We aimed to evaluate the predictive power of longitudinal and circumferential fibers according to left ventricular ejection fraction (LVEF) in successfully reperfused acute ST elevation myocardial infarction (STEMI) patients.Total 691 patients  underwent clinical evaluation and conventional and strain echocardiography , global circumferential strain (GCS)). The clinical outcome was defined as the composite of death, hospitalization for heart failure, non-fatal myocardial infarction, and ventricular arrhythmia.2 = 31.8→45.8, p<0.001), although GCS offers no additional benefit. In the subgroup analysis according to LVEF, adjusted with clinical factors, GLS was significant predictive for outcome for the patients with mildly depressed  and significantly depressed  systolic function, although GCS and LVEF lost their power with LVEF<40%. For the patients with preserved LVEF (>50%), GLS, GCS and LVEF did not show significant predictive power.During a follow-up of 39±19 months, there were 47 (6.8%) clinical events. In multivariate Cox models adjusted clinical risk factors, age  and GLS  were independent predictors. The addition of GLS resulted in significant incremental improvement in the predictive value on LVEF . Left ventricular (LV) systolic function is an important prognostic marker after ST elevation myocardial infarction (STEMI). Left venLV systolic function is a complex, coordinated action involving longitudinal contraction, circumferential shortening, and radial thickening. Two-dimensional (2D) strain based on speckle tracking is an emerging innovative method providing information about the functional status of the left ventricle after AMI. Global longitudinal strain (GLS) has been discussed in numerous studies as a superior marker compared with LVEF for cardiac events, functionContraction of muscle fibers in the mid-wall, which is linearly related to circumferential strain, may bettIn contrast, GLS has been introduced as a prognostic marker in AMI patients with preserved LV systolic function. However, the study had an inclusion criteria of EF > 40%, therefore including patients with actual LV dysfunction. In the cWe hypothesized that both GLS and GCS will play an important role predicting clinical events in STEMI patients regardless of LV systolic dysfunction. We sought to evaluate the predictive power of longitudinal and circumferential fibers according to LV systolic function in successfully reperfused STEMI patients with low clinical risk.Between April 2006 and July 2012, 802 patients admitted for STEMI were enrolled at four cardiovascular centers. STEMI was defined following previous clinical guidelines.–[end-diastole])/ L(end-diastole) × 100, where the global strain is the change of the whole myocardium, not an averaged value of each segmental strain, and L is the whole LV myocardium as one big segment. GLS was averaged from global strains from the apical 4-, 3- and 2-chamber views. GCS was averaged from 3 short axis  views.All patients were seen within 4 weeks post-discharge and every 2 to 4 months thereafter for the duration of the follow-up period. The composite outcome included all-cause death, heart failure hospitalization, non-fatal myocardial infarction, and ventricular arrhythmia. Each factor was also analyzed individually.All data are expressed as mean value ± standard deviation, if not otherwise specified. Outcome data were compared between subjects by Cox-regression model. P-values less than 0.05 were considered to indicate statistically significant differences. To minimize correlation effects, we used 5 Cox proportional hazards models, and analyzed LVEF, GCS, and GLS separately. Model 1 only adjusted for age. Model 2 included adjustments for age, hypertension, DES (drug eluting stent) implantation, and WMSI . Model 3 adjustments included factors in model 2 and LVEF, and Model 4 adjusted factors in model 2 and GLS. Model 5 included adjusted factors in model 2 and GCS. The incremental prognostic value was defined by a significant increase in global chi-square.Receiver-operating characteristic (ROC) curve analysis was used to determine optimal cutoff values of continuous variables. The best cutoff value was defined as the point with the highest sum of sensitivity and specificity. The subjects were divided into three groups according to their LVEF as preserved (EF > 50%), mild depressed (40–50%), and significantly depressed (EF < 40%).All analyses were performed with IBM SPSS Statistics Version 21.0 .Of total 691 patients, and global strain was acquired in 678 (98.1%) patients for longitudinal strain and in 653 (94.5%) patients for circumferential strain. Variability in the measurement of strain was evaluated in 20 randomly selected patients. For intra-observer variability, the same observer measured strain for each of the selected patients again 15 days later. The interclass correlation coefficiency of intra-observer variability for GLS and GCS were 0.96 and 0.94 respectively. For the inter-observer variability, a second independent observer repeated the analysis. The interclass correlation coefficiency of inter-observer variability for GLS and GCS were 0.94 and 0.88 respectively.Total 691 consecutive patients (mean age 59.3 ± 13.1) satisfied all inclusion criteria. During mean follow-up duration of 39 ± 19 months, clinical events occurred in 47 patients (6.8%), including 19 deaths (12 cardiac deaths), 14 heart failure hospitalizations, 13 myocardial infarctions, and 1 non-fatal ventricular arrhythmia. The clinical and echocardiographic characteristics are described in By univariate analysis using Cox proportional hazards model, age, hypertension, DES implantation, WMSI, LVESD (left ventricular end systolic diameter), LVEF, and both GLS and GCS were significantly associated with composite outcome. The hazard ratio (HR) of each variable is shown in To exclude confounding effects of clinical risk factors, subgroup analyses of each factor were performed. Detailed data according to each factor is described in By analyzing the ROC curve, the best cut-off points of GLS  and GCS  were -9.9%  and -12.6% , respectively. Patients with GLS>-9.9% and GCS>-12.6% showed 8.0 and 3.0 times higher risk of composite events than patients with GLS ≤9.9% and GSC ≤12.6%, respectively.Patients were categorized into three groups according to LVEF (preserved / mildly depressed / significantly depressed). In survival analysis according to LVEF, GCS lost its predictive power. GLS was the most powerful independent predictor for patients with mildly and significantly depressed LVEF, but lost its significance in the preserved LVEF group . Among pThere were 19 (2.7%) deaths (including 12 cardiac deaths), 14 (2.0%) hospitalizations for heart failure, 13 (1.9%) cases of myocardial infarction, and one (0.1%) non-fatal ventricular arrhythmia. The GLS, GCS, and LVEF mean values for composite outcomes and each individual components are described in Cox-regression survival analysis for composite outcomes and each individual components, using model 2, showed GLS to be the most powerful predictive power, rather than GCS or LVEF. Although GLS, GCS, and LVEF were predictors for composite outcome, their prediction power was best for cardiac death . DetaileThe role of global 2D-strain in AMI patients engaged clinicians’ interest and a number of researches were reported recently.–15 GlobaWe have shown that GLS and was the best independent prognostic factors to predict composite events in successfully revascularized STEMI patients. GLS showed incremental predictive effect in addition to LVEF. Subgroup analysis according to baseline LV systolic function, for the patients with significantly depressed LV systolic function (<40%), GLS showed the powerful predictive power, although GCS and LVEF lost predictive power.After successfully reperfused therapy in STEMI, dysfunctional myocardial segments subtended by the infarct-related artery can follow two different natural courses: functional recovery or irreversible remodeling. IrreversThe GLS did not predict outcome for the patients with preserved LVEF (LVEF >50%). In the present study the most important reason LVEF lost its predictive power in the group with preserved LVEF was, that there were only 13 (3.6%) clinical events among 356 patients, which was too small a number to have statistical power. when we set the cut-off value of preserved LVEF as 40%, GLS showed significant predictive power as previously reported.[Contraction of muscle fibers in the mid-wall, which is linearly related to circumferential strain, may bettp = 0.266). In the univariate and multivariate analyses for study outcome, QRS duration could not predict clinical outcome and the predictive power of LVEF and global strains did not change although we corrected QRS duration as confounding in Cox-regression analyses , and the history of previous PCI or CABG. Infarct size has been known to be a valuable surrogate marker for patients with myocardial infarction. However a study which has investigated predictors of 6-month cardiac events after myocardial infarction from the Korea Acute Myocardial Infarction Registry (KAMIR) showed that cardiac biomarkers did not predict clinical outcome. In our sanalyses . Also, wanalyses ). There A number of limitations of this analysis should be noted. Cardiac death was significantly lower than previous published data. Although this was a prospective analysis performed at multi-center, lots of patients (n = 107) with mechanical support, ongoing cardiogenic shock, age over 85 or cardiac arrest at admission, were excluded from the analysis. It could be a baseline selection bias. This meant the patients who could be thought to have poorer outcome were already excluded before analysis. The patients in our study are those who would benefit most from the right treatment strategy according to the outcome prediction. Following strict inclusion criteria which could lead a selection bias, we were able to control various confounding factors that could influence prognosis. All study patients were stable enough to perform routine echocardiography at a dedicated echocardiogram exam center. Even so, 1 patient had a poor echocardiography window, and was excluded from the study. LVEF was acquired in all 691 patients, and global strain was acquired in 678 (98.1%) patients. Secondly, we could not undergo the analysis of scheduled follow-up echocardiography, we could not definitely address 2D-strain is associate with LV remodeling in our cohort. Finally, vagaries of coronary anatomy, morphology, combined non-culprit stenosis, or TIMI score for STEMI patients are important factors in the aspect of clinical prognosis. We did not reflect these interventional data on our study, and it might affect the results as confounding bias.GLS is a powerful predictor of clinical events and appears to be a better parameter than ejection fraction in successfully reperfused STEMI patients, especially with LV dysfunction (LVEF ≤50%), although GCS and LVEF lost their predictive power for the patients with significantly depressed LV function (LVEF <40%) These data further support the concept that longitudinal function is sensitive to acute myocardial damage in STEMI patients. However GLS did not predict outcome for the patients with preserved LVEF (LVEF >50%).S1 Table(DOCX)Click here for additional data file.S2 Table(DOCX)Click here for additional data file.S3 Table(DOCX)Click here for additional data file.S4 Table(DOCX)Click here for additional data file.S5 Table(DOCX)Click here for additional data file.S6 Table(DOCX)Click here for additional data file.


Although she had no symptoms and no history of hypertension, echocardiography demonstrated severe LVH in the mid LV to the apex with no asymmetric septal hypertrophy. Thus, we suspected apical type of HCM and performed FDG-PET/MRI. After fasting for more than 18 hours, the patient was administered unfractionated heparin 50 IU/kg intravenously 15 minutes before FDG administration to suppress physiological FDG uptake on the myocardium.Cine MRI of 2-chamber view. Severe myocardial hypertrophy was observed in the left ventricle with the exception of base and apex. Myocardial thinning and akinetic wall motion were detected in the apex. Supplementary material 1 (WMV 177 kb)Cine MRI of 4-chamber view. Marked left ventricular hypertrophy in the middle region, and myocardial thinning and dysfunction in the apex. Right ventricular function was normal with no right ventricular hypertrophy. Supplementary material 2 (WMV 146 kb)Below is the link to the electronic supplementary material.


The biological role of the ductus arteriosus (DA) in neonates varies from an innocent bystander role during normal postnatal transition, to a supportive role when there is compromise to either systemic or pulmonary blood flow, to a pathological state in the presence of hemodynamically significant systemic to pulmonary shunts, as occurs in low birth weight infants. Among a wide array of clinical manifestations arising due to the ductal entity, systemic circulatory insufficiency and hypotension are of significant concern as they are particularly challenging to manage. An understanding of the physiologic interplay between the DA and the circulatory system is the key to developing appropriate targeted therapeutic strategies. In this review, we discuss the relationship of systemic hypotension to the DA, emphasizing the importance of critical thinking and a precise individual approach to intensive care support. We particularly focus on the variable states of hypotension arising directly due to a hemodynamically significant DA or seen in the period following successful surgical ligation. In addition, we explore the mechanistic contributions of the ductus to circulatory insufficiency that may manifest during the transitional period, states of maladapted transition (such as acute pulmonary hypertension of the newborn), and congenital heart disease . Understanding the dynamic modulator role of the ductus according to the ambient physiology enables a more precise approach to management. We review the pathophysiology, clinical manifestations, diagnosis, monitoring, and therapeutic intervention for the spectrum of DA-related circulatory compromise. The ductus arteriosus (DA) has a multifaceted biological role in the context of hemodynamic instability in neonates that ranges from physiologic and supportive to pathologic Figure  1). Fou. Fou1). via the foramen ovale (2) and prostaglandin I2 (PGI2), within the lumen of the DA to ensure its ongoing patency. In addition, the intricate balance between the maternal uterine and fetal placental prostaglandin pathway expression profiles also drives the shunt direction.At birth, infants must navigate a series of complex adaptive sequences to successfully transition from fetal to postnatal circulation. The placenta serves as the organ of gas exchange as the lungs only receive a small amount of blood flow , 4. In ten ovale . The lefen ovale , 7] receAfter birth, the first breath of relatively oxygenated air results in lung expansion and clearance of fetal lung fluid. Mechanical stretch of the newly inflated lungs and relief of alveolar hypoxia dramatically decrease PVR, leading to an increase in pulmonary blood flow (PBF) . Clampinfunctional closure of the DA within the first 10–15 h after birth. In healthy infants, the functional closure triggers the subsequent process of anatomical closure that begins at the pulmonary end  promotes the constrictive effect of oxygen on the ductal tissue . This conary end  and is onary end , and theThe clinical significance of systemic hypotension must be carefully interpreted based on the variance in biologic role of the DA in different physiologic phases and disease states , 10. ThiIn clinical terms, neonatologists generally use one of the following three thresholds  to definSince MAP rises as gestational and postnatal age increase , a singlThe individual components of MAP, SAP, and DAP may provide more valuable insight into the overall wellbeing of the cardiovascular system as compared to MAP alone and may facilitate enhanced diagnostic consideration regarding specific causality , 10. SAPAn hsDA in the premature neonate can result in a wide spectrum of complex circulatory consequences that manifest with clinical signs of pulmonary overcirculation or systemic hypoperfusion as the volume of left ventricular output (LVO) is increasingly diverted from the systemic to the pulmonary circulation. The magnitude of transductal blood flow is modulated by the specific sub-components of Poiseulle’s law namely transductal pressure gradients, diameter, length, and viscosity. There are multiple mechanistic pathways that lead to the circulatory imbalance with an hsDA that can be characterized by the presence of diastolic and/or systolic hypotension , 10. TheIn the initial stage of systemic hypotension related to the hsDA, the LV myocardium adapts to increasing left atrial preload (from the increased pulmonary venous return) by increasing its stroke volume (SV). The hsDA will enlarge the vascular bed and lower net SVR, further augmenting the SV with a reduction in LV afterload. Clinically, this results in the “paradox” of high ventricular performance with increased pre-ductal CO , 30–32, In general, the premature myocardium is more sensitive to changes in afterload with an ineffective contractile mechanism and a steeper slope on the stress–velocity curve . In addiThe “ductal steal phenomenon” is another major contributor to circulatory imbalance. In the presence of a high systemic to pulmonary pressure gradient, an hsDA allows a high volume left-to-right shunt to “steal” blood away from the systemic circulation and into the pulmonary bed. This progressive steal phenomenon will lead to systemic circulatory insufficiency that can further compromise end organ perfusion, particularly in the postductal organs such as kidneys and gastrointestinal tract , 37–40. The presence of diastolic and/or systolic hypotension can provide insight into the degree of hemodynamic significance of the PDA; however, this approach is yet to be assessed systematically . Diagnos2 requirement . Li. Li74). Non-cardiovascular contributors to the PLCS pathophysiology, such as adrenal insufficiency, have also been investigated , 78, 79.A holistic appraisal with careful monitoring and appropriate interpretation of all the clinical and laboratory measures of cardiovascular wellbeing, in addition to the use of targeted neonatal echocardiography (TNE), is crucial in the immediate postoperative period. Clinical and biochemical signs of CO and end organ perfusion, such as tachycardia, capillary refill time, urine output, lactate, base excess, etc., are non-specific, but may provide clues as to the state of the underlying circulation . Blood pThe management strategy in the post PDA ligation period strives for prevention of PLCS with early modulation of the postoperative physiology of elevated afterload, reduced preload, and systolic and diastolic dysfunction . BaselinA different management strategy should be considered when a neonate presents with hypotension, and echocardiographic evidence of myocardial dysfunction ± low CO in the post-ligation period. The optimal approach is to augment contractility without increasing afterload. Intravenous dobutamine represents a rational choice for patients with isolated SAP whereas epinephrine may be preferable for patients with low MAP. Agents that increase afterload such as dopamine, vasopressin, and norepinephrine should be avoided. Milrinone therapy, although biologically plausible as a preventative agent, is undesirable in an already hypotensive neonate. The role of hydrocortisone in the postoperative period is controversial. There is no evidence to support preoperative use of stress dose hydrocortisone . Neonate2 and oxygen saturation gradients. Survival in acute PH depends on proper hemodynamic coupling of the RV with the compliant PA circulation. With the failure of PVR to fall and a non-compliant PA circulation, the RV adapts to the increased afterload in acute PH by raising ventricular contractility to maintain the hemodynamic coupling of the RV to the PA circulation. However, with the RV’s limited contractile reserve and a further rise in RV afterload contributing to increased systolic wall stress, ventricular contractility will eventually not be able to meet the demand and the RV will uncouple from its afterload with a decrease in RV efficiency  and low CO, despite initial normal LV and RV systolic performance . The rigficiency . In thisficiency . In addificiency . As a reficiency .via the DA (RV–DA–Aorta route), than from the traditional pathway through the pulmonary circulation to the left side of the heart. In patients with RV dysfunction where the DA is closed or restrictive, forward blood flow is limited by increased PVR and not shunted through the DA to support the systemic circulation, leading to a further deterioration in RV performance owing to increased systolic wall stress (Deterioration of RV performance may eventually affect LV function, in part due to shared myocardial fibers and ventricular–ventricular interaction . With adl stress . In thisl stress . TherapeThere are certain critical congenital cardiac lesions that can lead to acute cardiovascular collapse and rely on the supportive role of the DA to provide systemic and/or PBF Table . NeonateFor those infants diagnosed in the antenatal period with ductal-dependent CHD, PGE is initiated shortly after delivery . In neonIn congenital heart lesions that involve an anatomic obstruction to SBF, the DA is required to maintain adequate systemic perfusion . These lThe DA has a specific role in lesions with parallel circulations and poor mixing . With thThe role of the DA is more complicated in other mixing lesions, though the clinical presentation may still be cyanosis. In lesions with complete mixing in the presence of a left-to-right shunt at the DA, the source of cyanosis is often from increased PBF, which can lead to tachypnea and respiratory distress . In thesThe DA is essential in fetal life, important in early transition, potentially detrimental in select preterm populations, and supportive in specific disease processes in the structurally normal and abnormal heart. Cardiovascular compromise and hypotension may occur at each postnatal stage and the DA plays a dynamic role in modulating the neonatal circulation. Therefore, understanding the pathophysiologic basis of such instability arising due to the DA and early recognition of the typical clinical phenotype characterizing the various disease stages is essential for proper diagnosis, monitoring, and therapeutic support. In certain cases, the treatment for an hsDA can lead to serious cardiovascular compromise in premature neonates. Analyzing systolic and diastolic hypotension separately, instead of focusing solely on MAP, may provide early insight into the mechanistic contribution of the DA, which can then be evaluated in conjunction with using TNE. A holistic diagnostic approach that synthesizes clinical signs, biochemical values, and echocardiographic evidence can be utilized to devise focused, individualized, physiology-based treatment strategies that optimize management of these challenging patients and has the potential to improve patient outcomes.DR and SB drafted the first version and equally contributed to writing the first draft of the review. PL and PM are responsible for study conception and performed the critical revision. Each author has seen and provided final approval of the version to be published, with agreement to be accountable for all aspects of the work.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


The aim of the current study was to determine the effects of gadolinium contrast agent on right (RV) and left ventricular (LV) volumetric, aortic flow and pulse wave velocity (PWV) quantification using manual, semi-automatic and fully automatic analysis techniques.−1 gadoterate meglumine. The images were analysed manually, and using a semi-automated and a fully automated technique.61 participants free from known cardiovascular disease were recruited. Cardiac MR was performed on a 3 T scanner. A balanced steady-state free precession stack was acquired of the ventricles with phase contrast imaging of the aorta performed pre- and post-administration of 10 ml 0.5 mmol ml vs  post: 1028 ± 540, p = 0.003) with no significant change in contrast-to-noise ratio . On LV analysis, post-contrast analysis yielded significantly higher end systolic volume , and lower ejection fraction . On RV analysis, gadolinium contrast resulted in no significant differences. Similar results were seen using the semi-automated and fully-automated techniques but with a larger magnitude of difference. Conversely, using both manual and software analysis aortic flow and PWV quantification proved robust to the effects of contrast agent producing only small non-significant differences.54 completed the study. Gadolinium-based contrast administration significantly increase the signal-to-noise ratio (pre: 830 ± 398Gadolinium contrast administration significantly alters LV endocardial contour detection with this effect amplified when using semi-automated analysis techniques. In comparison, RV and PWV analysis is robust to these effects.Contrast administration alters LV quantification but not flow analysis. However, these differences are small. However, without exception normal reference ranges for ventricular quantification have been published based on sequences obtained without contrast agent.16 A literature search on the effects of contrast agents yielded conflicting results with some studies showing no changes in ventricular parameters before and after contrast administration while others reported a significant impact up on left ventricular (LV) quantification.19 In light of the small cohorts and the lack of right ventricular (RV) analysis within said studies, the validity of comparing values obtained after the administration of gadolinium-based contrast agents with non-contrast sequences is potentially questionable.The use of gadolinium-based contrast agents in cardiac MR (CMR) has become a common place due to its use in the detection, characterization and quantification of myocardial scarring.The effects of contrast agents on PWV analysis are similarly unknown. Furthermore, there is no evaluation of the effects of contrast agents on contour detection software, which are commonly used for post-processing purposes. Thus, to better delineate the effects of contrast agents on cardiac MR-based volumetric and flow quantification, this study aims to: (i) assess the effects of contrast agent on both LV and RV stroke volume (SV), end-systolic volume (ESV), end-diastolic volume (EDV), ejection fraction (EF) and left ventricular mass (LVM); (ii) to determine the effects of contrast on aortic flow and PWV measurements; (iii) to compare the effects of gadolinium contrast on these measures obtained using manual and automated techniques.–2) free from clinically apparent cardiovascular disease from the general population, following advertisement of the project in a University distributed newsletter. All images were reviewed and screened for evidence of regional wall motion abnormality or remodelling by an experienced radiologist, as well as careful analysis of EF to ensure absence of silent ventricular systolic dysfunction. All participants provided written informed consent for the study, which was conducted in accordance with the Declaration of Helsinki and was approved by a research ethics committee.The study recruited 61 study participants  with a 32-channel cardiac coil. Sequences were obtained for the quantification of ventricular mass and volumes and aortic flow both before and after contrast administration.2, FOV Phase = 84.4%, matrix = 256 × 256, parallel acceleration factor = 2, slice per breath-hold = 1.For the ventricular acquisition, a short axis stack was planned aligned to the atrioventricular groove. A balanced steady-state free precession acquisition was then performed in breath-hold from the atrioventricular ring to the apex . Slice t–1, bandwidth per pixel = 340 Hz, flip angle = 15°, FOV = 320 × 320 mm2, matrix = 512 × 512.For aortic flow acquisition, a “candy-cane” view of the aorta was first acquired to allow both slice planning and subsequent interslice distance measurement. Two phase-contrast sequences were planned, the first at the level of the right pulmonary artery to be perpendicular to the ascending aorta, the second just below the level of the diaphragm . The pha−1 gadoteric acid  was administered at 1.5 ml s−1 through a cannula in the antecubital fossa followed by a 20 ml saline flush administered at 1.5 ml s−1. After 2 min  the ventricular .Ventricular analysis was performed as per Society of Cardiovascular Magnetic Resonance guidelines.21Manual analysis (MA), semi-automated analysis (SA) and “fully” automated analysis (FA). For the purpose of MA, epicardial and endocardial contours were drawn around the right and left ventricles at end systole and end diastole by a radiologist with 5 years of cardiac MRI experience. The septum was treated as belonging to the left ventricle for LV mass quantification. Papillary muscles and trabeculations were included in the LV cavity volume and excluded from the LV mass quantification. SA was achieved by manually positioning the cursor over a central region within the ventricle in a single slice at end-diastole, thereafter, the signal intensity thresholding tool was used to grow an endocardial contour towards the edge until the entirety of the ventricular cavity was encompassed. Subsequently, the software generated an automatic epicardial contour. This process was repeated for each sequential slice. Manual adjustments were made where gross discrepancies were evident. For the FA, the mitral plane and ventricular apex were defined, after which the ventricular cavity was centred in the middle of the image and the automatic segmentation tool in circle used to automatically detect and draw the endocardial and epicardial contours. No subsequent adjustment was made to any contours generated using this technique. From the contours generated using these three techniques , the LV 22Aortic flow was calculated using a manual (MA) and semi-automatic (SA) technique. For MA, a contour was drawn around the aorta and propagated throughout the cardiac cycle. Each image was then manually adjusted to ensure accurate contouring of the aorta. For SA analysis, a signal intensity thresholding tool was used to grow a region of interest (ROI) to encompass the lumen of the vessel then automatically propagated throughout the remainder of the cardiac cycle. Only images where the contour included either the superior or inferior vena cava, pulmonary artery or other major vessel were adjusted. This process was repeated for the ascending, descending and abdominal aorta. The ascending aorta contour was used for aortic flow calculation . For PWVd” is the difference in distance between the sites of flow measurement and “Δt” is the difference between the arrival time of the flow waves at the two sites. This was determined using the intersection of the baseline flow with a line drawn through the data points in the 20–80% portion of the systolic upstroke.23 To calculate d, the sagittal-oblique candy-cane view of the aorta was used to measure (i) the distance between the ascending and descending aorta and (ii) distance from the descending to abdominal aorta. Arch PWV was calculated as: the time delay between flow waves arriving at the ascending and descending thoracic aorta (Δt), and the distance between these two points on the candy-cane view (d). To compute the aortic PWV, the time delay between the arrival of the flow wave within the ascending thoracic aorta and the arrival within the abdominal aorta was used in combination with the distance between the two .where “26 For the phase contrast SNR, two ROIs were drawn in the ascending and descending aorta on the first magnitude image of the stack. Two further ROIs were drawn outside the body. SNR was calculated as: [(ascending aorta + descending aorta)/2] / [(Air 1 SD +Air 2 SD)/2].For ventricular signal-to-noise ratio (SNR) and contrast-to-noise ratio (CNR) quantification, four ROIs were drawn within the heart  and two outside of the image area . All SNR27 Quantitative differences between measurements 1  and 2  were analysed using Bland–Altman plots.Repeated measurements were made by the same experienced observer in 20 participants following a time interval of greater than 3 months between the first and second reading. These 20 were selected randomly from those free from cardiac or respiratory motion artefact on all sequences so as to ensure that any differences in repeatability were due to the effects of contrast rather than variances in the image quality.t-test. Correlation of continuous variables was performed using a Pearson’s correlation. Bland–Altman plots were generated to assess repeatability of the analysis pre- and post-contrast administration. A p < 0.05 was considered statistically significant for differences between the measures. All statistical analysis was performed using SPSS .Continuous data were presented as mean ± SD, while nominal data was reported as N (%). Normality of distribution and equality of variances were tested. Comparison of pre- and post-gadolinium contrast measurements was performed using a paired-sample From the original 61 participants, 54 completed the imaging protocol, with 7 participants lost due to claustrophobia or discomfort precluding any scanning, or abandonment of the scan acquisition prior to the administration of contrast agent due to technical issues.vs post: 1028 ± 540, p = 0.003) with no significant change in the CNR  calculations.Administration of gadolinium contrast agent led to an increased SNR  and LVEF significantly lower  following the administration of gadolinium contrast agent  and a reduction in LVEF . In comparison, the FA, gadolinium contrast produced statistically significant differences with a higher LVEDV , and LVESV , with a lower LVM .The results from MA found LVESV was significantly higher  and RVESV  were noted to increase significantly in SA post contrast administration analysis, whereas RVEF was significantly reduced . Similarly, FA analysis with contrast agent showed a rise in RVEDV  and RVESV  and a decrease in RVEF .Administration of contrast agent produced no significant difference in manual RV quantification . Conversp < 0.001), which disappeared post-administration of gadolinium contrast dye . These findings were also seen on FA analysis . In comparison, RVSV and LVSV were in agreement (p = 0.18) and following contrast administration . SA analysis showed significant correlation between RVSV and LVSV both pre-contrast  and post administration of contrast agent . FA analysis showed a moderate correlation between RVSV and LVSV both pre  and post  contrast administration. No significant differences were observed on MA .In absence of contrast agent, SA found a significant difference between RVSV and LVSV .Gadolinium contrast resulted in a statistically significant increase in aortic SNR (pre: 36.6 ± 18.7 p = 0.06), with no difference observed in semi-automatic analysis (p = 0.14). There was no significant change in aortic PWV using either manual or semi-automatic techniques  with similar observation seen post-contrast administration .Gadolinium contrast administration produced a non-significant difference in MA of aortic flow , systolic blood pressure  or diastolic blood pressure .In post-gadolinium contrast administration, there were no significant changes in heart rate . Analysis of images post-contrast administration resulted in marginally wider limits of agreement for the intraobserver reproducibility of LVEF and RVEF, although there remained no significant difference between the readings  and the current study (gadoteric acid) with the former having a much shorter plasma half-life.29Conversely, Caspar et al purported no differences between pre- and post-contrast analysis.Elsewhere, Krombach et al found contrast agent did not significantly alter LV volumes, however this study used a 1.5 T scanner where contrast has less effect on tissue relaxation compared with 3 T.In addition, the current study is larger and thus better powered to detect small between group differences, which may explain the apparently conflicting findings. Furthermore, this work included three analysis techniques, all of which produce similar post contrast outcomes, further validating our current results.19 The finding of a lack of significant difference in before and after contrast administration studies is reassuring, and adds robustness to the comparison of studies regardless of contrast administration status. However, this robustness did not hold true for a semi-automated technique utilizing a signal intensity threshold algorithm, where significant differences were observed in RVEDV, RVESV and RVEF. The fact that differences in LV and RV SV reduced post-contrast administration and agreement with manual RV quantification improved, indicates that the increased SNR provided by the administration of the contrast agent improves the detection of the thin RV wall and the discrimination between cavity and trabeculae.We observed no significant differences in MA of the right ventricular volumes or function. LV quantification has received more attention than its neighbouring RV, with the effects of contrast agent on RV quantification not previously examined.31 However, this is not without its challenges and limitations with capacity for error both during semi-automated detection (software error) and during manual adjustments (observer error). Ideally, the combination of software detection and manual correction by an observer may offer a more reliable and less time-consuming approach.31 This notion is supported by Jaspers et al32, who not only reported high correlations between manual and semi-automatic ESV, EDV and EF, but also found that the semi-automatic technique provides careful distinction between muscular structures and ventricular blood pool.The analysis of cardiac MR images using a semi-automated contour detection protocol is attractive due to reduction in analysis time and the potential for improved inter- and intraobserver reproducibility.In the current work, we only allowed adjustments for major contouring errors such as extension of the contours into adjacent non-ventricular structures by the software, but made no adjustments for minor errors such as inclusion/exclusion of trabeculae within the cavity. This is of course not the practice in the clinical realm where all contours will be closely scrutinized, however, was necessary to determine the true effects of contrast agent on software ventricular analysis. Allowing greater manual adjustment may have minimized the differences and thus, minimized the effects of contrast agent, however such adjustments would simply dilute the effects of the contrast on the software, and start to replicate the results of a MA, if every minor contour error were to be adjusted.35 Despite these findings, previous studies have found no significant difference in absolute flow quantification, a finding in contradistinction to our own finding of a slight increase in aortic flow post-contrast administration.14 The differences in aortic flow quantification, however, were small amounting to only a 2 ml (2.5%) difference before and after contrast administration. Conversely, SA analysis resulted in no such difference. This may reflect the difficulties in contouring the aorta during very early systole and when flow is slower leading to greater inaccuracy. It was noted by the authors that the software appeared to largely undersize the aorta during slower flow periods, which may make for a more consistent contour delineation allowing for measurements robust to small changes in contour visualization and detection and background noise. In comparison, PWV was unchanged post-contrast administration, and was not affected by the analysis technique used.Multiple previous studies have demonstrated both an improved SNR, phase-to-noise ratio and velocity-to-noise ratio for phase-contrast imaging post-administration of contrast.vs fully automated quantification irrespective of the presence of contrast. Continuing advances in image processing and machine learning may advance this field in the future, but at present the current results suggest judicious review and contour correction is required following the automatic generation of endo and epicardial contours in the left and right ventricles.In our current study, we found a marked discrepancy between EF in manual –1. Secondly, even if this was the case, then the changes would still be real, just not by the expected mechanism. The dose of contrast agent administered was not weight-dependent in this study, which varies from routine clinical practice where a weight-based dosage is frequently used, however, we observed no significant relationship between CNR and weight, thus the confounding nature of this is likely limited. The current analysis was performed using a single post-processing software vendor, thus, further work is required to determine if these differences are observed using other software platforms. This is particularly pertinent with regards to the semi- and fully automated techniques, where proprietary software algorithms can differ substantially.There are limitations in the current study. There exists a time lag between images obtained without contrast and following contrast administration, thus the latter may be potentially prone to movement artefact due to developing participant discomfort. Additionally, the administration of 10 ml contrast agent and 20 ml saline could in theory change the measured volumes and flow due to change in volaemic status rather than the effects of the gadolinium on the generated RF signal. However, 30 ml is a small volume in healthy adults free from cardiovascular disease, and in fact, approximately matches the expected urinary output of 30–75 ml hAdministration of contrast agent significantly alters endocardial contour detection regardless of technique used resulting in changes in ventricular quantification. This effect is amplified when using SA techniques, thus care must be taken when comparing values obtained before and after contrast administration and when comparing post-contrast volumes with reference values. PWV analysis remained robust to these differences.


Myocardial fibrosis leads to a restrictive diastolic filling pattern of the left ventricle which is associated with a poor prognosis in patients with heart failure. We investigated the relationship between cardiac fibrosis and restrictive filling pattern of the left ventricle measured by Tc99m left ventriculography in patients with chronic symptomatic heart failure. Serum cardiac extracellular matrix markers including type I and III aminoterminal propeptide of procollagen (PINP and PIIINP), matrix metalloproteinase-2,9 , and tissue inhibitor of MMP-1 (TIMP-1) were analyzed. Fifty-one  patients were enrolled. Their median age was 51.8 years, and median left ventricular ejection fraction was 31.9%. Time to peak filling rate of the left ventricle was significantly correlated with serum levels of the three cardiac extracellular matrix markers . The patients with a restrictive diastolic filling pattern of the left ventricle (time to peak filling rate = 154 ms) had significantly higher levels of these extracellular matrix markers. In receiver operating characteristic curve analysis, areas under the curve of PIIINP, TIMP-1, and MMP-2 were 0.758, 0.695, and 0.751 to predict the presence of a restrictive pattern. In C-statistics, all three cardiac extracellular matrix markers significantly increased the area under the curve after adding creatinine. In net reclassification improvement and integrated discrimination improvement models, PIIINP and MMP-2 significantly improved the predictive power of age, creatinine and brain natriuretic peptide. In conclusion, serum extracellular matrix markers are significantly correlated with restrictive diastolic filling pattern of the left ventricle in patients with heart failure. Heart failure (HF) is a major health problem affecting more than 23 million patients worldwide . DespiteSystolic function has been reported to be a prognostic predictor in HF with reduced ejection fraction (HFrEF) . HoweverLeft ventricle remodeling plays a critical role in the progression of HF. Several serum markers of cardiac extracellular matrix (ECM) have been shown to play an important role in myocardial remodeling in various types of heart disease in patients with HFrEF and in animal models  9] 10] [10]9] [ [10]9] , 12. DetSeveral cardiac ECM markers including type III aminoterminal propeptide of procollagen (PIIINP), matrix metalloproteinases (MMPs), and tissue inhibitor of metalloproteinase-1 (TIMP-1) have recently been shown to be of prognostic value in patients with HF  14–17].–17.14–17In this study, we investigated the relationships between serum markers of cardiac ECM and LV restrictive filling using Tc99m left ventriculography in patients with HFrEF.We studied 51 patients with chronic HF secondary to LV systolic dysfunction (LVEF ≤ 45% measured using Tc99m left ventriculography) who regularly visited the HF clinics at National Taiwan University Hospital. The clinical history of all patients was recorded, and they all received a full examination by a cardiologist. Demographic data including sex, age, current medications, functional status, and cardiovascular risk factors were recorded. Venous blood samples were collected in serum separation tubes after overnight fasting. After clotting and centrifugation, the serum was collected and stored at −60°C until analysis. In echocardiography, LVEF was measured via an apical 4-chamber view (area-length method) according to the procedures of the American Society of Echocardiography . The stuWe assessed LV diastolic function using Tc99m left ventriculography. Peak-filling rate (PFR) was defined as the greatest filling rate in early diastole, and corresponded to the peak value of the first derivative of the diastolic portion of the time-activity curve. The filling was normalized to end-diastolic volume (EDV), and thus the unit for PFR was EDV/s. The time to peak filling rate (TPFR), expressed in milliseconds, was defined as the interval between end-systole and PFR.The normal PFR of the left ventricle has been reported to be 2.2 +/- 0.6 EDV/sec, and 198 +/- 22 ms for TPFR –17, 19. Serum levels of brain natriuretic peptide (BNP) were measured using an enzyme immunoassay kit . The intra- and inter-assay variations were < 5% and < 14%, respectively. Serum levels of type I aminoterminal propeptide of procollagen (PINP) were measured using a rapid equilibrium radioimmunoassay kit . The intra- and inter-assay variations were both < 7%, and the detection limit was 2 μg/l. Serum PIIINP was determined using a coated-tube radioimmunoassay method . The intra- and inter-assay variations of serum PIIINP were both < 5%, and the detection limit was 0.3 μg/l. TIMP-1 was measured using an ELISA kit . The intra- and inter-assay variations of serum TIMP-1 were both < 5%, and the detection limit was 0.08 ng/ml. Serum MMP-2 was measured using an ELISA kit . The intra- and inter-assay variations of this method were < 6% and < 8%, respectively, with a detection limit of 0.16 ng/ml. MMP-9 was also measured using an ELISA kit . The detection limit was 0.156 ng/mL, and the intra- and inter-assay variations were < 3% and < 8%, respectively.Continuous data were expressed as median value and interquartile range. Comparisons between groups for continuous data were performed using the Mann-Whitney U-test, and differences between proportions were assessed using the chi-square or Fisher's exact test. Spearman's non-parametric correlation test was used to analyze associations between ventricular diastolic function and serum fibrosis markers. Linear regression analysis was used to examine associations between serum fibrosis markers and LV restrictive diastolic function. The serum cardiac ECM markers were log-transformed due to non-normality as detected using the Kolmogorov-Smirnov test before being entered into the linear regression model. To compare the ability of serum cardiac ECM markers to predict the patients with a restrictive pattern in diastolic filling (TPFR ≤ 154 ms), we used areas under the receiver operator characteristic (ROC) curve determined by logistic regression analysis. The optimal cutoff points of serum fibrosis markers were obtained from the Youden index.http://www.r-project.org/) version 2.15.2. Statistical significance was set at p < 0.05.We also used C-statistics to describe the discrimination of the models before and after adding serum cardiac ECM markers –22. Net Fifty-one  patients were enrolled with a median age of 51.8 years, and median LVEF of 36.2% by echocardiography and 31.9% by Tc99m left ventriculography. Their median NYHA was 2, with 8, 27, 14, and 2 patients having a NYHA of I, II, III, and IV, respectively. The other demographic and biochemical data and medication history are shown in Table We divided the patients into two groups based on the presence of a restrictive pattern in Tc99m radionuclide ventriculography. The mean age was significantly older in the restrictive pattern group (p = 0.028), but there were no significant differences in the other demographic data Table .The mean level of serum fibrosis markers and Tc99m radionuclide ventriculography are shown in Table The patients with a restrictive LV pattern in Tc99m radionuclide ventriculography had significantly higher levels of serum fibrosis markers including TIMP-1 (p = 0.019), PIIINP p = 0.002), and MMP-2 (p = 0.002) , ROC analysis showed an area under the curve (AUC) of 0.632 for PINP, 0.758 for PIIINP, 0.695 for TIMP, 0.751 for MMP-2, and 0.467 for MMP-9  to predict the patients with a restrictive pattern in diastolic filling after adding two clinical parameters (age and sex) or BNP. In C-statistics, all three cardiac ECM markers significantly increased the AUC of creatinine Table . FurtherThe major findings of this study are: 1) serum ECM markers including TIMP-1, PIIINP, and MMP-2 were significantly correlated to LV TPFR; 2) the patients with a restrictive pattern of LV diastolic function had higher TIMP-1, PIIINP, and MMP-2 levels; 3) the results were still significant after adjusting for age and sex; 4) cardiac ECM markers significantly improved the power of the clinical parameters to predict the patients with a restrictive pattern in diastolic filling; and 5) the combination of PIIINP and creatinine had greatest power (AUC 0.944) to predict the patients with a restrictive pattern in diastolic filling. These findings not only reconfirmed the association between LV restrictive filling and cardiac ECM turnover, but also showed the potential of cardiac ECM markers as a useful tool to detect LV restrictive filling.ECM turnover regulates a fine balance between synthesis and degradation , 23, andIn the present study, PIIINP had the best AUC. Both collagen types I and III are present in normal and diseased myocardial tissue. PINP and PIIINP are by-products of collagen type I or III synthesis, and therefore they are considered to be markers of collagen biosynthesis . PIIINP MMPs are a family of enzymes that contribute to extracellular remodeling in several disease states. In addition, a family of inhibitors called tissue inhibitors of MMPs (TIMPs) have been shown to exist and to tightly regulate MMP activity. TIMP-1 has been shown to be highly associated with collagen accumulation and cardiac diastolic function , 29. BotIn the Val-HeFT trial, a decrease in LVEF was associated with an increase in all-cause mortality at 23 months . In addiDiastolic function can be estimated using echocardiography, cardiac MRI, and gate myocardial perfusion SPECT. Echocardiography is a simple, non-invasive and cost-effective tool, however alterations in loading conditions, operator technique, and the limitation of an acoustic window can all affect interpretation of the results. Although more expensive than echocardiography, cardiac MRI provides excellent spatial resolution and more precise parameters to assess diastolic function , howeverGated myocardial perfusion SPECT is widely used to assess perfusion abnormalities, LV systolic function and volume. First-pass and gated blood-pool scintigraphy including Tc99m left ventriculography are useful methods to assess LV diastolic dysfunction due to more stable parameters and independence from the influence of systolic function, age and sex . ProlongThere are several limitations to this study. First, the number of enrolled patients was relatively small, and may not be sufficient to provide definitive evidence. However, our findings may be sufficient to prompt further research. Second, we focused on HFrEF, and we are not sure whether similar results would be found in patients with HF with preserved ejection fraction. This could form the basis of further studies. Third, no histological evidence was available to prove myocardial remodeling and to assess the extent of fibrosis. Elevated levels of serum cardiac ECM markers may not present to the same degree over the myocardium. However, without clinical indications, it is not ethical to perform endomyocardial biopsies in these patients. Fourth, there is currently no general consensus on the definition of LV diastolic dysfunction and restrictive filling pattern in radionuclide ventriculography. Therefore, we used our own definition of “restrictive pattern” in this study. Further large-scale clinical studies and consensuses are needed to define the criteria of diastolic dysfunction and restrictive pattern in radionuclide ventriculography. Finally, a longer period of follow-up may be necessary to ascertain the clinical prognostic value of each biomarker and to differentiate outcomes between the patients with and without a restrictive LV filling pattern.In conclusion, PIINP, MMP-2, and TIMP-1 are significantly associated with a shortened TPFR in Tc99m left ventriculography and the presence of restrictive LV filling in patients with HFrEF. These serum markers have the potential to detect the presence of LV restriction in patients with HFrEF.


To investigate the potential of diffusion and perfusion indices  at baseline to 1.56 × 10−3 mm2/s  at 6-month follow-up (P = 0.0003). Conversely, the median perfusion fraction f was significantly decreased after UAE (P = 0.0001), with a median pre-UAE value of 14.2%  and a median post-UAE value of 9.2% . Significant correlations were found between fibroid volume reduction rate and percentage changes in ADC and perfusion fraction f at 6-month follow-up relative to baseline, with ρ values of −0.50 (P = 0.04) and 0.55 (P = 0.02), respectively. Seventeen fibroids were studied. The median ADCs showed a significant increase from 1.20 × 10f obtained from DWI at 3.0 T may help to evaluate treatment response to UAE. ADC and perfusion fraction  Uterine leiomyomas (fibroids) are the most common benign gynecological tumors, primarily affecting women of reproductive age . The estMRI has been demonstrated to be the most accurate imaging tool that is currently available to assess fibroid changes after UAE . Typicalb-values (a combination of diffusion gradient strength and pulse duration), quantitative data such as apparent diffusion coefficient (ADC) can be yielded. Several studies have shown that ADC may be a useful imaging marker to monitor the treatment response after UAE on 1.5-T MRI platforms , where D and D∗ are the diffusion coefficient and pseudodiffusion coefficient related to the tissue diffusivity and microvascular perfusion, respectively. To obtain a more robust estimate for the perfusion fraction f, a segmented fitting approach was applied as previously published ) to separate these two components and assess the perfusion changes of fibroids before and 6 months after UAE. The preliminary results showed a substantial drop in post-UAE measurement of perfusion fraction f. The perfusion changes after UAE revealed by IVIM can be explained by histologic changes. Because the perfusion fraction f is considered to represent blood volume [f value. The inherent vascular pattern of fibroids appears to represent a localized expansion of the myometrial vasculature [f in the ischemic lesion [As pointed out in the limitation of the previous studies –12, the n effect . Specifid volume , low vasculature . Embolizculature . Fibroidculature . In thisc lesion , 28, in f values are assumed to be independent of magnetic field strength. However, previous studies reported substantial variability in ADC and perfusion fraction f values at 1.5- and 3.0-T MRI systems [Nowadays, 3.0-T MRI systems are widely used in clinical practice. 3.0-T DWI is superior to 1.5 T in terms of higher SNR and greater spatial resolution. To our knowledge, this study represents the first human study of monitoring fibroid response to UAE using DWI at 3.0 T. Theoretically, the ADC and perfusion fraction  systems –32, whicf values 6 months after UAE, there still remained an overlap between pre- and post-UAE quantitative measurements. This is probably due to the individual difference of patients, and DWI could only depict the diffusion-related features of fibroids. Utility of multiparametric MRI would certainly be worthwhile to explore in future studies.Our study have limitations. First, the sample size was relatively small, which may limit the statistical power. Large-scale prospective studies are merited to confirm and extend the present observations. Second, no pathological findings were available as none of the patients received myomectomy or hysterectomy in case of symptom worsening or tumor recurrence at 6-month follow-up. The underlying mechanism in diffusion-weighted parameter alterations after UAE still remains incompletely understood. Therefore, radiological-pathological correlation was merely hypothesized. Third, as the main purpose of the preliminary study was to investigate the role of diffusion and perfusion indices obtained from DWI in monitoring fibroid response after UAE, other imaging features derived from conventional sequences including T2-weighted, T1-weighted, and contrast-enhanced MRI were not taken into account. Although all patients showed significantly elevated ADC values and decreased perfusion fraction f) from DWI at 3.0 T may help to evaluate treatment response to UAE. They hold the potential to provide insight into physiological changes in fibroids after embolization, though further validation is needed in clinical practice.In conclusion, diffusion and perfusion indices (ADC and perfusion fraction


Myocardial T1 mapping enables quantification of extracellular volume fraction (ECV), which correlates with myocardial fibrosis and is predictive of adverse cardiac events. Quantitative T1 mapping is highly useful in detecting scar tissue in patients with prior myocardial infarctions or those with other cardiomyopathies. The Modified Look-Locker inversion recovery (MOLLI) sequence is a clinical standard for T1 mapping, however it is limited by low spatial resolution which prevents it from being used for infarct characterization. In this study, a novel technique to convert high resolution 3D phase sensitive inversion recovery (3D-PSIR) images into high resolution T1 maps by calibration with low-resolution MOLLI T1 maps.+2, an exponential relationship was derived between signal intensity and T1 using a 3D-PSIR segmented Turbo FLASH and MOLLI acquisition, respectively. 9 canines with chronic infarcts (ranging from 12-14 weeks) were scanned on a 1.5 T Siemens Avanto scanner. A 3D-PSIR (covering the entire left ventricle) and a single long axis MOLLI were collected prior to contrast injection and 15-20 minutes after contrast injection (Gd-DTPA). The MOLLI and 3D-PSIR images had resolutions of approximately isotropic in-plane 1.3-1.4 mm × 8.0 mm and 1.4 × 1.4 × 1.4 mm, respectively. Six regions of interest  were drawn in the MOLLI image and the corresponding 3D-PSIR image to determine the exponential relationship between signal intensity and T1 based upon the Bloch equation. The high resolution reconstructed T1 maps of the left ventricle from the 3D PSIR images were then compared to the MOLLI T1 maps based on 10 ROI's drawn from the anteroseptum to the inferolateral wall of the left ventricle in the long axis slice. Mean T1 values were collected for each ROI and a linear regression was used to test the association of T1 values from the MOLLI image to the T1 values from the reconstructed T1 maps.From a phantom study using 11 agarose vials doped with different concentrations of Ni2 value of 0.938 for all nine dogs . The figure also illustrates a clearer delineation of the myocardial and scar boundaries in the calibrated 3D-PSIR based T1 maps when compared to the MOLLI T1 maps.The correlation between T1 values of MOLLI and reconstructed T1 maps showed an R3 for the T1 reconstructed maps versus 15.7 mm3 for the 2D MOLLI T1 maps. The T1 maps developed using this calibration technique can provide a better understanding of the 3D architecture of scar in patients with prior myocardial infarctions and can further delineate normal tissue versus scar tissue within infarct zones.High resolution 3D maps provide a resolution of 2.74 mm


Cardiac amyloidosis results in diffuse involvement of the myocardium. For non-invasive diagnosis of myocardial amyloidosis, delayed post contrast MRI is the current gold standard. However, myocardial involvement can be patchy; not infrequently the delayed enhancement images are technically limited and many patients cannot tolerate contrast due to poor renal function. Hence an alternative method for evaluation of myocardial amyloidosis is needed that would replace or supplement the current techniques. Myocardial strain analysis has been used in the past using echocardiography and grid tagged MRI. Echo is limited by spatial resolution and lack of 3D imagery and grid tagged strain analysis require prospective acquisition of grid tagged images. On the other hand, feature tracking strain analysis can utilize the routine multiplanar cine images for strain analysis, hence being quite easy to incorporate as part of routine cardiac protocol.Hypothesis: Strain evaluation using feature tracking can be successfully utilized to diagnose amyloidosis. We hypothesize that global myocardial strain values should be reduced in patients with amyloidosis when compared to patients with normal cardiac function.A retrospective IRB approved review was performed of 63 patients who underwent cardiac MRI at our facility for evaluation of myocardial amyloidosis. Baseline, demographic and imaging parameters were recorded from chart review. The diagnosis of amyloidosis was established on tissue biopsy. Conventional short axis, vertical long axis and 4-chamber cine SSPF images from the cardiac MRI scans were used to generate 2D and 3D myocardial strain maps using myocardial feature tracking software. Global radial, circumferential and longitudinal stain values were computed.Cardiac MR feature tracking strain analysis was able to differentiate patients with myocardial amyloidosis from those without myocardial amyloid with high sensitivity and specificity. There was significant reduction of global radial, circumferential and longitudinal stains in patients with myocardial amyloidosis using both 2D and 3D parameters . The area under the ROC curve for peak radial, circumferential and longitudinal strain were 89.5%, 91.3% and 88.1% using a cut off of 24.85, -15.12 and -11.59 respectively. The maximum sensitivity (89.3%) was achieved if any of the three parameters were abnormal, and the maximum specificity (85.7%) when all three parameters were abnormal.Global myocardial strain is significantly reduced in patients with cardiac amyloidosis. Cardiac MRI feature tracking global myocardial strain analysis is a robust tool for detection of cardiac amyloidosis.


High-risk hypertensives with a heart rate above 80 beats per minute (bpm) have an increased risk of cardiovascular events, particularly heart failure and sudden death, according to a new analysis of the Valsartan Anti-hypertensive Longterm Use Evaluation (VALUE) trial, presented at the 2010 American Society of Hypertension congress (ASH).The VALUE trial, first reported almost six years ago, showed that valsartan and amlodipine-based therapy were essentially equal in reducing the primary endpoints of cardiac morbidity or mortality in patients with hypertension and at least one high-risk factor.The researchers also identified that most of the risk occurred in those trial participants with heart rates of 79 bpm or more. There was a striking increase in the primary endpoint in the highest quintile of heart rate (≥ 79 bpm) compared with the pooled lower four quintiles.Annual incidence of new primary endpoint events in the highest quintile (compared with the lower four) was 30% higher in the first year of the study, 55% higher in the third year, 52% more in the fourth, and 46% higher in the fifth year of the study. A similar trend was seen throughout the trial for the heart failure and sudden death components of the endpoint.The negative effect of the in-trial tachycardia was not modified by the blood pressure control achieved in the study. The relative increase in the primary endpoint of the tachycardic group was 68% in the blood pressure-controlled and 63% in the blood pressure-uncontrolled groups (p < 0.0001).The results of the SHIfT trial will answer unequivocally whether reducing the heart rate in patients with systolic heart failure with the specific heart ratelowering agent ivabradine, will improve prognosis. The trial randomised to either ivabradine or placebo, heart failure patients in NYHA classes II to IV, with ejection fraction less than 35% and a heart rate greater than 70 bpm, who were already being treated with beta-blockers, ACE inhibitors and diuretics. The primary endpoint of the study is cardiovascular death and hospitalisation for heart failure.The SHIfT study is the largest ever study undertaken in heart failure, with 6 500 patients. The results of the SHIfT study will be announced at the hotline session on 29 August 2010 at the European Society of Cardiology’s annual congress in Stockholm.This trial is pivotal to the clinician’s perception of the value of heart rate lowering with ivabradine to reduce cardiovascular morbidity and mortality in these high-risk cardiovascular patients.


This study aimed to (1) validate plaque lipid quantification by T2 mapping against histology and (2) investigate if it could discriminate between symptomatic and asymptomatic patients.In-vivo carotid CMR is able to identify features of plaque vulnerability such as lipid core size. However, the current standard (multicontrast CMR) requires contrast media, extensive post-processing and subjective interpretation. Recently we proposed to use quantitative T2 mapping (DANTE-MESE) to acquire 5 slices in 4 min . T2 maps were generated using nonlinear fitting; lumen and external carotid boundary were contoured semi-automatically; lipid core was segmented from other plaque components by T2 thresholding; and the optimal segmentation (i.e. highest correlation with histology) was automatically calculated using leave-one-out cross-validation , either symptomatic or asymptomatic, were imaged at 3T (Siemens Verio) max 24 h before surgery . We used our novel black-blood Multiecho Spin-Echo sequence for T1w images as references to match T2 map locations. Plaque lipid area was manually segmented on sections stained with H&E and Masson Trichrome, with Oil-red-O used to confirm lipid distribution.Plaques were collected at the time of carotid endarterectomy, processed and cut at 1 mm intervals using carotid bifurcation and T2 than normal vessel wall and fibrous tissue, whereas haemorrhage infiltrating the lipid core increased T2. Thresholding T2 < 42 ms and T2 > 90 ms resulted in the globally optimal lipid core segmentation  had good quality and 60 slices with matching locations on plaque histology. Lipid without haemorrhage had shorter T2 mapping can be used to quantify plaque lipid content with good accuracy and reproducibility, and to classify plaques as symptomatic or asymptomatic based on their lipid core size. This technique can potentially be used to identify patients at risk of plaque rupture; informing decisions of stents vs. surgery; stratify for more intensive lipid treatment; and monitor response to treatment in clinical trials.We have demonstrated that carotid T


There is a wide variability as to where the musculocutaneous nerve (MCN) leaves the axillary sheath. Therefore multiple injection techniques, or the use of a proximally directed catheter, should be appropriate to block the MCN.Axillary plexus blocks (AXB) are widely used for upper limb operations. It is recommend that AXB should be performed using a multiple injection technique. Information about the course and position of the musculocutaneous nerve (MCN) is of relevance for AXB performance. The objective of this study was to examine the position of the MCN and its relationship to the axillary sheath using MRI. 54 patients underwent an AXB with 40 ml of local anaesthetic before MRI examination. The course of the MCN and the position where it left the axillary sheath and perforated the coracobrachial muscle (MCN exit point), in relation to the axillary artery and the block needle insertion point in the axillary fold, were recorded. The MCN was seen clearly in 23, partly in 26, and not identified in five patients at the MCN exit point. The mean distance from the insertion point of the block needle in the axillary fold to the MCN exit point was 36.8 mm . In 37 patients the MCN exit point was positioned inside the Q Axillary plexus blocks (AXB) are widely used as an anaesthetic method for upper limb operations. It is recommend that AXB should be performed using a multiple injection technique . At termEthical approval for this study  was provided by the Committee for Medical Research Ethics, Region South East (REK Sør-Øst), Pb 1130 Blindern, 0318 Oslo, Norway on June 8th, 2004 as for a previous study [The study was conducted at Oslo University Hospital, Rikshospitalet, in the period November 10th, 2014–February 1st, 2015. Forty five patients from a study on axillary brachial plexus blocks [All patients underwent an AXB with 40 ml of local anaesthetic (LA) injected before MRI scanning  was performed . MRI scaThe patients were examined in the horizontal supine position with the arms adducted. T1- and T2-weighted images were obtained according with a previously described protocol , and theThe course of the MCN and the surrounding anatomy was analysed in the short axis view . Data were described by mean, standard deviation (SD), range and counts.Of the 54 patients, 30 had a plexus block on the right and 24 on the left side. In 23 patients the MCN exit point was clearly and 26 patients partly visible. In 5 patients the MCN exit point could not be identified.The mean distance from the top of the humeral head to the insertion point of the block needle was 98.4 mm . The MCN exit point was positioned at a mean distance of 61.5 mm  from the humeral head. Thus, the mean distance from the insertion point of the block needle in the axillary fold to the MCN exit point was 36.8 mm  and in 11 patients inside the Q2 quadrant . In only one patient the MCN exit point was found in the Q4 quadrant at −8.1° to the zero angel line  for all patients  and the medial anterior (Q2) quadrant and spread over a distance of approximately 10 cm proximally to the axillary fold.The MRI of the axillary brachial plexus from 54 patients showed a wide variability as to where the musculocutaneous nerve (MCN) leaves the axillary sheath. The MCN exit points were mainly localized in the lateral anterior . When only a proximal catheter was used for LA injection, the success rate for MCN block decreased to 73 % .Non-ultrasound-guided and single injection axillary plexus block techniques have typically used high doses of LA –14. We tOur study has several limitations. MRI measurements are substantially observer dependant. Therefore the images were discussed among the three authors, and a consensus agreement was achieved. In five of the 54 patients (9 %) the MCN exit point could not be identified. One reason for our missing data may be that the MCN exit point was outside the scanning field. However, the MCN can sometimes be missing, or it demonstrates unusual anatomy with an alternative course, passing over the surface of the coracobrachial muscle instead of perforating it –20. In tThe MRI examination was performed just after the LA had been injected. The distortion of the anatomy , due to Because of the closed scanner construction, our MRI examinations were performed with the arm adducted. Distances and nerve positions might differ when the arm is abducted, as in the typical position when performing the AXB.There is a wide variability as to where the musculocutaneous nerve (MCN) leaves the axillary sheath. Therefore multiple injection techniques, or the use of a proximally directed catheter, should be appropriate to block the MCN.


The coexistence of congenital left ventricular aneurysm and abnormal cardiac trabeculation with gene mutation has not been reported previously. Here, we report a case of coexisting congenital left ventricular aneurysm and prominent left ventricular trabeculation in a patient with LIM domain binding 3 gene mutation.LDB3 gene .A 30-year-old Asian man showed paroxysmal sinus tachycardia and Q waves in an electrocardiogram health check. There were no specific findings in physical examinations and serological tests. A coronary-computed tomography angiography check showed normal coronary artery and no coronary stenosis. Both left ventricle contrast echocardiography and cardiac magnetic resonance showed rare patterns of a combination of an apical aneurysm-like out-pouching structure with a wide connection to the left ventricle and prominent left ventricular trabecular meshwork. High-throughput sequencing examinations showed a novel mutation in the LDB3 gene mutation. Congenital left ventricular aneurysm and prominent left ventricular trabeculation may share the same genetic background.Our finding indicates that the phenotypic expression of two heart conditions, congenital left ventricular aneurysm and prominent left ventricular trabeculation, although rare, can occur simultaneously with  LDB3) gene mutation , which was not reported in the public databases of Human Gene Mutation Database  or Single Nucleotide Polymorphism database . The genetic discovery from this case may open the door to a better understanding of abnormal cardiac development and affect clinical care of patients with congenital LVA.Congenital left ventricular aneurysm (LVA) is a rare cardiac malformation first described in 1816, and characterized as an akinetic or dyskinetic structure with a wide connection to the left ventricle . The patA 30-year-old Asian man was admitted to our hospital because of the finding of unusual Q waves of electrocardiogram (ECG) in his first health examination and an abnormal pattern of his left ventricle in a following transthoracic echocardiography check. He has no risk factors of cardiovascular diseases, and no history of coronary artery disease or myocarditis. He presented for years with unspecific symptoms like palpitation and vague, intermittent chest pain, which were unrelated to physical exertion, and he did not receive any medical intervention for these symptoms in the past.On general physical examination he had a body temperature of 36.7 °C and a heart rate of 84 beats per minute in a normal condition and 121 beats per minute in a cardiopalmus condition. His respiratory rate was 16 breaths per minute. He had a blood pressure of 110/72 mm Hg and an oxygen saturation of 98% on room air. His cardiac examination was normal; there were no murmurs or extracardiac sounds on auscultation. His complete physical examination including a neurological examination was unremarkable. Laboratory tests revealed: normal markers of myocardial injury, for example MB isoenzyme of creatine kinase (CK-MB), high-sensitive troponin I (hsTnI), lactate dehydrogenase (LDH), and aspartate aminotransferase (AST); a positive enterovirus (EVs) -ribonucleic acid (RNA); and negative coxsackievirus B (CoxB)3 -immunoglobulin M (IgM), CoxB5-IgM, and cytomegalovirus ©-IgM in the virologic examination. The antinuclear antibody (ANA) spectrum showed a positive anti-double-stranded deoxyribonucleic acid (dsDNA) antibody, and the titer of anti-ANA was within a normal range. Other ANAs were negative. The inflammatory indicators of C-reactive protein (CRP), antistreptolysin O (ASO), and erythrocyte sedimentation rate (ESR) were within the normal ranges. Routine laboratory tests for liver, renal, electrolytes, and blood glucose were normal. His low-density lipoprotein (LDL) cholesterol was mildly elevated (3.4 mmol/L) in the serum lipid profile and the other lipids were within normal range Table . A subseLDB3 gene  was identified. The variant was considered to be probably pathogenic because of the following criteria: (1) it was not reported in the public databases of HGMD (http://www.hgmd.cf.ac.uk/ac/index.php) or dbSNP (http://www.ncbi.nlm.nih.gov/projects/SNP/); and (2) it predicted pathogenic mutation by multiple in silico algorithms . No other mutations were identified in the gene test. The final diagnosis of our patient was coexistence of congenital LVA and prominent LV trabeculation with LDB3 mutation . There was no occurrence of complications or new cardiac symptoms during a 3-month follow-up evaluation and he had a normal cardiac function after he was discharged. An annual follow-up was scheduled for further assessment.For further evaluating the genetic background of this rare combination, his DNA was isolated from a peripheral blood sample and screened for mutations by high-throughput sequencing, which aimed at cardiomyopathy-related genes, after informed consent was obtained. These mutations have been reported in five major cardiomyopathies, including hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), restrictive cardiomyopathy (RCM), and LV non-compaction (LVNC) . In thisTo the best of our knowledge, this is the first report of a case of a combination of congenital LVA and prominent LV trabeculation with a gene mutation. Congenital LVA is a rare cardiac anomaly, which is described as an akinetic or dyskinetic structure with a wide connection to the left ventricle , and canet al. [LDB3 gene was identified. Autoimmune connective tissue disorder, such as systemic lupus erythematosus (SLE), has been reported to induce LV aneurysm [The current diagnosis of congenital LVA is based on exclusion of other diseases which may induce acquired LV aneurysms, that is, coronary artery disease , autoimmet al.  reportedet al. . This paet al. . We thenet al. , 28, andet al. , 30. Moret al. . For aidet al. . This paet al. , 34. Caret al. , and apiet al. . The diaet al. , 36, andet al. , was alsaneurysm . Testinganeurysm . We evalaneurysm , 38. Theaneurysm . Howeveraneurysm . Hence, aneurysm . TraumatLDB3 gene, which was submitted to HGMD and dbSNP databases after the detection.We found a rare case combining both congenital LVA and prominent LV trabeculation in an adult. There have been concerns that excessive trabeculation may be a marker of underlying heart muscle disease . For insLDB3 gene, also known as Z-band alternatively spliced PDZ motif (ZASP), encodes a PDZ-LIM domain-binding factor that plays an important role in maintaining the structural integrity of the striated muscle Z-disc in multiple species [et al. [et al. [LDB3 alone was sufficient for interaction with ACTA1. The long ZASP isoform lacking exon 10 also interacted with ACTA1, indicating an additional actin-binding region encoded by the exon 8–11 junction that is not present in the other isoforms. These findings together suggested that LDB3 gene is important for skeletal muscle structural integrity. Mutations in the LDB3 gene have been identified in some cardiomyopathies, such as DCM, HCM, and LVNC [LDB3 gene also presented with congenital LVA. Conceivably, the LDB3 gene mutation may be associated with the development of myocardial lesion in this patient. Despite the specific genetic finding, it does not in itself change the current diagnostic and therapeutic strategy for congenital LVA; however, the identification of genetic abnormality, when integrated with the clinical characteristics, may influence the overall case assessment, and may appropriately impact the clinical recommendations in the setting of congenital LVA.The  species . PDZ dom species . Faulkne [et al.  determin [et al.  showed t [et al.  found thand LVNC –46. In tLDB3 gene mutation, suggesting the same genetic background may be shared within congenital LVA and cardiomyopathies. However, a single case of such a rare combination with a single gene mutation does not strongly support the link. More evidence is still needed to elucidate the association of genetic variations and congenital LVA. Comprehensive diagnostic assessment may provide a better understanding of the genotype–phenotype correlation between these two heart conditions. Our finding may help cardiologists and medical scientists to gain new insights into the basic mechanisms leading to congenital LVA and abnormal cardiac trabeculation.This case presents the phenotypic expression of two heart conditions, congenital LVA and prominent LV trabeculation, coexisting with


Risk prediction for sudden death remains imperfect in HCM. We compared CMR-derived LV fibrosis and strain measures to outcomes in a cohort of HCM patients.Consecutive patients undergoing CMR on identical 1.5 Tesla scanners to evaluate HCM were identified at a tertiary referral center. LV fibrosis was measured using a previously validated iterative technique  from LGE images acquired using standard inversion recovery techniques. LV volume, mass and EF were assessed from contiguous short axis SSFP cines. LV circumferential and radial strain were measured from a single mid-SAX SSFP cine . Sustained VT/VF, ICD placement, appropriate ICD therapy, CHF admission, septal reduction and/or death comprised the Event Group, n = 13 and those without the No Event Group, n = 34. Baseline characteristics were reported as mean ± SD for continuous variables and frequencies for categorical variables with group differences examined by t and χ2 tests. A Shapiro-Wilk test was used to evaluate normality and a Wilcoxon-Kruskal-Wallis test was used for non normal distributions. Linear regression analysis evaluated the independent association of predictive variables and receiver operator curves were generated .At a median of 12 months post-CMR, 13 of 47 identified patients experienced an event (Figure LV strain, fibrosis burden and wall thickness contribute to risk stratification in HCM beyond clinical predictors. These findings support further studies to develop multivariable prediction models that incorporate multiple CMR measures.


The aim of this study was to evaluate the utility of adding quantitative assessments of cardiac function from echocardiography to clinical factors in predicting the outcome of patients with acute pulmonary embolism (PE).Patients with a diagnosis of acute PE, based on a positive ventilation perfusion scan or computed tomography (CT) chest angiogram, were identified using the Duke University Hospital Database. Of these, 69 had echocardiograms within 24–48 h of the diagnosis that were suitable for offline analysis. Clinical features that were analyzed included age, gender, body mass index, vital signs and comorbidities. Echocardiographic parameters that were analyzed included left ventricular (LV) ejection fraction (EF), regional, free wall and global RV speckle-tracking strain, RV fraction area change (RVFAC), Tricuspid Annular Plane Systolic Excursion (TAPSE), pulmonary artery acceleration time (PAAT) and RV myocardial performance (Tei) index. Univariable and multivariable regression statistical analysis models were used.2. Twenty-nine percent of the cohort had a history of cancer, with a significant increase in cancer prevalence in non-survivors . Clinical parameters including heart rate, respiratory rate, troponin T level, active malignancy, hypertension and COPD were higher among non-survivors when compared to survivors (p ≤ 0.05). Using univariable analysis, NYHA class III symptoms, hypoxemia on presentation, tachycardia, tachypnea, elevation in Troponin T, absence of hypertension, active malignancy and chronic obstructive pulmonary disease (COPD) were increased in non-survivors compared to survivors (p ≤ 0.05). In multivariable models, RV Tei Index, global and free  wall RVLS were found to be negatively associated with survival probability after adjusting for age, gender and systolic blood pressure (p ≤ 0.05).Out of 69 patients with acute PE, the median age was 55 and 48 % were female. The median body mass index (BMI) was 27 kg/mThe addition of echocardiographic assessment of RV function to clinical parameters improved the prediction of outcomes for patients with acute PE. Larger studies are needed to validate these findings. Acute pulmonary Embolism (PE) is a major cause of morbidity and mortality in the United States and Europe, accounting for 100,000 and 300,000 deaths annually, respectively . PE can To address this, risk assessments based on clinical and imaging parameters have been developed. The Pulmonary Embolism Severity Index (PESI) is an excellent clinical predictor of outcomes in patients with PE . It is bWe retrospectively identified patients who had a diagnosis of acute PE between January 2010 and April 2014, confirmed by contrasted computed tomography (CT) scan of the chest and/or ventilation-perfusion (VQ) nuclear medicine imaging at Duke University Medical Center  using the Duke Enterprise Data Unified Content Explorer (DEDUCE) . SubjectAll echocardiographic studies were performed on GE Vivid E9 using a 3.5 MHz probe  or Philips IE33 . Off-line analyses of images were performed in Xcelera  and Image-Arena  by a single experienced reader and analysis was confirmed by a separate experienced reader; inter-reader variability for these studies have been shown to be low . TAPSE wfree) was calculated as the average of the three free wall segments and the longitudinal strain of the RV septum (RVsept) was calculated as the average of the three septal segments. Global RVLS was calculated as the average of strains from all segments. All strain and other 2D echo-derived parameter analyses were performed blinded to clinical data.2D strain analysis was performed from the apical 4-chamber view as previously described  Fig. 1b1b. The rDemographic and clinical characteristics of participants were presented in the study by survival status and the two groups were compared using Fisher’s exact test for categorical variables and a Kruskal-Wallis rank sum test for continuous variables. Continuous variables were summarized by the median and interquartile range and categorical variables were summarized by counts and percentages Tables  and 2. TDuring the study period, 135 patients were admitted with a clinical diagnosis of acute PE. Among these, 95 patients diagnosed with an acute PE had a transthoracic echocardiogram within the initial 24–48 h of admission. 26 patients did not have suitable images for offline analysis, resulting in a cohort of 69 analyzed subjects. Six of the subjects underwent thrombolysis. At 30 days, of these 69 subjects, 14 had died and 55 survived. (Table n = 38) of patients were females. The median body mass index (BMI) was 27 kg/m2 (range 20–68). With respect to comorbidities, 29 % (n = 20) of the cohort had a history of cancer, with a significantly higher prevalence in non-survivors compared to survivors . The only other significant difference in comorbidities between non-survivors and survivors was in the prevalence of hypertension . 13 % of patients (n = 9) had a history of prior venous thromboembolism. On presentation, 90 % (n = 62) had NYHA class III symptoms (Table p ≤ 0.05) between non-survivors and survivors . For example, a significant proportion of non-survivors had global and free wall RVLS of more than −12.5 .A number of echocardiographic parameters were assessed in our cohort Table . These ip ≤ 0.05) associated with 30 day mortality. These included active malignancy, serum troponin, global and free wall RVLS, RV Tei Index, and patients with PESI classes four and five. For multivariable regression analysis, we attempted to include PESI as a clinical predictor with selected echo parameters, but could not because PESI score  demonstrated non-trivial collinearity with global and free wall RVLS and RV Tei index, resulting in unstable standard errors of estimates. Similarly, both heart rate and systolic blood pressure displayed significant collinearity, so only systolic blood pressure was included as it was a predictor in the univariable model. As the multivariable models could not include those clinical and echocardiographic predictors together, we instead used a multivariable model that included parameters used to calculate PESI [p ≤ 0.05).On univariable analysis, a number of clinical and echocardiographic parameters were statistically significantly  of images for offline analysis and poor tracking observed in the basal and mid-free wall segments. In our analysis, we found significant collinearity between PESI and RVLS and Tei index, preventing us from combining our echo predictors with PESI score. Therefore, it is possible that echo predictors do not add significant information to the PESI score. Previous studies from our group have demonstrated small inter- and intra-observer variability in assessment of RV strain , so such


Adult congenital heart disease (ACHD) has become an important subspecialty in cardiology. Over the past 40 years, life expectancy of patients with congenital heart disease (CHD) has greatly increased, in particular by developments in the field of cardiac surgery together with improved perioperative management and care in outpatient clinics. Up until 6 decades ago, only 15 % of patients born with CHD survived into adulthood without surgical correction. Nowadays, over 90 % of these patients are expected to survive into adulthood [In Westernised countries, the need for specialised care for ACHD patients has long been recognised and the amount of ACHD centres in these countries outnumber the worldwide proposal of one ACHD centre per 10 million population . In the In clinical care interdisciplinary collaboration between ACHD cardiologists, paediatric cardiologists, electrophysiologists, cardiac surgeons, specialised nurses, haematologists, psychologists and gynaecologists is of paramount importance. To aid clinical decision-making, both the American College of Cardiology/American Heart Association and the European Society of Cardiology (ESC) published guidelines on the management of grown-up congenital heart disease in 2008 and 2010 respectively , 5. Not In a worldwide survey on the research output of ACHD centres, Kempny et al. demonstrated that the summed impact factor for ACHD publications has increased dramatically between 1995 and 2011 . At presWith the increasing implementation of electronic patient files and electronic healthcare data, more clinical information will become available for research purposes. How to deal with these Big Data is still under investigation, but this can be useful in predictive analytics and as an adjunct to the current registry data.In this issue of the Netherlands Heart Journal, the variety of Dutch research in ACHD is clearly demonstrated. Continuous efforts for collaboration on a national and international level are needed to increase patient numbers and outcome measures and will provide important data to improve international guidelines and the care for ACHD patients.


FXN gene. Recent report demonstrates that specific-gene therapy may prevent and reverse the cardiomyopathy in a mice model of FRDA. Myocardial interstitial fibrosis is a hallmark of FRDA's cardiomyopathy and a potential substrate for arrhythmias and HF. Myocardial tissue characterization by cardiac magnetic resonance (CMR) allows access to tissue-based phenotypes that may better describe LV remodeling in FRDA's cardiomyopathy.Heart Failure (HF) is the most common cause of death in Friedreich's ataxia (FRDA), a mitochondrial disease characterized by neurodegeneration, hypertrophic cardiomyopathy, caused by homozygous GAA expansions in the ic), a measure of cardiomyocyte hypertrophy, using T1-weighted CMR imaging in cohort of patients with FRDA without HF.The aim of this study was to perform direct quantification of myocardial extracellular volume fraction (ECV) and intracellular lifetime of water  and in 30 healthy volunteers as control subjects (mean age 49 ± 15) using a 3T CMR system. The T1 quantification by Look-Locker gradient-echo before and after contrast applying a 2-site model for transcytolemmal water Exchange was used for ECV and τ2, p < 0.05). In 4 out 27 FRDA patients a non-ischemic LGE pattern was present. Both ECV and intracellular lifetime of water (τic) were significantly higher FRDA patients .FRDA patients revealed normal LVEF with increased LV Mass-index compared with health controls  determined by T1 measurements characterized early signs of myocardial tissue remodeling in FRDA with normal LVEF. Early changes in tissue-phenotypes are detectable by novel-CMR methods in FRDA patients, and may be useful to track effects of new genetic therapies for FRDA cardiomyopathy.ECV and intracellular lifetime of water (τ