function and unique changes in loading conditions, limited cardiac reserves and therefore also less compensatory ability. After birth, volume load on the left heart increases sharply, from less than 35% of the total output to 50% of combined ventricular output. Afterload also increases due to marked increase in the systemic vascular resistance. If an additional insult such as the acute pressure load of arterial hypertension is added to an ‘untrained’, adapting left ventricle, acute left ventricular failure becomes a realistic scenario.The dilated coronary arteries in two-thirds of our patients are intriguing. Adult studies have shown coronary arteries to proportionately increase in diameter in association with left ventricular hypertrophy.Cardiac failure was treated using milrinone, which in our clinical judgment gave a beneficial response in all our patients and improved cardiac function. Milrinone is a treatment of choice under these circumstances since the phosphodiesterase inhibitors decrease systemic and pulmonary vascular resistances and increase cardiac contractility.19It should be noted that the reference ranges for plasma renin activity show marked variation and vary from laboratory to laboratory. Although transient elevation of PRA or/and plasma aldosterone levels, with subsequent normalisation after control of hypertension, was observed in all patients, underlying renal causes could only be demonstrated in two patients during admission. Whether this renin–angiotensin–aldosterone system activation was part of a reactive stress response or a causative event remains unclear.The study was limited by the small number of patients and its retrospective nature. In order to reduce observer bias and variability, all echocardiograms were independently re-examined by a single cardiologist. As a consequence of the small sample of patients, the true incidence of hypertension cannot be determined. Only patients with circulatory failure were included, therefore patients with milder forms of disease may not have been detected. Diastolic functional assessments were not carried out at admission and are therefore not available. Further studies are required to elucidate the underlying pathophysiology.Findings of this study are based on a limited number of patients. However, early neonatal circulatory collapse due to arterial hypertension is a rare but potentially life-threatening condition. The echocardiographic presence of mild aortic regurgitation combined with left ventricular hypocontractility in an otherwise structurally normal heart should alert the physician to the possibility of underlying hypertension. Clinicians and echocardiographers should be aware that at presentation, hypotension, especially in the presence of a dysfunctional left ventricle, does not exclude a hypertensive crisis and failure to consider the diagnosis may lead to death. Long-term cardiac outcome in survivors appears good.


Cardiovascular magnetic resonance (CMR) tissue tagging has been established as non-invasive technique for accurate measurement of myocardial motion. However, additional tagging sequences are necessary and the postprocessing procedure is time consuming. Recently, the novel technique of feature tracking (FT) was introduced. With FT myocardial strain is derived directly from standard steady-state free precession (SSFP) sequences. First studies in children showed a good agreement between tagging and FT for the measurement of circumferential strain (cc). But these results are limited for the circumferential strain of the medial slice.Aim of this study was to compare more comprehensive tagging data with data derived by FT in adult patients with high grade aortic stenosis (AS).A total number of 28 patients with severe AS underwent cardiac MRI at 1.5 T (Philips Achieva). SSFP images were performed for the short and long axis. Tagging of three short axis planes  was acquired using the CSPAMM technique. The serial short axis slices were used for the assessment of LVEDV, LVESV, LV mass and function.Mean LVEF (56±14%), LVEDV (106±58ml) and LVESV (47±48ml) were normal. All patients demonstrated concentric hypertrophy of the LV with an increased basal septal thickness .A highly significant correlation was observed between tagging and FT for the derived basal and medial cc  and systolic/early diastolic strain rate (kappa 0.7/0.5).To our knowledge is this the first study, which compares different tagging parameters beyond medial cc with FT. FT is a promising method to assess cc using the medial and basal slice in patients with AS. We found a highly significant correlation for the medial cc, which is in agreement with previous published data. Moreover the basal cc showed an excellent correlation, while the correlation for the apical cc was weak. This may be caused by myocardial hypertrophy with less apical endsystolic ventricular volume in patients with AS.No correlation was observed for different other tagging parameters beyond cc. This could be based on the different technical methods and is hampered by the limited number of patients included.no funding


Mitral annular disjunction (MAD) consists of an altered spatial relation between the left atrial wall, the attachment of the mitral leaflets, and the top of the left ventricular (LV) free wall, manifested as a wide separation between the atrial wall-mitral valve junction and the top of the LV free wall. Originally described in association with myxomatous mitral valve disease, this abnormality was recently revisited by a surgical group that pointed its relevance for mitral valve reparability. The aims of this study were to investigate the echocardiographic prevalence of mitral annular disjunction in patients with myxomatous mitral valve disease, and to characterize the clinical profile and echocardiographic features of these patients.We evaluated 38 patients with myxomatous mitral valve disease  and used standard transthoracic echocardiography for measuring the MAD. Mitral annular function, assessed by end-diastolic and end-systolic annular diameters, was compared between patients with and without MAD. We compared the incidence of arrhythmias in a subset of 21 patients studied with 24-hour Holter monitoring.MAD was present in 21 (55%) patients (mean length: 7.4 ± 8.7 mm), and was more common in women . MAD patients more frequently presented chest pain . Mitral annular function was significantly impaired in patients with MAD in whom the mitral annular diameter was paradoxically larger in systole than in diastole: the diastolic-to-systolic mitral annular diameter difference was -4,6 ± 4,7 mm in these patients vs 3,4 ± 1,1 mm in those without MAD (p < 0.001). The severity of MAD significantly correlated with the occurrence of non-sustained ventricular tachycardia (NSVT) on Holter monitoring: MAD›8.5 mm was a strong predictor for (NSVT), ; sensitivity 67%, specificity 83%). There were no differences between groups regarding functional class, severity of mitral regurgitation, LV volumes, and LV systolic function.MAD is a common finding in myxomatous mitral valve disease patients, easily recognizable by transthoracic echocardiography. It is more prevalent in women and often associated with chest pain. MAD significantly disturbs mitral annular function and when severe predicts the occurrence of NSVT. Mitral annular disjunction consists of a perceptible separation between the left atrial wall-mitral valve junction and the top of the left ventricle wall Figures  and 3.. This abnormality was forgotten until a Canadian surgical group recently stated the relevance of its recognition prior to mitral valve repair . In thesFor a long time, at our echo laboratory, we have been confronted with the recognition of this structural abnormality which we have empirically associated with distinct clinical features, namely a high incidence of arrhythmias.The aims of our study were to determine the prevalence of echocardiographically-recognized mitral annular disjunction in patients with myxomatous valve disease, and to compare the clinical profile and echocardiographic features of patients with and without this abnormality.For the purpose of this study, we reassessed the clinical and echocardiographic data from all patients with myxomatous mitral valve disease who underwent a transthoracic echocardiographic examination in our laboratory between July 2003 and September 2006. Myxomatous mitral valve was defined as the presence of excess leaflet tissue and leaflet thickening greater than 5 mm, resulting in a prolapse greater than 2 mm into the left atrium on parasternal long axis view [European Association of Echocardiography recommendations [European Association of Echocardiography recommendations [All patients underwent a complete transthoracic 2D, M-mode, and Doppler examination using commercially available systems . Image acquisitions and measurements were carried out by senior echocardiographers in accordance with the ndations . The lefndations .The length of annular disjunction was measured from the left atrial wall-mitral valve posterior leaflet junction to the top of the LV posterior wall during end-systole Figures  and 3. MIn order to evaluate the arrhythmic profile, a subset of 21 patients not submitted to mitral valve surgery was further studied with 24-hour Holter monitoring. The frequency of ventricular tachycardia was quantified by the sum of beats in ventricular tachycardia.t test for continuous data and the chi-square test or Fisher's exact test for categorical data, as appropriate. The Mann-Whitney test was used to compare differences between continuous non-parametric variables. The ANOVA method was used to compare differences between more than two groups. Receiver Operating Characteristic (ROC) curve analysis was performed to determine the disjunction length that more accurately predicted the occurrence of non-sustained ventricular tachycardia. Inter and intra-observer correlations were evaluated by Pearson correlation coefficient analysis. Statistical significance was accepted for a two-tailed P < 0.05. SPSS for Windows version 13  and MedCalc for Windows version 9.3.8.0  were used to perform the statistical analyses.All data presented are shown as mean ± standard deviation or absolute number (percentage). To compare the differences between groups, we used Student's Clinical and echocardiographic characteristics of the population are shown in tables Mitral annular disjunction was seen in 21 (55%) patients and on average measured 7.4 ± 8.7 mm. Inter and intra-observer correlations were 0.97 and 0.94, respectively. The most severely affected patient had a disjunction length of 30 mm. This particular patient had involvement of the entire annular circumference, and this feature was well documented by transoesophageal echocardiography  were in permanent atrial fibrillation, and another 4 patients (11%) had at least one episode of paroxysmal atrial fibrillation. There were no differences regarding atrial fibrillation frequency between the group with and without annular disjunction . Although impressive, this proportion is nevertheless smaller than the 92% prevalence found by Hutchins et al , and theReal-time three-dimensional (3D) and 3D reconstruction transoesophageal echocardiography affords a better accuracy in patients with complex MV pathology when compared with 2D transoesophageal echocardiography.,7 The coTo the best of our knowledge, this is the first study where the recognition of mitral annular disjunction is described by transthoracic echocardiography. On intraoperative transoesophageal echocardiography, Eriksson et al described a significantly higher rate of mitral annular disjunction in patients with advanced versus mild or moderate mitral valve degeneration (98% vs 9%). In our Mitral annulus contractility contributes significantly to mitral valve function. Shortening of the annulus diameter during systole facilitates coaptation of the mitral leaflets -10. ImpaIn the presence of annular disjunction, the valve insertion in the "atrial wall" is responsible for an increased diameter of the mitral valve circumference during systole, and hence impaired annular function due to coaptation deficit. Underestimating this abnormality during mitral valve repair can result in recurrent mitral regurgitation, since paradoxical systolic enlargement will persist .The risk of sudden death is increased in patients with mitral regurgitation due to myxomatous mitral valve disease. Prior to surgery, the incidence of sudden death is 1.8% per year, accounting for one-fourth of the causes of death. Patients with severe symptoms, atrial fibrillation, and reduced LV systolic function are at higher risk. However, even asymptomatic patients in sinus rhythm with normal LV function are not exempt of risk, which occurs with an incidence of 0.8% per year -15. IncrThe limitations of this study are its retrospective nature, the rather small population, and the performance of 24-hour Holter monitoring in a limited subset of patients. Larger and prospective studies are needed to validate our findings. Nevertheless, the novelty of the subject and the implications for both surgical management and for arrhythmic events remain of paramount importance.Mitral annular disjunction is a common finding in patients with myxomatous mitral valve disease, easily detected and measured by transthoracic echocardiography.We found an association between this abnormality and several clinical features namely chest pain, annular contractile dysfunction, and non-sustained ventricular tachycardia in cases of wider magnitude of the disjunction. It proved to be relevant for the success of mitral valve repair.Further and larger studies are needed to completely understand the clinical implications of annular disjunction, particularly its association with malignant arrhythmic events.MAD: Mitral annular disjunction; LV: Left ventricular; NSVT: Non-sustained ventricular tachycardia;The authors declare that they have no competing interests.PC and MJA were responsible for study design, analysis and interpretation of data, manuscript drafting and critical revision. CA contributed to analysis and revised the manuscript for important intellectual content. RR contributed to study design. RG and JAS have supervised and commented the manuscript. All authors read and approved the final manuscript.Movie 1. Disjunction affecting the entire mitral annular circumference in a transoesophageal echocardiogram.Click here for fileMovie 2. Parasternal long axis view showing significant disjunction of the posterior annulus.Click here for fileMovie 3. Apical 4-chambers view showing disjunction of the lateral annulus.Click here for fileMovie 4. Apical 2-chambers view showing wide disjunction of anterior and inferior mitral annulus.Click here for fileMovie 5. Significant mitral disjunction of the posterior annulus (top left), and of the inferior annulus (top right). Paradoxical annulus enlargement during systole (Bottom).Click here for file


Transthoracic echocardiography (TTE) is the first-line tool for diagnosis and followup of pediatric and young adult patients with congenital heart disease (CHD). Appropriate use of TTE can reduce the need for more invasive modalities, such as cardiac catheterization and cardiac magnetic resonance imaging. New echocardiographic techniques have emerged more recently: tissue Doppler imaging, tissue tracking (strain and strain rate), vector velocity imaging (VVI), myocardial performance index, myocardial acceleration during isovolumic acceleration (IVA), the ratio of systolic to diastolic duration (S/D ratio), and two dimensional measurements of systolic right ventricular (RV) function . These may become valuable indicators of ventricular performance, compliance, and disease progression. In addition, three-dimensional (3D) echocardiography when performed for the assessment of valvular function, device position, and ventricular volumes is being integrated into routine clinical care. In this paper, the potential use and limitations of these new echocardiographic techniques in patients with CHD are discussed. A particular focus is on the echocardiographic assessment of right ventricular (RV) function in conditions associated with increased right ventricular volume  or pressure  in children and young adults. Echocardiography has become the most important and routinely applied noninvasive imaging technique for the diagnosis and followup of patients with congenital heart disease (CHD). Cross-sectional Doppler echocardiography allows a detailed description of cardiovascular anatomy, ventricular, and valvular function. The diagnostic accuracy for describing cardiac morphology is very high, with a reported incidence of less than 100 errors in more than 50.000 echocardiograms –4, lowerTraditionally, dimensional changes or volumetric changes during systole of the cardiac cycle have been used to assess systolic ventricular function. This can be done using M-mode techniques to calculate fractional shortening or by volumetric techniques to calculate ejection fraction. These techniques are especially load-sensitive (preload dependent) and rely on geometrical assumptions. Therefore, they may not be readily applicable to congenital heart disease. Recent technical developments have renewed the interest in the assessment of ventricular performance in patients with CHD.Newer techniques including tissue Doppler echocardiography, tissue tracking (speckle-tracking-based strain imaging), and vector velocity imaging (VVI) provide direct quantitative information about myocardial motion and deformation. Three-dimensional (3D) echocardiographic techniques enable the acquisition of full volumetric datasets, which can be analysed offline for the calculation of ventricular volumes, mass, and EF.Transthoracic echocardiography (TTE) has the advantage of lower cost, less risk, and greater availability compared with cardiac magnetic resonance imaging or cardiac catheterization. In this paper we discuss and highlight recently developed echocardiographic techniques and according variables such as tissue Doppler imaging, tissue tracking and vector velocity imaging (deformation imaging for strain and strain rate), myocardial performance index, myocardial acceleration during IVA, systolic to diastolic ratio. Also measurements of longitudinal systolic RV function such as tricuspid annular plane systolic excursion (TAPSE) and peak systolic velocity (TAPSV) and 3D echocardiography are discussed .In the 1990s, tissue Doppler imaging started being recognized as a potentially clinically useful technique for the assessment of global and regional myocardial systolic and diastolic function . As the Normal paediatric tissue Doppler imaging (TDI) data has been published . It was The use of TDI velocities in a functionally univentricular heart has also been studied. Frommelt et al.  used theApplications of TDI for RV function analysis in pediatrics have also been investigated. In healthy neonates born at or near term, the longitudinal systolic tricuspid annular velocity was only 1.2 times that of the mitral annular velocity . This raColor TDI was introduced as an alternative technique for measuring tissue velocities. In contrast to pulse wave (PW) Doppler, which measures peak velocities, color TDI uses autocorrelation techniques in order to measure regional mean velocities. An advantage of colour TDI versus PW TDI is that tissue velocities can be recorded simultaneously in different myocardial segments during the same cardiac cycle. Kukulski et al.  demonstrThe advantage of TDI in CHD is that the technology can be applied to any chamber morphology and variables are not based on assumption of chamber geometry. The limitations of PW TDI and color Doppler TDI are mainly related to the variability of the measured velocities with different loading conditions. This is often more of a problem when assessing the RV free wall than septal of LV free wall TDI velocities.The myocardial performance index (Tei index) evaluates global ventricular function by measuring the ratio of isovolumic time intervals to ventricular ejection . The lonr = 0.94) in healthy subjects in this study [In the neonatal period, the RV and LV TEI were found to inversely correlate with RV output and EF, respectively, while the LV Tei index was found to correlate inversely with LV output and LV EF in the neonatal period as assessed using pulsed-Doppler echocardiography . In addiis study . The useis study . In thisis study . The RV is study  and has is study . Robersois study . Cheung is study , making TDI-Tei index (TDI-MPI) differs from the conventional Tei index in that it is measured in a single cardiac cycle, increasing its accuracy. The TDI-Tei has also been used to assess RV function in patients with pulmonary regurgitation (PR) after TOF repair and was significantly higher (=impaired) in TOF patients compared with controls, while there was no difference in conventional Tei index between TOF patients and control subjects . In postLimitations of the Tei index include the combination of systolic and diastolic time intervals in a single index which does not allow distinction between systolic and diastolic dysfunction . An additional limitation of the RV (versus LV) Tei index is that the Doppler beam cannot be aligned in a way that it runs through the RV inflow and RV outflow, which is possible for the mitral inflow and LVOT. Therefore, for the RV Tei index, two traces need to be recorded at identical heart rates, which may be impractical in the clinical setting. Caution is warranted when interpreting RV Tei indices obtained at even slightly different heart rates.2. The heart-rate sensitivity of IVA has been used to assess contractile reserve by studying the force-frequency relationship during pacing, stress echocardiography, or exercise [The isovolumic acceleration assesses myocardial motion during the isovolumetric contraction period. During this time period, there is a myocardial motion, related to the shape change from a more spherical ventricle to a more ellipsoid shape. The rate of myocardial acceleration during the isovolumic period has been described to correlate with intrinsic myocardial contractility and is thought to be relatively independent of loading conditions . IVA canexercise , 34. In exercise . Vogel eexercise  used triexercise .The advantage of IVA is that it is a relatively load-independent measure of RV function. A limitation of IVA is that it is sensitive to heart rate and carries—according to our experience—a relatively high interobserver variability.Wall motion abnormalities are relatively common in patients with CHD. Strain and strain rate seem to be useful in detecting such motion abnormalities. Regional deformation (strain) and strain rate can be calculated noninvasively in both the LV and RV, providing information on regional myocardial dysfunction in a variety of clinical settings. Myocardial velocities and displacement are influenced by global cardiac translational motion and by motion in adjacent myocardial segments  which limits their use in the assessment of regional myocardial function. This limitation can be overcome by using regional myocardial deformation or strain imaging.Myocardial strainand strain rate are measures of deformation within segments of the myocardium and provide additional measurements of myocardial mechanical function independent from myocardial velocity. Strain is expressed as the percentage change in length from the original length. Strain rate is the rate of deformation (per second). Assessment of values from several segments can be done simultaneously. Strain imaging has been shown to be able to quantify RV function in patients with CHD [with CHD  and to dwith CHD  and in ywith CHD . Weidemawith CHD . Deformawith CHD –43 and iwith CHD .Two different technologies are currently available for studying regional myocardial deformation. The first technique is based on TDI and calculation of myocardial velocity gradients. The second is based on tracking speckles on the grey-scale images from frame to frame throughout the cardiac cycle and calculating the displacement of the speckles throughout the cardiac cycle (speckle tracking).TD-derived deformation imaging is based on assessment of differences in tissue velocities within myocardial segments and reflects either lengthening or shortening of the segment in the longitudinal, radial, or circumferential dimension. The strain rate is calculated as the velocity difference between two or more points within the myocardial segment divided by the distance between them, assessing the velocity gradient within the segment. Such velocity gradients actually quantify the instant regional deformation within the segment, expressed as the strain rate. The temporal resolution is high, and higher than for two-dimensional speckle tracking techniques and for MRI. The disadvantage of TDI-derived deformation imaging is that it is angle dependent and requires postprocessing . MeasureSpeckle tracking is based on grey-scale images and is relatively easy to perform. In contrast to TDI-derived parameters, speckle tracking is an angle-independent technique as the movement of speckles can be followed in any direction within the 2D grey scale image. As the method is based on recognition of speckles between frames, there are upper and lower limits for the frame rate. The frame rate has to be high enough so that speckles are recognizable between frames; if the frame rate is too low, then the speckles might change too much between frames or the speckles might not be recognized due to movement out of the plane. The temporal resolution is low compared to TDI, which might be an issue especially in smaller children with higher heart rates. Current two-dimensional longitudinal deformation analysis is to large extent based on assessment of the AV-valve plane motion. Regional differences might therefore not be detected, but, at the same time, the global values might be less influenced by local or regional artefacts. Software from different vendors can yield different results . Good coTissue velocities and strain measurements have been shown to be useful to quantify global and regional RV function in adult pulmonary arterial hypertension (PAH) patients. The more severe the PAH, the lower the end-systolic longitudinal strain in the RV free wall , 63, demTissue tracking of cardiac magnetic resonance imaging (CMR) is a novel method with only little published research on this topic –73. Niemr = 0.66) and RVEDV (r = 0.74) determined by either 3D echocardiography or MRI measurements [Three-dimensional (3D) echocardiographic rendering of cardiac structures of patients with CHD has gained interest during recent years with the development of high-frequency paediatric probes with improved image quality . Using turements . The useurements , 87. Morurements . Normal urements . 3D echourements . Validaturements . Sorianourements . The 3D urements .Friedberg and Silverman described the Doppler-derived mitral regurgitation time interval ratio of systolic (S) duration to diastolic (D) duration (S/D) . The S/DLimitation of S/D duration ratio is that the measurement of this ratio to assess ventricular function requires the presence of defined onset and end of MR/TR on spectral Doppler tracings.Sheehan and Redington recently reviewed that the RV differs from the LV in its physiology with the LV output being less sensitive to afterload changes than the RV . They sur = 0.90) [z-score < −2 SD), an inverse and significant correlation was found between TAPSE and RVEF measured by MRI in 88 children (r = 0.54) [r = 0.48) [r = −0.42; P < 0.001) in TOF and PAH-associated with congenital heart disease (CHD) patients as shown in The TAPSE is an indicator of ventricular contractile function that correlates well with RVEF in adults  and can  = 0.90) . Data ha = 0.90) . They sh = 0.90) . A signi = 0.90) . New ech = 0.90) . An abno = 0.90) . Adult s = 0.90) , 117; it = 0.90) . Recentl = 0.90) . TAPSE a = 0.90) , 119. Th = 0.90) . In an o = 0.90)  as shown = 0.90) , as show = 0.90) . In TOF  = 0.54)  and in a = 0.48) . The corOne limitation is that, although TAPSE appears to be a good indicator of global RV function, it does not take into account segmental RV function and contractility. A major limitation to any novel assessment of RV systolic function is the lack of an acceptable quantitative echocardiography standard of comparison.The future of newer echocardiographic techniques, such as tissue Doppler imaging (TDI), deformation imaging (tissue tracking), myocardial acceleration during isovolumic contraction (IVA), S/D duration ratio, 3D echocardiography, and measurements of systolic RV function, depends on further validation and demonstration of clinical utility. There is still a long way to go before relevant clinical decisions can be based solely on these new echocardiographic measures. In particular, prospective comparative studies and well-designed randomized controlled trials with such variables as primary and secondary outcomes are desirable. However, the new imaging methodologies already offer new insights into the effects of congenital heart disease on LV and RV function and mechanics. The assessment of ventricular function by echocardiography is an area that is undergoing intensive research, as the development and implementation of new modalities become available. Cardiac sonographers should become familiar with both traditional and newer techniques such as tissue Doppler imaging, deformation imaging, S/D duration ratio, 3D echocardiography, and measurements of systolic RV function, for more detailed assessments of ventricular performance.


Non-randomized studies have reported a prognostic advantage with percutaneous coronary intervention (PCI) in the treatment of chronic total occlusions (CTO). However failure to cross and successfully open a CTO confers a worse clinical outcome. Quantitative assessment of global left ventricular (LV) function in those with failure to open a CTO has not demonstrated deleterious alterations in cardiac function/remodeling. We hypothesized that more sensitive techniques that assess regional wall motion, such as strain imaging, may identify segments where cardiac function is adversely affected in those with failure to open the CTO.30 patients referred for PCI to a single vessel de novo CTO underwent CMR examination before/after PCI. Left ventricular (LV) function and transmural extent of infarction (TEI) were assessed using standard cine SSFP and late gadolinium enhanced T1-weighted imaging on a 1.5T MRI system. Regional cardiac function was assessed from cine SSFP images using Velocity Vector Imaging software  on 20 patients. Data from each segment was summated and presented as global longitudinal, circumferential and radial strain/strain rate. All data was analyzed blind to the outcome of PCI. Data is presented as mean +/- SD or percent change [delta (Δ)] in final value from baseline.Successful CTO opening (TIMI 3 flow) occurred in 63% of patients. Ejection fraction (EF) increased post PCI within the successful group (Δ= +12 ± 20%), p < 0.0003). Within the failed group there was no change EF (p = 0.09). In those with dysfunctional but viable segments (25%) successful PCI increased EF (p = 0.04) in contrast to failed revascularization where the EF was not different.Regional cardiac function varied significantly. Global longitudinal strain (GLS) fell significantly in the failed group  in contrast to successful PCI where GLS did not change . Global longitudinal strain rate followed a similar pattern to GLS . In contrast, radial and circumferential strain/strain rate were not different between groups after success/failed PCI (see table).In this cohort, regional cardiac function assessment using Velocity vector imaging demonstrated a significant decline in GLS and GLSR in patients in whom PCI failed to open a CTO, with no change in global measures of cardiac function. Larger, randomized studies are required to assess the long-term benefits and morbidity of PCI in the treatment of CTOs, and potential impact of failure upon function and outcomes.Funding was provided in part by the CIHR, Canada.


Long term survival benefits from CTO revascularisation has been suggested in several large observational studies despite a significant failure rate1,3. Data about the physiological consequences of successful opening of a CTO are limited and heterogeneous4. CMR imaging can provide quantitative information on MBF  in this context.The perceived clinical benefits of undertaking revascularisation to coronary artery chronic total occlusions (CTO) relate to improvements in angina symptoms and prognosisTo determine changes in hyperaemic myocardial blood flow in patients undergoing revascularisation of CTO.5. Following motion correction, a circular ROI was selected in the left ventricle to measure the arterial input function. MBF maps were created by model-free analysis. Two myocardial ROIs were drawn on these maps, one in the CTO territory and one in a remote normal region. MBF for these ROIs was calculated using the Fermi model6. Statistical calculations were performed using SPSS.Twenty patients with CTO were recruited from clinical waiting lists and underwent perfusion-CMR before and after attempted CTO revascularisation on a Philips 1.5 T Intera system. Perfusion imaging was performed every heartbeat during the first pass using a T1-weighted fast (spoiled) GE sequence in 3 short-axis imaging planes. Stress perfusion imaging was performed using IV adenosine for 4 minutes (at 140mcg/kg/min) and 0.05 mmol/kg of gadolinium chelate via a power injector. The dynamic contrast enhanced image data were post-processed off-line using the software PMI 0.4In 16 of the 20 patients, revascularisation was successful but failed in 4. There were highly significant improvements in adenosine-stress myocardial perfusion in the perfusion territories of the revascularised vessels. The improvements in myocardial blood flow were confined to the segments that were revascularised (p<0.001), whilst there were no changes seen in the remote myocardium (p=0.176). Figure Stress perfusion CMR demonstrates significant improvements in hyperaemic MBF in patients with successful revascularisation of CTO.


The purpose of this study is to assess the potential value of T1 mapping in assessing fibrosis in hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM) in comparison to late contrast enhanced imaging using cardiac MR.Myocardial fibrosis causes 1) increased stiffness and induces pathological signaling in cardiomyocytes resulting in progressive cardiac failure, 2)impairs mechano-electric coupling of cardiomyocytes and increases the risk of arrhythmias. T1 mapping allow to quantify the total amount of myocardial extracellular volume and to subsequently define the amount of myocardial fibrosis. Quantification of fibrosis in dilated and hypertrophic cardiomyopathies and it's link to delayed myocardial enhancement is unclear.Twelve patients with HCM, 10 with DCM, and 5 controls subjects were prospectively included. All patients underwent cardiac MR with the following sequences (parameters set according to recommendation of the SCMR): 1) cine sequence, 2) Molli sequence  acquired before and 5, 7, 9 min after gadolinium injection . 3) Late gadolinium enhanced sequence 10min after injection. T1 values were assessed in blood and in the 6 segments of the mid LV slice. Patient with (+) and without (-) enhancement on LGE were analysed separately. Were calculated: R1, ΔR1, ΔR1 ratio .in controls before injection T1 value was 0,98s ±0,05 s in myocardium, and 1,63 s ±0,08 in blood. λ was measured at 0.43±0,03 in normal. In DCM, λ was not significantly different between patient with  and without  enhancement on LGE (p=0.2). In HCM, λ was significantly different between patient with  and without  enhancement on LGE (p<0.0001)(figure T1 mapping allow for quantification of the total amount of fibrosis in a myocardium. In DCM the distribution of fibrosis might be more diffused compared with HCM. These results have to be confirmed by larger study.PHRC national 2011, AORC excelence 2011.


Cardiac fibrosis is a known marker of adverse remodeling of the heart. Late-gadolinium-enhanced (LGE) T1 mapping is the only proven method to assess diffuse myocardial fibrosis. The most widely used LGE cardiac T1 mapping pulse sequence is MOLLI, which is based on inversion-recovery (IR) magnetization pre-conditioning and Look-Locker imaging. Unfortunately, MOLLI is sensitive to heart rate and rhythm and T2 effects and requires a long breath hold duration. We present an arrhythmia-insensitive, rapid (AIR) cardiac T1 mapping pulse sequence which is also insensitive to T2 effects.We developed the AIR cardiac T1 mapping pulse sequence based on B1-insensitive saturation recovery (SR) magnetization pre-conditioning (insensitive to heart rate-rhythm) and two single-shot balanced steady-state free precession (b-SSFP) image acquisitions (proton density and T1-weighted) with centric k-space ordering . We compared its performance against MOLLI in an arrhythmia phantom  with an effective heart rate of 111 beat-per-minute (bpm) and in ten human subjects and 17 large animals in sinus rhythm pre-contrast and 5, 10, and 15 min post contrast agent (0.1 mmol/kg of Gd-BOPTA) administration.Compared with the reference T1 measured by IR fast spin-echo pulse sequence in the phantom, MOLLI and AIR T1 measurements had maximum coefficient of variation (CV) of 10% and 0.8%, respectively, at arrhythmia and normalized root-mean-square-error (NRMSE) of 22% and 3%, respectively, at arrhythmia. For in vivo, mean heart rates in humans, dogs, and goats were 57±9 bpm, 86±15 bpm, and 107±15 bpm, respectively. Compared with AIR T1 maps, MOLLI T1 maps yielded lower values but more T2 blurring (Fig.Our findings in vitro and in vivo suggest that AIR is more accurate than MOLLI for cardiac T1 mapping. This rapid cardiac T1 mapping pulse sequence may be clinically useful for assessment of diffuse myocardial fibrosis in patients with rapid heart rates and/or in arrhythmia.American Heart Association: 0730143N; Ben B. and Iris M. Margolis Foundation.


Objective. To ascertain pathological information on hyperplasia of mammary glands (HMG) of patients via the infrared radiation temperature of acupoints. Method. Patients with HMG and healthy controls were tested using an infrared thermal imager. Results. In controls, no significant difference in temperature was observed between points with the same name (P > 0.05). The temperature of all tested points was found to be higher in the group with HMG than in that of the healthy controls, except for the left and right Zusanli (ST36). The temperature of the right Rugen (ST18), Guanyuan (CV4), Qihai (CV6), and Hegu (LI4) reached a statistically significant heightened level (P = 0.046~P < 0.001). The temperature of the Zusanli (ST36) and Hegu (LI4) present on the right side was significantly higher than that of the left , while the temperature of the left Youmen (KI21) was significantly higher than that of the right (P = 0.008). Conclusion. The temperature of the bilateral acupoints in healthy controls was symmetrical, and the raised temperatures observed of the Rugen (ST18), Guanyuan (CV4), Qihai (CV6), and Hegu (LI4) acupoints of HMG patients and the imbalance of the temperature of the bilateral acupoints Zusanli (ST36), Youmen (KI21), and Hegu (LI4) carried special pathological information about HMG disease.  Acupuncture points are sites where qi and the blood of zang-fu organs and meridians are transported. The changes in qi and blood can be reflected in these acupoints. Thus, acupuncture points not only can receive stimulation by acupuncture and moxibustion but also may reflect disorders in patients. Therefore, when there are disorders in zang-fu organs, there could be tenderness, pain, soreness, numbness, or thermosensitivity in the related acupoints. The changes can also be reflected as changes in the biophysical character of the acupoints, such as changes in the volt-ampere characteristics of the points , 2 or anP = 0.631). Detection time was from September 2008 to November 2008 and from August 2009 to December 2009.This study included 101 female HMG patients from the breast clinic of Longhua Hospital, which is affiliated to the Shanghai University of Traditional Chinese Medicine. All of them volunteered to participate in the program and signed informed consent forms. Out of these patients, 73 had bilateral HMG, 13 had only right-lateralized HMG, and 15 were only left-lateralized. The youngest patient was 21 years old and the oldest was 51; the mean age of the sample was 33.14 ± 8.001 years. Thirty-five healthy controls who participated in this study were all interns and staff members of the hospital working at different posts. Seven were medical care workers and the remaining 28 were from house-keeping, finance, and the catering departments. In the control group, the youngest participant was 18 years old and the oldest was 49. The mean age of the control group was 32.23 ± 10.085 years. There was no significant difference in the age distribution between the two groups  was used. It uses the focal plane array (FPA) principle, an uncooled microbolometer, and has ultra-high thermal sensitivity (0.08°C). The temperature measuring range is 0–500°C; the IFOV, 1.3 mrad; and the wavelength range, 7.5–13 According to the national standard GB12346-90 Acupoints Location released by the State Bureau of Technical Supervision in 1990 , we chosPatients entered the lab, loosened their underwear, and sat down for 20 min to adapt to the environment. During this period, they are asked questions to obtain the relevant required information. After questioning, the patients exposed their testing sites, and the analyser sat 1 meter away from the testing site in a fixed position prepared to film the procedure. The infrared camera was placed right above the testing site to obtain infrared images. t-tests for comparison between patients and healthy controls, and paired t-tests for comparison between bilateral acupoints with the same name (α = 0.05).The thermaCAM reporter 2000 professional analysis system, matching the infrared thermography, was used to analyse the infrared thermograph picture and obtain the skin temperature at the detected acupoints; Microsoft Excel was used to construct a database and the SPSS 11.5  software package was used to analyse the statistical data. We used mean and standard deviation to analyse the measurement data, group P = 0.046~P < 0.001) , the infrared radiation temperature of 10 out of 12 testing points was higher than that of the healthy controls. Among these 10 testing points, the temperature of the right Rugen (ST18), Guanyuan (CV4), Qihai (CV6), and bilateral Hegu (LI4) was significantly higher than that of the healthy controls (< 0.001) . P > 0.05)  and Qimen (LR14) on the left side were higher than those on the right side, while the temperature of the other 3 acupoints on the right side was higher than those on the left side. However, the difference was not statistically significant ( > 0.05) . P = 0.008), while the temperature of Hegu (LI4) and Zusanli (ST36) on the right side was significantly higher than that on the left side  , Qimen (LR14), and Youmen (KI21) on the left side was higher than that on the right side, while the temperature of the other 2 acupoints was higher on the right side than that on the left. The temperature of Youmen (KI21) on the left side was significantly higher than that on the right side (= 0.004) . Zhen Jiu Jia Yi Jing that: “Therapy of breast pain and swelling of the chest lies in the Rugen point.” Pu Ji Fang mentioned that “Therapy of breast pain lies in Rugen point.” Nowadays, many textbooks [P < 0.05). Researchers [It's recorded in the extbooks –11 take extbooks , 13 haveextbooks . Youmen extbooks  in the Cextbooks . Therefoearchers , 18 repoXi Hong Fu says: “Unbearable pain of the hands and shoulders should be treated with Hegu and Taichong.” Sheng Yu Ge says: “Soreness and pain of the hands, which makes it hard to hold things, should be treated with Hegu, Quchi, and Jianyu.” There are also reports on Hegu (LI4) for pain treatment in a modern clinic [P > 0.05). This was consistent with the symmetry law of bilateral, normal, skin temperature, while the infrared radiation temperature difference of bilateral Youmen (KI21), Zusanli (ST36), and Hegu (LI4) of HMG patients was statistically significant (P < 0.05). This showed the temperature imbalance phenomenon of bilateral acupoints of HMG patients and indicated the pathological nature of liver qi stagnation, phlegm and blood stasis, and Chong and Ren meridian disturbance of HMG patients. There have been ancient records for Hegu (LI4) on the treatment of painful diseases. n clinic –22. Resen clinic  proved tn clinic . He belin clinic  thought n clinic . Yan's sn clinic , 28. He The infrared radiation temperature of the bilateral acupoints in healthy controls was found to be symmetrical, and the raised temperatures observed of the Rugen (ST18), Guanyuan (CV4), Qihai (CV6), and Hegu (LI4) acupoints of mammary gland hyperplasia patients and the imbalance of the infrared radiation temperature of the bilateral acupoints Zusanli (ST36), Youmen (KI21), and Hegu (LI4) carried special pathological information about mammary gland hyperplasia disease. These results can be used as an auxiliary diagnosis for mammary gland hyperplasia. This experiment provides new research ideas and methods for TCM diagnosis using acupoints, the research of acupoint specificity, acupoint selection at the clinic, and the objective research on the evaluation of curative effectiveness.


Delayed gadolinium enhancement (Gd-DE) is widely used to detect scar formation following myocardial infarction (MI) , but canex vivo. Infarcted mice (n=7) were imaged in vivo. MI was produced in C57BL6 mice via permanent ligation of the left coronary artery. In vivo DTI was performed on a 9.4T scanner (Bruker) using a 3D fat-suppressed single-shot 3D spin echo EPI sequence with motion-compensated diffusion-encoding gradients in 24 directions. Other parameters were: TR/TE=2000/13.5 ms, b-value 500-700 s/mm2 and isotropic resolution of 280 μm. The human and sheep hearts were imaged on a clinical 3T Siemens scanner with an isotropic resolution of 2 mm3, TR/TE=8430/96 ms, and a b-value of 2000 s/mm2. The tractographic propagation angle PA was defined as the angle between two adjacent principal eigenvectors  relative to a given fiber  and infarcted sheep hearts (n=6) were studied r Figure . PA valu2=0.94) was seen between percent infarct size by Gd-DE and tractographic PA (Figure Tractography of a normal human heart color-coded by the PA is shown in Figure A Figure .in vivo. The use of DTI, and hence the tractographic PA, does not require exogenous contrast and can be performed in all patients regardless of renal function. The technique provides a complementary and valuable adjunct to Gd-DE.PA detects the loss of tract coherence in infarcted myocardium and robustly delineates myocardial infarcts R01HL093038


Dyssynchronous myocardial motion aggravates cardiac pump function. Cardiac resynchronization using pacing devices is a standard-of-care in the management of heart failure. Post-infarction, however, scar tissue formation impedes the efficacy of device-based therapy. The present study tests a regenerative approach aimed at targeting the origin of abnormal motion to prevent dyssynchronous organ failure. Induced pluripotent stem (iPS) cells harbour a reparative potential, and were here bioengineered from somatic fibroblasts reprogrammed with the stemness factors OCT3/4, SOX2, KLF4, and c-MYC. In a murine infarction model, within 30 min of coronary ligation, iPS cells were delivered to mapped infarcted areas. Focal deformation and dysfunction underlying progressive heart failure was resolved prospectively using speckle-tracking imaging. Tracked at high temporal and spatial resolution, regional iPS cell transplantation restored, within 10 days post-infarction, the contractility of targeted infarcted foci and nullified conduction delay in adjacent non-infarcted regions. Local iPS cell therapy, but not delivery of parental fibroblasts or vehicle, prevented or normalized abnormal strain patterns correcting the decrease in peak strain, disparity of time-to-peak strain, and pathological systolic stretch. Focal benefit of iPS cell intervention translated into improved left ventricular conduction and contractility, reduced scar, and reversal of structural remodelling, protecting from organ decompensation. Thus, in ischaemic cardiomyopathy, targeted iPS cell transplantation synchronized failing ventricles, offering a regenerative strategy to achieve biological resynchronization. The pumping function of the heart depends on ordered initiation and propagation of myocardial excitation. Cardiac output is compromised by inconsistent timing and direction of wall motion, leading to dyssynchrony and organ failure.Myocardial infarction induces irreversible heart damage. Extensive damage hampers effective pacemaker-based cardiac resynchronization therapy, the current standard-of-care. Establishment of alternative approaches is thus warranted.High-resolution imaging was here utilized to non-invasively map suitable therapeutic targets within a dyssynchronous heart. Speckle-tracking echocardiography unmasked the source of progressive cardiac dyssynchrony within the primary infarcted region.Bioengineered stem cells with a capacity to induce a regenerative response were implanted into infarcted areas. Speckle-tracking echocardiography and histology assessment revealed that cell therapy achieved cardiac resynchronization and long-term repair.This proof-of-concept study thus introduces a stem cell-based regenerative solution to address cardiac dyssynchrony post-infarction.Cardiac pump function relies on coordinated myocardial motion secured through ordered electromechanical activation . DevelopRegenerative interventions are increasingly considered in the management of ischaemic cardiomyopathy . In thisAll protocols were carried out under the National Institutes of Health guidelines with approval obtained from the Institutional Animal Care and Use Committee, and the Biosafety Committee at Mayo Clinic. All procedures on living animals were conducted under general inhalation anaesthesia. Animals demonstrating signs of organ failure were removed from the study for humane considerations and sacrificed with carbon dioxide. Following recommendations of the American Veterinary Medical Association and the Institutional Animal Care and Use Committee, all animals were sacrificed with carbon dioxide at the end of the study.in vitro , in vivo (teratoma formation) and in utero (diploid aggregation and contribution to organogenesis) criteria  2 days prior to and 5 days after surgery. Post-ligation, infarcted mice  were randomized into vehicle-treated (25 athymic nude), fibroblast-treated , and iPS cell-treated  cohorts. Fibroblasts or derived iPS cells  were delivered into mapped peri-infarcted anterior walls of the left ventricle (LV) by epicardial injection  within 30 min following coronary ligation  as described  up to 3 months post-infarction (−1 and a frame rate >180 s−1 (average heart rate of 478 ± 14 beats min−1 and frame rate of 219 ± 11 s−1). Ninety-five per cent of sampling points were successfully tracked under pre-established criteria, and further analysed for peak systolic strain/velocity and time-to-peak strain/velocity. Strain (ɛ) was defined as change in length during myocardial contraction and relaxation, and expressed as a percentage: ɛ= (L1–L0)/L0=ΔL/L0, where L0 is original length, L1 is final length, and ΔL is change in length . Cardiac cycles were acquired digitally from the parasternal long-axis and mid-ventricular short-axis views for assessment of radial, circumferential and longitudinal systolic strain/velocity, and time-to-peak systolic strain/velocity . The LV −1 D-luciferin i.p., Xenogen, Alameda, CA, USA), and β-galactosidase antibody  colocalized with α-actinin  and 4′,6′-diamidino-2-phenylindole (Molecular Probes) as previously described . Kaplan-Meier analysis with log-rank testing was applied for survival analysis. P < 0.05 was predetermined as significant.Data are presented as means ± SEM. Paired group analysis was performed using Student's in vivo to murine hearts beating at physiological rates (387–560 beats min−1), enabled tracking of tissue speckle patterns , yet it improved to 41 ± 4% in iPS cell-treated counterparts , significantly higher than 17 ± 2% measured in the fibroblast-treated cohort  to 68.7 ± 4.3% at 3 months  were superior in iPS cell- versus fibroblast-treated ventricles. Fibroblast-treated ventricles had extensive scar in the dyssynchronous anterior-septum  were delivered into acutely infarcted regions in a murine model. High-resolution speckle-tracking echocardiography unmasked global and regional dynamics of cardiac wall motion