In comparison to the well established changes in compliance that occur at the large vessel level in diabetes, much less is known about the changes in compliance of the cardiovascular system at the end-organ level. The aim of this study was therefore to examine whether there was a correlation between resistance of the intrarenal arteries of the kidney and compliance of the left ventricle, as estimated by measurements of diastolic function, in subjects with type 2 diabetes.We studied 167 unselected clinic patients with type 2 diabetes with a kidney duplex scan to estimate intrarenal vascular resistance, i.e. the resistance index (RI = peak systolic velocity-minimum diastolic velocity/peak systolic velocity) and a transthoracic echocardiogram (TTE) employing tissue doppler studies to document diastolic and systolic ventricular function.Renal RI was significantly higher in subjects with diastolic dysfunction (0.72 ± 0.05) when compared with those who had a normal TTE examination . Renal RI values were correlated with markers of diastolic dysfunction including the E/Vp ratio , left atrial area , the E/A ratio  and the E/E' ratio . These associations were independent of systolic function, hypertension, the presence and severity of chronic kidney disease, the use of renin-angiotensin inhibitors and other potentially confounding variables.Increasing vascular resistance of the intrarenal arteries was associated with markers of diastolic dysfunction in subjects with type 2 diabetes. These findings are consistent with the hypothesis that vascular and cardiac stiffening in diabetes are manifestations of common pathophysiological mechanisms. Diabetes is associated with the premature development of large vessel arterial stiffness . IncreasEarly in the course of diabetes, progressive cardiac fibrosis and hypertrophy lead impaired relaxation and a stiff left ventricle. We have previously found 'diastolic dysfunction' in over half of all unselected patients with type 2 diabetes attending a diabetes clinic at a tertiary care center ,4. FurthThese findings led us to hypothesize that reduced compliance of the intrarenal arteries that is associated with diabetic kidney disease, is mirrored by changes in the diabetic heart, which are manifested by worsening markers of diastolic function. The aim of this study was therefore to search for a link between compliance of the intrarenal arteries and compliance of the left ventricular wall in individuals with type 2 diabetes.We studied 167 unselected patients with type 2 diabetes attending the diabetes clinics at the Endocrine Centre, Austin Health, Australia. The Austin Health Human Research Ethics Committee approved the research described in this study and informed consent was obtained from all participants.Intra-renal vascular resistance was estimated by renal duplex ultrasound as previously described . The renTransthoracic echocardiogram (TTE) was performed using a commercially available ultrasound system  in the left lateral decubitus position and methods that have been described previously . Recordi99mTc-diethylene-triamine-penta-acetic acid (DTPA) employing the Brochner-Mortensen correction. Glycated haemoglobin (HbA1c), fasting lipids, serum electrolytes and urinary albumin excretion (AER) were measured as previously described [An isotopic glomerular filtration rate (iGFR) was measured by the plasma disappearance of isotopic escribed ,13. A fuContinuous data are expressed as mean ± standard deviation (SD). Categorical data are expressed as frequency (%). Where appropriate, means and proportions were compared by using analysis of variance and chi-square tests, respectively. The Pearson method was used to test for a correlation between the intrarenal RI and the echocardiographic parameters of diastolic function. A multivariate linear regression model was used to confirm the contribution of independent association between RI and cardiac parameters. All variables known to be associated with diastolic function were included in the final model, along with any variables associated with diastolic function in univariate analyses with a p value < 0.01. The potential for multiple colinearity was tested using the variance inflation factor (VIF) and condition number (CN), where VIF <10 and CN <30 are desirable.2.The clinical and biochemical characteristics of the study population are shown in Table Subjects were further stratified according to results of their TTE examination. Thirty five percent of participants had normal cardiac function on TTE examination (n = 59). However, the majority of diabetic subjects (65%) had abnormal cardiac function n = 108), including 66 individuals with diastolic dysfunction alone (40%), 30 with concurrent diastolic and systolic dysfunction (18%) and in a small number of individuals (n = 12) isolated systolic dysfunction was the only finding (7%). Subjects with diastolic dysfunction were generally older, had a greater frequency of hypertension and renal impairment than those with a normal TTE examination. As expected, subjects with concurrent systolic dysfunction had a greater prevalence of past CVD . As shown in Figure As shown in figure This study shows that resistance of the intrarenal arteries is closely linked with abnormal cardiac function in patients with type 2 diabetes. In particular, there was a continuous relationship between increasing resistance of the intrarenal arteries and established indices of left ventricular filling pressure and diastolic function. These associations were independent of systolic function, hypertension, the presence and severity of chronic kidney disease, the use of renin-angiotensin inhibitors and other potentially confounding variables.These data support the hypothesis of a common pathway to diabetic complications, which promotes stiffening and progressive dysfunction in both the heart and major vessels. Such common contributory factors may include oxidative stress, insulin resistance, inflammation, the accumulation of advanced glycation end products (AGEs), activation of the rennin angiotensin system (RAS), arterial calcification and endothelial (dys)function. However, it is likely that a parallel increase in the resistance of the intrarenal arteries and indices of diastolic dysfunction also partly reflects a generalized reduction in vascular compliance associated with arteriosclerosis in a range of vascular beds. In support of this suggestion, both the intra-renal RI and parameters of diastolic dysfunction have been shown to be associated with systemic pulse wave velocity, arterial compliance, and systemic vascular damage in both diabetic and non-diabetic subjects ,15.One of the weaknesses of this study was that we did not measure total arterial compliance and that we have used the intrarenal RI and indices of diastolic dysfunction as surrogate markers of intrarenal arterial and ventricular compliance, respectively. It is appreciated that functional as well as structural parameters may have influenced our results. Also, we did not account for the possibility of the presence of an elevated central venous pressure or autonomic neuropathy, factors that may have contributed to the development of both intrarenal arterial compliance and diastolic dysfunction.This study demonstrates that worsening indices of diastolic function in subjects with type 2 diabetes parallels increases in resistance of the intrarenal vessels. These findings are consistent with the hypothesis that both the development of vascular and myocardial stiffening in diabetes are manifestations of common pathophysiological mechanisms. It is possible that these processes also contribute to the link between renal dysfunction and cardiovascular outcomes in subjects with type 2 diabetes ,8.99mTc-DTPA employing the Brochner-Mortensen correction); E: early mitral flow velocity; E': early diastolic velocity of the mitral annulus; A: late mitral flow velocity; Vp: flow propagation velocity; BMI: body mass index; BP: blood pressure; RAS: renin angiotensin system; CHD: coronary heart disease; AER: albumin excretion rate; CRP: C-reactive protein; HbA1c: glycated haemoglobin.TTE: transthoracic echocardiogram; RI: resistance index; iGFR: isotopic glomerular filtration rate (measured by the plasma disappearance of The authors declare that they have no competing interests.RJM drafted the paper. RJM, SP, TJS, PGM, MCT, GJ, LMB & PMS were involved in the study design, coordination and data acquisition. MCT performed the statistical analysis of the data presented. LMB & PMS coordinated and interpreted the TTE studies whilst PGM & HH coordinated and interpreted the renal arterial RI studies. All authors approved the final version of the manuscript.


Using high resolution cardiovascular magnetic resonance (CMR), we aimed to detect new details of left ventricular (LV) systolic and diastolic function, to explain the twisting and longitudinal movements of the left ventricle.Using CMR phase contrast velocity mapping  regional wall motion patterns and longitudinally and circumferentially directed movements of the left ventricle were studied using a high temporal resolution technique in healthy male subjects .Previously undescribed systolic and diastolic motion patterns were obtained for left ventricular segments  and for basal, mid and apical segments. The summation of segmental motion results in a complex pattern of ventricular twisting and longitudinal motion in the normal human heart which underlies systolic and diastolic function. As viewed from the apex, the entire LV initially rotates in a counter-clockwise direction at the beginning of ventricular systole, followed by opposing clockwise rotation of the base and counter-clockwise rotation at the apex, resulting in ventricular torsion. Simultaneously, as the entire LV moves in an apical direction during systole, the base and apex move towards each other, with little net apical displacement. The reverse of these motion patterns occur in diastole.Left ventricular function may be a consequence of the relative orientations and moments of torque of the sub-epicardial relative to the sub-endocardial myocyte layers, with influence from tethering of the heart to adjacent structures and the directional forces associated with blood flow. Understanding the complex mechanics of the left ventricle is vital to enable these techniques to be used for the evaluation of cardiac pathology. Left ventricular (LV) function is geometrically and mechanically complex. Advances in cardiac imaging techniques have accompanied ongoing efforts to define the mechanisms of three dimensional ventricular motion -7. CurreCardiovascular magnetic resonance (CMR) has allowed detailed evaluation of LV wall motion throughout the cardiac cycle, using myocardial velocity encoding techniques ,14. HereCMR scans were performed on fourteen healthy male volunteers (age 23 ± 3 years). All subjects were non-smokers, with no history of cardiovascular disease. The study was conducted according to the principles of the Declaration of Helsinki and was approved by a local Oxfordshire Clinical Research Ethics Committee. Each subject provided written informed consent.black blood segmented k-space spoiled gradient echo sequence  [CMR scans were performed using a 1.5 Tesla Siemens Sonata clinical scanner. Pilot images, followed by horizontal and vertical long axis cine images were acquired using an SSFP pulse sequence. Cine images for navigator gated high temporal resolution phase contrast velocity mapping were acquired using a 56 × 96) .To obtain velocity measurements, three equidistant short-axis slices along the left ventricle were evaluated using 3-directionally encoded, time resolved phase contrast velocity maps. Velocity encoding was performed by including a phase image with no velocity encoding followed by images with a bipolar gradient in read, phase or slice direction after each RF pulse to the otherwise identical sequence . Acquisition was prospectively gated, typically covering between 80 to 90% of the cardiac cycle, meaning the contribution of atrial contraction on left ventricular motion was not possible to assess. Post-processing was performed in the standard fashion of subtracting the phase from the image with no velocity encoding, followed by conversion of the phase data into velocity maps.The basal slice was positioned parallel to the base of the heart, distal to the LV outflow tract. Basal, midventricular and apical slices were positioned 15 to 20 mm apart, depending on the heart size. With cardiac and respiratory gating, each short axis acquisition took approximately 3-5 minutes, with an average of 60-70 phases per cardiac cycle. Cardiac phases were determined, with the end systole defined as the smallest LV cavity.Analysis was performed using customized software , where the left ventricle was divided into 16 segments  according to the American Heart Association model . Ventrica). By the middle of the rapid ejection phase, the basal and mid-ventricular segments . The apical segments, however, continued their counter-clockwise rotation until ventricular repolarization occurred . In most basal segments, a brief increase in clockwise motion occurred at the beginning of diastole . By the end of isovolumetric relaxation, the entire ventricle was rotating in a counter-clockwise direction , with peak rotational velocities reached from the LV base towards the apex. This wave of recoil motion was particularly prominent in anterior  and anterolateral  segments. Subsequently, the amplitude of the rotational movement decreased with little circumferential movement during diastasis. Average motion for basal, mid and apical slices are provided in Figure At the beginning of systole, all LV segments rotated in a counter-clockwise direction, as viewed from the apex, reaching peak velocities at the beginning of rapid ejection Figure , arrow aa). Subsequently, the torsion rate progressively increased, peaking at the end of rapid ejection  and subsequently in negative waves on the ventricular torsion rate graph .The variation in global ventricular torsion rate, which reflects the speed of ventricular twisting motions or the gradient between apical and basal motion, is displayed in Figure n Figure , arrow bn Figure , arrow ca). By the end of the rapid ejection phase, the velocity of this downward movement decreased to a plateau in basal and mid-ventricular segments , whilst at the apex it reached negative values, indicating the apex was moving towards the base of the heart . Overall, there was little apical displacement during systole. Repolarization was followed by a steep deceleration in downward displacement  with a change in movement direction before the end of systole. Subsequently, a fast recoil motion in an opposite direction occurred in all segments. This upward movement along the longitudinal axis continued during isovolumetric relaxation, reaching peak velocities by the end of this phase . There was a brief deceleration in the upward recoil displacement at the beginning of diastole . During the phase of rapid ventricular filling, the longitudinal velocity abruptly fell in all segments  with little subsequent motion along the longitudinal axis.At the beginning of systole, all segments moved downwards (towards the LV apex) along the LV longitudinal axis, reaching peak velocities during the first half of rapid ejection Figure , arrow as Figure , arrow ct Figure , arrow bt Figure , arrow de Figure , arrow fe Figure , arrow es Figure , g with a, segments 2, 3, 8, 9, 14) compared to other segments from the same ventricular slice. In all segments diastolic velocities were considerably higher than corresponding systolic values, indicating rapid recoil motion shortly after the commencement of ventricular repolarization. Changes in overall longitudinal motion can also be appreciated using global longitudinal strain rate curves , followed by a relatively constant shorthening rate throughout the rest of the rapid and reduced ejection. Ventricular repolarization was followed by a sudden drop in longitudinal shortening , with a subsequent peak of longitudinal lenthening in early diastole .Both systolic and diastolic velocities were higher in basal segments, compared with midventricular and apical segments, showing greater displacement of the LV base in a longitudinal direction. The septal segments, which are a direct continuation of great vessels, showed lower values of peak systolic longitudinal motion . Similarly, contraction of myocardial aggregates limited to the left ventricle  and a counter-clockwise motion of the apex, as observed in the velocity graphs. Since these aggregates are shorter, net motion only becomes apparent after the initial counter-clockwise rotation of the entire left ventricle, which is dominated by the longer (type 1) aggregates. The contraction of both type 1 and type 2 aggregates together may therefore explain the displacement of the ventricular apex anteriorly , whilst the simultaneous contraction of the outer surface aggregates limited to the left ventricle (type 2), explains the clockwise rotation of the LV base and the counter-clockwise movement of the apex  are tethered in the mediastinum and thorax by large vascular and pericardial attachments, and which  are free to rotate. This is evident at the beginning of ventricular repolarization where there is a sudden change in movement direction occurring only at the apex (Figure Longitudinal motion of the LV during systole may be explained by contraction of the predominantly longitudinally oriented epi- and endocardial, trabecular and papillary myocytes. Repolarization leads to cessation of active myocardial contraction, although minimal blood flow still occurs at the end of reduced ejection due to the kinetic energy of the ejected blood . There aVentricular filling is likely to have multiple contributing factors. Firstly, a passive component, where repolarization leads to cessation of myocardial contraction and to ventricular wall relaxation, allowing blood to be expelled from the atria. Whether a suction component results from ventricular untwisting remains controversial ,22. We aThe complex pattern of LV motion can be explained by the helical orientation of cardiomyocytes in the healthy heart. From these results we believe that left ventricular torsion is mainly a consequence of the relative orientations and moments of torque of the sub-epicardial relative to the sub-endocardial myocyte layers, with influence from tethering of the heart to the mediastinum and great vessels and the directional forces associated with blood flow. Whether assessment of these detailed systolic and diastolic parameters would assist in the early diagnosis of cardiac disease requires further evaluation.The authors declare that they have no competing interests.IC and CJH drafted the manuscript, MDR and BAJ participated in data interpretation and manuscript revision, SJG and KC were involved with study design and manuscript editing. All authors read and approved the final manuscript.


It is well established that cardiac resynchronisation therapy (CRT) using biventricular pacing  prolongs survival by its effects on pump failure. The rate of sudden cardiac death in patients undergoing CRT, however, remains high. Animal and human studies have shown that reversal of normal sequence of myocardial activation during epicardial pacing, as applied during CRT, increases the transmural dispersion of repolarisation (TDR), a substrate for ventricular arrhythmias. Cohort studies in humans suggest that CRT has a differential effect on the arrhythmogenic substrate, antiarrhythmic in some and proarrhythmic in others. This review the focuses on the possibility that CRT may, under certain circumstances, promote arrhythmogenesis. Cardiac resynchronisation therapy (CRT) using biventricular pacing (BiVP) has revolutionised the treatment of heart failure. In the Cardiac Resynchronization Heart Failure (CARE-HF) study, which included patients with with a QRS complex ≥120 ms, NYHA class III or IV and a left ventricular ejection fraction of ≤35%, CRT was associated with a 36% reduction in all-cause mortality [The rate of sudden cardiac death remains high in patients with heart failure who are treated with CRT . In the Increasing evidence indicates that the myocardium is electrically and mechanically heterogenous. Reversal of normal sequence of myocardial activation following epicardial pacing, as it occurs during CRT, has been shown to increase the TDR, a substrate for ventricular arrhythmias ,8. TheseThe concept of electrical heterogeneity in the myocardium arose from a seminal study in 1991, in which Sicouri and Antzelevitch described a subpopulation myocardial cells with distinct electrophysiological properties, known as M cells . Figure ElectricSurrogate measures of electrical heterogeneity can be found in the12-lead ECG, which is, in effect, a single image of electrical activity of the myocardium propagated in three dimensions. The QT interval is a macroscopic measure of ventricular repolarisation which reflects, ultimately, the cellular action potential. The unipolar chest leads from V1 to V6 more accurately reflect local activity [The morphology of the T wave reflects voltage gradients generated by epicardial, endocardial and M layers. The peak of the T wave corresponds to full repolarisation of the epicardial action potential, whereas the end of the T wave coincides with repolarisation of the M cells. The length of the QT peak interval therefore depends on the duration of the epicardial action potential, whereas the QT interval duration reflects the duration of the M-cell action potential.peak-Tend has been linked to spontaneous development of ventricular tachycardia and, interestingly, with increased inducibility [peak-Tend marks the presence of an arrhythmogenic substrate.The Tpeak-Tend interval provides a measure of the transmural dispersion of repolarisation (TDR) ,13-16. Acibility . In effeAlthough QT dispersion has been shown to predict mortality in population based studies , and in peak-Tend interval are dependent on the activation sequence of the myocardium. In an elegant study using mathematical modelling, Fish et al [peak-Tend interval. In heterogenous myocardium consisting of epicardium, endocardium and M cells, reversal of the transmural direction of activation does lead to a prolongation of TDR and the Tpeak-Tend interval, reflecting the fact that the epicardium depolarises and repolarises earlier and the M calls depolarise and repolarise later.The QT interval, the morphology of the T wave and the Tsh et al  demonstrpeak-Tend compared to RV endocardial pacing. In dogs with DCM, both mean action potential duration and transmural dispersion of repolarisation increased with LV-epicardial and biventricular pacing. This group proposed that such changes could result in the formation of unidirectional block and re-entry, a phenomenon which is linked to the development of malignant ventricular arrhythmias.Bai et al studied the effects of epicardial LV pacing and biventricular pacing in an experimental model of dilated cardiomyopathy in dogs . In contIn experiments using isolated arterially perfused rabbit LV wedge preparations, Medina-Ravell et al  have recIn humans, reversal of the normal sequence of activation has similarly been linked to arrhythmogenesis. Medina-Ravell et al  showed tEarly studies suggested that CRT exerts an antiarrhythmic effect. Paul's group showed that CRT significantly reduced ventricular ectopic counts when compared to isolated right ventricular pacing . In the Although the CARE-HF Extension study reported a reduction in sudden cardiac death following CRT , such anIncreasing interest is being focused on factors which might predict benefit from CRT. A panoply of echocardiographic studies have explored numerous measures of dyssynchrony , but onlWe sought to determine whether QT interval duration and QT dispersion prior to and following implantation predict major arrhythmic events (MAE) following CRT . In a stpeak-Tend interval following CRT, we found that CRT led to an overall reduction in the Tpeak-Tend interval, averaging -16.5 ms for a cohort of  75 patients [peak-Tend of 81 ±  13.8 ms (mean ± SD). It would appear from this standard deviation that a reduction in Tpeak-Tend interval was not found in all patients. The salient finding from our study is that CRT has a differential effect on the the Tpeak-Tend interval and that this is related to the development of MAE.With regard to the Tpatients . The redOur demonstration that CRT is apparently proarrhythmic is some patients is relevant to the observation from the CARE-HF study that CRT reduces all-cause mortality but not sudden cardiac death . ArguablAs discussed above, CRT appears to have a differential effect on QT dispersion, which is related to the risk of developing ventricular tachyarrhythmias. No studies have explored which factors are responsible for this differential effect. Intuitively, patients with worse left ventricular function may be at an increased risk. In this respect, Pai et al have shown that in patients with heart failure, QT dispersion is negatively related to ejection fraction . In a stPerfusion may also influence the arrhythmogenic substrate. Bonnemeier et al have shown that QT dynamicity, a temporal measure of QT dispersion, is increased following percutaneous coronary intervention in patients with TIMI 2 flow, compared to patients with TIMI 3 flow , suggestIt is well established that in patients with ischaemic -38 and nVentricular dyssynchrony may also influence the arrhythmogenic substrate. In the study of Spragg et al , adult dThe incidence of sudden, presumed arrhythmic, deaths in patients with heart failure treated with CRT remains high, comparable to patients receiving pharmacological therapy alone. Both animal and human experimental evidence indicates that reversal of the normal direction of myocardial activation, as it occurs during conventional CRT, promotes arrhythmogenesis. Such findings are consistent with human experiments showing that epicardial pacing leads to increased transmural dispersion of repolarisation and ventricular arrhythmias. Studies of patients undergoing CRT have shown that CRT has a differential effect on QT dispersion, with some patients exhibiting an increase above pre-implant values. Such an increase in QT dispersion carries a risk of arrhythmic events.Little is known about which factors might modulate the arhythmogenic substrate in patients undergoing CRT. Identification of factors that render some patients susceptible to the putative arrhythmogenic effects of CRT may be useful in patient selection. Further studies are needed to determine whether underlying left ventricular function, dyssynchrony and the site of the LV pacing lead in relation to myocardial scars are relevant in this respect. An alternative solution to the apparent arrhythmogenic effects of CRT is to use LV endocardial pacing, as this would preserve the natural direction of myocardial activation.


Due to the rise in the number of reports of stress cardiomyopathy in the literature, awareness of this condition is increasing. Although different names have been used to describe this condition, the similarities in clinical, electrocardiographic, echocardiographic, and angiographic features suggest that they represent the same spectrum of diseases with different underlying causes. The pathophysiology of stress cardiomyopathy remains controversial.We describe a series of four cases of stress cardiomyopathy admitted to our institution over a period of six months with different presentations, but similar clinical course, EKG, echocardiographic, and catheterization findings. The ages ranged from 22 to 81 years; all four females. All showed characteristic wall motion abnormalities by imaging in the absence of significant coronary artery disease, with spontaneous recovery of left ventricular function with conservative therapy.Although the patients presented with different clinical scenarios, all four showed characteristic features of stress cardiomyopathy suggesting that the pathophysiology affecting the myocardium was the same. We present a review of the literature with a discussion of the history of this condition, characteristic clinical features, and diagnostic criteria used in the past as well as the suggested pathophysiology of this condition.Stress cardiomyopathy is an underdiagnosed reversible cardiomyopathy triggered by severe emotional or physical stress. It represents a spectrum of conditions with reversible severe left ventricular systolic dysfunction that includes neurogenic cardiomyopathy. It is not confined to the Japanese population and can affect people of any ethnic background or nationality. Stress cardiomyopathy is one of the few reversible cardiomyopathies. First described in 1990, awareness and recognition of this condition is increasing. We report here on a series of four cases that presented to our institution within a period of six months.A review of the literature reveals characteristic clinical, echocardiographic, and angiographic features that overlap with neurogenic cardiomyopathy, which leads one to believe that these are not separate entities, but are representations of the same spectrum of disease with different underlying causes.A 57-year-old, white female with a history of longstanding rheumatoid arthritis, hypertension, and fibromyalgia was admitted six weeks after kyphoplasty with a complaint of severe anterior chest pain, odynophagia, and generalized weakness. Physical examination was remarkable only for diffuse joint deformities and oral candidiasis. EKG on admission showed diffuse T wave inversion  in the pShe was treated with ACE inhibitors and continued antihypertensive therapy. Further investigations revealed the presence of severe erosive esophagitis secondary to candida albicans, which was treated with antifungal agents prior to discharge home.She returned to cardiology clinic for follow-up about six weeks later and repeat echocardiography showed normal left ventricular systolic function with no regional wall motion abnormality.A 57-year-old lady with a known history of chronic obstructive pulmonary disease (COPD), on home oxygen, mild pulmonary hypertension, and history of anemia, was brought to the ER with complaints of increased shortness of breath due to COPD exacerbation. The patient required intubation due to respiratory failure. It was noted that her ECG showed diffuse ST-T wave abnormalities and cardiac enzymes demonstrated an increased troponin level up to 0.87 ng/mL. She had a heart catheterization that showed an ejection fraction of 10% with normal epicardial coronary arteries. Echocardiography confirmed this finding with severe hypokinesis in the mid and apical segments of the left ventricle. Repeated echocardiogram at one month showed normal global left ventricular systolic function with normal ejection fraction of 60% with no wall motion abnormalities.An 81-year-old, hypertensive and diabetic, African-American female was admitted to the surgical service with intractable nausea and vomiting. She was diagnosed with gallstone pancreatitis. Shortly after administration of intravenous cefazolin, she developed an acute allergic reaction manifesting as wheezing, dyspnea, and hypertensive urgency. Over the next 12 hours, she progressed to respiratory distress, requiring intubation and ventilatory support.EKG showed ST elevations in precordial leads V2 to V6. Cardiac enzymes showed a peak troponin of 1.47 ng/mL. A cardiac catheterization was performed which showed mild nonobstructive coronary artery disease. Left ventriculogram showed basal hyperkinesis with apical and anterior wall hypokinesia. Echocardiogram performed four days later revealed normal systolic function with no regional wall motion abnormality. She remained hemodynamically stable throughout her stay and was discharged home after a laparoscopic cholecystectomy three weeks later.A 22-year-old, white female with a history of adult polycystic kidney disease, hypertension and depression was admitted to the neurosurgery service from a community hospital where she presented with severe sudden onset headache and had been diagnosed with subarachnoid hemorrhage. Diffuse T wave inversions developed in the precordial leads on the EKG over the next 24 hours and troponin elevation to 4.89 was noted. Echocardiography revealed severely reduced ejection fraction of 28% with akinesis of the distal two thirds of the ventricle and basal hyperkinesis. Although the EKG remained abnormal, she remained hemodynamically stable and denied any significant chest symptoms. She appeared to improve from the neurological standpoint over the next four days.Unfortunately, she developed another subarachnoid hemorrhage two days later, which resulted in her death. Repeat echo on the day of her death, prior to removal of life support, showed return of cardiac function to normal with an ejection fraction of 56% and no regional wall motion abnormality.Stress cardiomyopathy is a severe but reversible form of left ventricle dysfunction that mimics acute myocardial infarction in its presentation, with the absence of any obstructive coronary artery disease.In the large majority of cases, ballooning of the akinetic apical segments of the left ventricle is seen. Pavin et al reported first case from France.Originally thought to mainly affect the Japanese population, cases have since been reported from the USA, Brazil, Germany, Belgium,Although the true incidence of stress cardiomyopathy is unknown, it has been reported to represent approximately 1.2%–2.2% of cases admitted as acute myocardial infarctions in the USA.Stress cardiomyopathy is characterized by severe reversible systolic dysfunction typically affecting the apical and mid segments of the myocardium. The majority of reported cases have been postmenopausal females. Patients present with severe chest pain and/or dyspnea. EKG shows ST segment elevation in the precordial leads with gradual resolution of ST elevation and/or inversion of T waves in most cases. Tachy-arrhythmia, like ventricular tachycardia and ventricular fibrillation, might be the initial presentation, and death can occur in rare cases.13The pathophysiology of stress cardiomyopathy remains unclear. Many hypotheses are considered, which include catecholamine-triggered injury and endothelial dysfunction which have many supportive data. Epicardial coronary spasm and obstruction of the left ventricle outflow tract have minimal and sporadic data and are becoming less viable.Cases of myocardial stunning in patients with large subarachnoid hemorrhages16If catecholamines are indeed the cause of myocardial dysfunction, the question then arises as to how they cause stunning of the myocardium. It has been proposed that catecholamine induced spasm of the epicardial coronary arteries or dysfunction of the coronary microcirculationThese findings of catecholamine cardiotoxicity might lead to high intracellular calcium concentration and it was proposed that overload of calcium might lead to cardiac dysfunction.21In the case series from Minneapolis-St. Paul by Sharkey et al, cardiac magnetic resonance (CMR) assessments showed the absence of delayed Gadolinium hyperenhancement, a finding usually seen in patients with myocardial scar or infarction, in 21 out of the 22 patients with stress cardiomyopathy.25Endothelial dysfunction has been proposed as one of the features of stress cardiomyopathy as well.Feola et al, studied the myocardial blood flow (MBF) and coronary flow reserve (CFR) by use of positron emission tomography (PET) scan at presentation and after three months.The criteria proposed by Bybee et al from the Mayo Clinic for the clinical diagnosis of stress cardiomyopathy, excludes patients with intracranial bleeding, pheochromocytoma, and recent significant head trauma.31In conclusion, we proposed to revise the Mayo Clinic diagnosis criteria by omitting the last criteria that exclude the pheochromocytoma and neurogenic pathology . In our Stress cardiomyopathy represents a spectrum of conditions manifesting as reversible severe systolic dysfunction, with characteristic EKG, echo, and angiographic findings. It is not confined to the Japanese or Caucasian population, and may affect patients of any ethnicity. Cardiomyopathy secondary to severe neurological events like subarachnoid hemorrhage, status epilepticus, or intracranial trauma is probably a manifestation of the same condition and not a separate entity. The true incidence of stress cardiomyopathy is probably higher than currently estimated, as it remains unrecognized and under-reported in many centers. Although the exact mechanism of stress cardiomyopathy remains not fully understood, it appears that the activation of the sympathetic nervous system, resulting in a surge of catecholamines due to severe emotional or acute physical disease, probably plays a central role in the pathology of this condition.


Left ventricular end diastolic volume (EDV), end systolic volume (ESV), mass, and ejection fraction (EF) were calculated for each method.Twenty-six subjects  underwent multiple breathhold 2D balanced turbo-field echo cine CMR in addition to k-t SENSE methods before contrast . Agreement improved following contrast . The 3D method underestimated all parameters compared to 2D . 3D image quality scores were significantly poorer than 2D, showing a non-significant trend to improvement following contrast administration. Parameters derived with k-t acceleration factors of 7 and 10 showed poorer agreement with 2D values.There was at least moderate agreement between the EDV, ESV, mass and EF calculated by 2D and 3D 5× k-t SENSE accelerated CMR. Contrast administration further improves agreement between 5× k-t SENSE and 2D-derived measurements. k-t acceleration factors greater than 5, though feasible, produce poorer agreement with 2D values.Left ventricular volumes and mass are reliably assessed using 3D 5×  The assessment of ventricular volumes and mass is an essential component of any cardiovascular magnetic resonance (CMR) study. Numerous studies have shown that CMR provides the most accurate and reproducible measurements of left ventricular dimensions -7, as itk-t SENSE) method allows substantial acceleration of data acquisition by applying sparse sampling along the spatial frequency (k) and temporal (t) encoding axes  × 100%. Left ventricular mass (LV mass) was calculated from the diastolic phase as LV mass = 1.05 g/cmStatistical analysis was performed using SPSS version 15.0. Mean values +/- SD were calculated for all contour-related measurements. The mean bias between the methods and their limits of agreement were calculated according to the method described by Bland & Altman . Lin's cThe reproducibility of left ventricular volume assessment for each technique was assessed using a sample of ten participants (five volunteers and five patients). In order to assess intraobserver variability, left ventricular contours were redrawn several months after the original contours, and without reference to them, by the original observer (AL). An additional observer (NM) independently drew contours using the same images in order to assess interobserver variability. The error between repeated measurements was assessed in the manner described by Bland and Altman . It was Data acquisition was successfully completed in all subjects. Figure k-t accelerated images were noted to suffer from some temporal blurring and a curved artefact, which varied in intensity between phases  are shown in table k-t SENSE technique demonstrated moderate agreement with 2D imaging for estimation of EDV and EF (concordance coefficients 0.92 for both), and substantial agreement for ESV and LV mass (concordance coefficients 0.95 and 0.97 respectively). The administration of contrast improved concordance coefficients for all parameters, producing substantial agreement for EDV, ESV and LV mass  and moderate agreement for EF (concordance coefficient 0.93). This improvement is also reflected by a reduction in the limits of agreement for the bias between 2D and 3D 5× k-t SENSE for all parameters after contrast administration .k-t acceleration factors of 7 and 10 was feasible. The results obtained using higher acceleration factors are shown in table k-t SENSE, which agrees substantially with the 2D value (concordance coefficient 0.96). However, the small sample size means that the confidence intervals are wide and the data should therefore be interpreted with caution.Acquisition with higher As demonstrated in table k-t SENSE-accelerated 3D CMR cines can be used to reliably assess left ventricular mass, ejection fraction, end systolic and end diastolic volumes.This study shows that k-t SENSE acceleration method presented here, a full LV study, with temporal and spatial resolution equivalent to the 2D reference method, could be acquired using a single 14-second breathhold followed by a 4-second breathhold for training data. This compares to several minutes for a full LV cine study using a conventional 2D approach. The differences observed between LV parameters derived from 2D and 3D k-t accelerated images in this study are sufficiently small to be irrelevant in most clinical scenarios. The rapid and reliable acquisition of left ventricular images through the 5× k-t SENSE acceleration method will be particularly of benefit when scanning unstable patients who are unable to tolerate long periods of time in the MR scanner.Using the 5× k-t SENSE accelerated images, though these differences did not prove statistically significant. However, the trend towards an improvement in image quality scores was mirrored by an improvement in the agreement between 2D and 3D volumetric measurements following contrast. In many CMR studies, contrast agents are administered for other components of the study, so that contrast-enhanced 3D cine imaging will not necessitate additional contrast administration. In most instances, conventional contrast agents, such as the one used in this work, will be chosen rather than intravascular agents. Although intravascular contrast has shown some advantages for 3D cine imaging and might further improve the delineation of the blood pool versus myocardium [The administration of T1 shortening contrast media reduced artefacts and improved the image quality scores of the 5× ocardium  our resuk-t reconstruction algorithm assumes a cyclic temporal function, discontinuities between the last and first phases of the acquisition can lead to reconstruction errors in the first and last heart phase images. Since we defined EDV as the first phase of the acquisition, such errors may have contributed to its underestimation. With retrospective gating, which has recently been implemented for k-t SENSE [It is interesting to note that, despite generally good agreement between the techniques, there was a systematic underestimation of LV volumes, ejection fraction and mass with the 3D methods compared with the 2D reference. There are several explanations for the differences between 2D and 3D acquisition results. During 3D imaging, a reduction in the inflow of unsaturated blood can reduce endocardial border definition and thereby influence contour placement. Furthermore, individual 2D slices are generally better defined than in 3D acquisition, where the reconstructed slices suffer from more partial volume effects due to the extent of the point-spread function in the slice selection direction. The appearance of the endocardial border therefore differs between the two methods. With 2D techniques, spatial misregistration can also affect volumetric measurements, although this would be expected to have a random effect. Finally, when prospective gating is used in data acquisition as used in the present study, the cardiac cycle may not be covered in its entirety, in particular if the actual heart rate differs from the anticipated heart rate. Because the -t SENSE , the potk-t BLAST, and prospective triggering, have shown similar results. A study of 40 patients by Jahnke et al has reported a mean bias for LVEDV of 4.9%, similar to the result of our study, with better agreement for ESV and EF [k-t BLAST acceleration vs. 2D cine: EDV 5 ml, ESV 1 ml, LV mass -0.9 g, EF 0.5%), and further demonstrated the tendency to underestimation of left ventricular volumes using k-t acceleration [Two recent studies using a similar acceleration method, V and EF . Meanwhileration . The conk-t SENSE is significantly more SNR efficient. The main factors limiting the degree of acceleration with this technique are temporal blurring and associated artifacts. In this study, up to 10-fold k-t acceleration factors were tested and found to be feasible in a small group of volunteers. For 10-fold acceleration, data acquisition required only a nine-second breathhold followed by a four-second breathhold for training data. At these higher acceleration factors however, temporal blurring and artefacts were observed on qualitative analysis. Consequently, the measurements made using these images showed poor agreement with the 2D reference results.Traditional parallel imaging techniques are generally limited by decreasing signal to noise ratios (SNR) as acceleration factors increase. However, acceleration by k-t acceleration factors of 7 and 10 were only tested in six volunteers. Though this establishes the feasibility of the technique, larger scale studies will be needed to determine whether our finding of poor agreement with the reference standard is reproduced.The higher k-t SENSE accelerated 3D left ventricular cine imaging can be used to reliably measure most clinically important left ventricular parameters. Images produced using a k-t acceleration factor of 5 generally showed good agreement with the 2D reference, particularly after the administration of contrast agent. Measurements derived from images using higher k-t acceleration factors of 7 and 10, though proven to be feasible, showed poorer agreement with the reference technique. Further studies with larger groups of patients will be needed to determine the role of k-t SENSE accelerated 3D cine CMR imaging in routine clinical practice.This study demonstrates that 2D mid-ventricular slice cine. This movie shows a mid-ventricular slice cine, taken with a 2D sequence.Click here for filek-t mid-ventricular slice cine, before contrast. This movie shows a mid-ventricular slice cine, taken with a 3D 5× k-t sequence, before the administration of contrast.3D 5× Click here for filek-t mid-ventricular slice cine, after contrast. This movie shows a mid-ventricular slice cine, taken with a 3D 5× k-t sequence, after the administration of contrast.3D 5× Click here for filek-t mid-ventricular slice cine. This movie shows a mid-ventricular slice cine, taken with a 3D 7× k-t sequence.3D 7× Click here for filek-t mid-ventricular slice cine. This movie shows a mid-ventricular slice cine, taken with a 3D 10× k-t sequence.3D 10× Click here for file


Cardiovascular magnetic resonance (CMR) imaging has evolved rapidly and is now accepted as a powerful diagnostic tool with significant clinical and research applications. Clinical 3 Tesla (3 T) scanners are increasingly available and offer improved diagnostic capabilities compared to 1.5 T scanners for perfusion, viability, and coronary imaging. Although technical challenges remain for cardiac imaging at higher field strengths such as balanced steady state free precession (bSSFP) cine imaging, the majority of cardiac applications are feasible at 3 T with comparable or superior image quality to that of 1.5 T. This review will focus on the benefits and limitations of 3 T CMR for common clinical applications and examine areas in development for potential clinical use. Cardiovascular magnetic resonance (CMR) has become a widely adapted imaging modality for the diagnosis of cardiovascular disease, and its clinical indications have expanded greatly in the last decade . Recentl0) [0 field homogeneity should be optimized over the heart with localized second order shimming and correct determination of the imaging frequency [1), currently implemented shim routines do not completely shift dark band artifacts from cardiac structures in all cases [For cardiac cine imaging, balanced steady-state free precession (bSSFP) techniques are employed at 1.5 T and are considered the gold standard for evaluating cardiac function . The app0) . High si0) . They de0)  and one 0) . There a0) : First, requency -19. Althll cases . Spoiled gradient echo (GRE) techniques are applied at 3 T for cine imaging and have shown superior results in comparison to 1.5 T , 21. A mMyocardial tagging has been increasingly employed for the accurate, semi-automated analysis of myocardial wall motion and strain measurement . The tec2) and may reduce the occurrence of dark ring artifacts. Significant improvements in SNR and overall image quality have been reported for perfusion imaging at 3 T [Myocardial perfusion magnetic resonance (MR) imaging has evolved considerably over the past decade and is used to assess the significance of coronary artery stenosis and microvascular dysfunction on the myocardium. Clinical studies performed with 1.5 T scanners have shown that MR perfusion imaging yields superior diagnostic results for the detection of CAD to clinically established nuclear perfusion techniques -31. By cg at 3 T , 32. Oneg at 3 T . A receng at 3 T . Dobutamine stress CMR has become a well established modality for the diagnosis of myocardial ischemia. It has improved sensitivity and specificity for the detection of myocardial ischemia when compared with other stress techniques such as dobutamine stress echocardiography and is beneficial in patients with poor acoustic windows for echocardiography . DobutamThe measurement of late gadolinium enhancement (LGE) of the myocardium using gadolinium has been broadly accepted in recent years as the imaging method of choice to evaluate myocardial scar . The tec3). One advantage is the possibility of reducing the dose of contrast agent at 3 T [There are several recent studies that examine potential benefits of 3 T imaging in LGE  provides a non-invasive, safe means to evaluate the coronary arteries, and may be improved at 3 T . An initThe use of CT to perform coronary angiography for the diagnosis of CAD has been progressing rapidly. A recently performed multi-center trial using multi-detector 64-Row CT reported a sensitivity of 85% and a specificity of 90% in detecting coronary stenoses > 50% on x-ray coronary angiography . HeavilyA recent meta-analysis of 51 studies that each examined coronary CTA or MRA reported a significantly higher sensitivity and specificity for CTA  compared with that of coronary MRA  for the detection of significant coronary stenoses . Actually, there are no data available comparing state of the art coronary CT and coronary MRA at 3 T, e.g. favoring the use of a 32-channel receiver coil and a contrast-enhanced whole-heart approach .4). In a study of patients with CAD, a free-breathing, navigator-gated technique for 3D coronary black blood imaging showed increased coronary vessel wall thickness in patients with mild CAD when compared to a healthy control population [Coronary vessel wall imaging using CMR permits the non-invasive quantification for “Glagov-type” outward arterial remodeling and allows for the accurate measurement of vessel wall thickness . Becausepulation . Prelimipulation , 54. 31P) MRS [1H) MRS [Cardiac MR spectroscopy (MRS) enables the non-invasive assessment of high-energy phosphate metabolism and even flux through the creatine kinase reaction , 56 when31P) MRS -61 or th[1H) MRS -64. Curr[1H) MRS . Cardiac[1H) MRS , 67 and Cardiac MR imaging has evolved rapidly and is now accepted as a powerful diagnostic tool with significant clinical and research applications. Although technical challenges remain for cardiac imaging at higher field strengths such as bSSFP cine imaging, the majority of cardiac applications are feasible at 3 T with comparable or superior image quality to that of 1.5 T. Imaging at 3 T particularly benefits protocols with sub-optimal SNR such as cardiac perfusion imaging, delayed enhancement imaging and myocardial tagging techniques. The integration of parallel imaging, motion compensation, and shimming algorithms at 3 T will contribute to further improvements of imaging quality and shorter scanning times.


Correlate the anatomic features of atrioventricular septal defect with echocardiographic images.Sixty specimen hearts were studied by sequential segmental analysis. Echocardiograms were performed on 34 patients. Specimen hearts with findings equivalent to those of echocardiographic images were selected in order to establish an anatomo-echocardiographic correlation.Thirty-three specimen hearts were in situs solitus, 19 showed dextroisomerism, 6 were in situs inversus and 2 levoisomerism. Fifty-eight had a common atrioventricular valve and 2 had two atrioventricular valves. Rastelli types were determined in 21 hearts. Nine were type A, 2 intermediate between A and B, 1 mixed between A and B, 4 type B and 5 type C. Associated anomalies included pulmonary stenosis, pulmonary atresia atrial septal defect, patent ductus arteriosus and anomalous connection of pulmonary veins. Echocardiograms revealed dextroisomerism in 12 patients, situs solitus in 11, levoisomerism in 7 and situs inversus in 4. Thirty-one patients had common atrioventricular valves and three two atrioventricular valves. Rastelli types were established in all cases with common atrioventricular valves; 17 had type A canal defects, 10 type B, 3 intermediate between A and B, 1 mixed between A and B and 3 type C. Associated anomalies included regurgitation of the atrioventricular valve, pulmonary stenosis, anomalous connection of pulmonary veins, pulmonary hypertension and pulmonary atresia.Anatomo-echocardiographic correlation demonstrated a high degree of diagnostic precision with echocardiography. Atrioventricular septal defect is characterized by a lack of separation between the right atrium and the left ventricle, creating communication between the two sides of the heart with a variety of aberrations of the atrioventricular valves, ventricular geometry, the fibrous skeleton and the cardiac conduction system . This coEchocardiography has provided precise images of the anatomic findings of this congenital malformation with details of the atrioventricular septal defect, the common atrioventricular valve or two separate atrioventricular valves, the relationships between the leaflets of the valves in both forms, septal structures and their defects, disproportion between the left ventricular inflow and outflow tracts, unwedged aorta and the shunts that determine the clinical presentation ,5,6. ThiThis study presents an anatomo-echocardiographic correlation that compares the anatomic features of specimen hearts with atrioventricular septal defect to echocardiographic images of equivalent findings in patients with this congenital malformation. These comparisons should aid in diagnostic interpretation -12.Sixty specimen hearts from the Department of Embryology of the Instituto Nacional de Cardiología "Ignacio Chávez" with atrioventricular septal defect were examined. Each specimen was described following the guidelines of the segmental sequential system utilized in the diagnosis of congenital heart defects . Atrial Between May 2002 and December 2006 thirty-four adult patients from adult outpatient clinic with clinical suspicion of atrioventricular septal defect were studied. Fourteen were women and 20 men with an average age of 24.95 ± 6.94 years.All patients underwent complete clinical history and transthoracic and/or transesophageal echocardiograms. The echocardiographic study was performed using a Philips Sonos 5500 machine with an S3 electronic probe and multiplane transesophageal probe.Abdominal situs was determined from the subcostal plane, and the atrial situs on the basis of the morphology of the atrial appendages visualized in the apical 4-chamber view. Atrioventricular valve features and Rastelli type  were evaWhen the patient had a poor acoustic window and/or when diagnosis was in doubt transesophageal echocardiography was performed using a multiplane probe.Anatomo-echocardiographic correlation was demonstrated by establishing the correspondence between the anatomic feature of the specimen and the equivalent echocardiographic image from a patient with the same type of malformation.Atrial situs was solitus in 33 hearts, 19 had dextroisomerism, 6 had situs inversus and 2 had levoisomerism. All presented a common fibrous atrioventricular ring. Fifty-eight hearts had a common atrioventricular valve, and two had two separate valves. All had atrioventricular septal defects. Hearts with common atrioventricular valves had ventricular septal defects. In those with two atrioventricular valves this ventricular septal defect was obliterated by the insertion of the left atrioventricular septal anterior and posterior leaflets in the crest of the ventricular septum. Ostium primum was present in 58 hearts. In two the atrial septum was well developed and there was no ostium primum. The two types of atrioventricular septal defect manifested all hallmarks of the atrioventricular septal defect: excavation of the ventricular septum, disproportion of the left ventricular inflow tract and outflow tract, unwedged aorta, a common fibrous ring, absence of the membranous septum, atrioventricular valves on the same plane, and elongation of the left ventricular outflow tract. In two specimens's insertion of the chordae tendineae of the left antero-superior leaflet caused obstruction of the left ventricular outflow tract.Hearts with one valve had a five leaflet pattern: two (right and left) antero-superior leaflets, two (right and left) lateral leaflets and a common posterior leaflet. Hearts with two atrioventricular valves had a pattern of three leaflets.Rastelli types were established in twenty-one hearts. Nine were type A, two were intermediate between A and B, one was a mixture of types A and B, four were type B and five were type C. Rastelli types were not determined in thirty-nine hearts because 10 had double inlet to a single ventricle, nine a right ventricular double inlet and eight a left ventricular double inlet. Eleven were surgically altered and one had deterioration.Atrioventricular and ventriculo-arterial connections as well as associated anomalies are shown in Table Echocardiographic studies showed dextroisomerism in twelve patients, atrial situs solitus in eleven, levoisomerism in seven and situs inversus in four. Thirty-one patients had common atrioventricular valves and three had two separate valves.All of the patients had atrioventricular septal defects. Ventricular septal defects were found in the group with common atrioventricular valve; obliterated in the group with two atrioventricular valves by the insertion of the septal anterior and posterior leaflets of the left atrioventricular valve. Ostium primum was present in thirty-one hearts, while in three the atrial septum was well developed and no ostium primum was found.As in the anatomic specimen hearts, patients with a common atrioventricular valve had a five leaflet pattern: two (right and left) antero-superior leaflets, two (right and left) lateral leaflets and a common posterior leaflet. Hearts with two atrioventricular valves had a pattern of three leaflets.Rastelli types were determined in all cases with common atrioventricular valves. Seventeen had type A, ten type B, three intermediate between A and B, three type C and one a mixture of types A and B.Atrioventricular and ventriculo-arterial connections and associated anomalies are shown in Table A comparison of the anatomic features of the atrioventricular canal with corresponding images obtained from patients with the same condition showed strong correlation.In the common valve type the chambers open into a wide canal with ostium primum, atrioventricular septal defect and ventricular septal defect. In Rastelli type A the chordae tendineae of the anterior leaflets insert in the crest of the ventricular septum Fig. . The apiIn the atrioventricular septal defect with two separate valve orifices these are contained within the common atrioventricular junction. The wide septal defect includes the ostium primum and the atrioventricular septal defect. The septal leaflets of the left atrioventricular valve insert in a continuous manner in the crest of the ventricular septum, obliterating the ventricular septal defect Fig. . The apiThe view of the atrioventricular septal defect with two valve orifices from the right chambers shows a well-developed atrial septum. The fusion of the septal leaflets of the left atrioventricular valve on the crest of the ventricular septum and the commissure that separates these septal leaflets, as well as the broad commissure between the septal and anterior leaflets of the right atrioventricular valve can be seen through the wide septal defect Fig. . The 4-cThe two-dimensional echocardiographic apical 4-chamber image with color Doppler of an atrioventricular septal defect with two valve orifices makes it possible to observe the atrial shunt above the atrioventricular valves  and in the papillary muscle adjacent to the ventricular septum  to the papillary muscle tethered to the apical portion of the ventricular septum Fig. . In the In a view from the right chambers of a Rastelli type C atrioventricular septal defect the left anterior leaflet can be seen to pass over the ventricular septum and insert in the papillary muscle in the right ventricular free wall Fig. . The 4-cFigure The Rastelli type C insertion of anterior leaflets and ventricular septal defect are shown in an atrioventricular septal defect with common atrioventricular valve in situs inversus Fig. . Color DIn atrioventricular septal defect with common atrioventricular valve the "scooped out" appearance of the ventricular septum can be seen, as well as the interchordal spaces forming the ventricular septal defect Fig. . The 4-cA comparison of the left ventricles in atrioventricular septal defect with common atrioventricular valve and with two separate valves shows the similarity of the insertions of left anterior leaflets in the two types. In the former there are interchordal spaces and in the latter continuous insertion and obliterated ventricular septal defect. In both types the atrioventricular septal defect shows a similarity in the shortening of the inlet and elongation of the outlet Fig . The echObstruction of the left ventricular outflow tract by encroachment of the left anterior leaflet of the left atrioventricular valve can be observed in an atrioventricular canal with two valve orifices Fig. . The parIn an atrioventricular septal defect with common atrioventricular valve dilatation of the valve ring produces unwedging of the aortic valve and the fibrous continuity between this valve and the left anterior leaflet of the common atrioventricular valve Fig. . The parAs this anatomic specimen shows, atrioventricular septal defect with common valve can be associated with right ventricular double outlet and Fallot's tetralogy. A Rastelli type C insertion of the left anterior leaflet, pulmonary stenosis and infundibular septum separating the two outflow tracts can be observed Fig. . The echThe atrioventricular septal defect can have a common atrioventricular valve that has equal connections to the two ventricles Fig. . The echLeft dominance is evident in an atrioventricular septal defect with common atrioventricular valve in dextroisomerism. Note that in the postero-superior view both atria have anatomically right characteristics, the valve ring is displaced toward the left ventricle and there is an atrial septal band that is typical of this atrial situs Fig. . The twoAn atrioventricular septal defect can also have right dominance in dextroisomerism. The internal view of the right ventricle reveals the predominant connection of the common atrioventricular valve with this chamber Fig. . The samThe atrioventricular septal defect occurs as a consequence of detained growth and development of the postero-inferior and antero-superior mesenchymal endocardial cushions of the atrioventricular canal. Thus, their fusion, which in the normal heart constitutes the atrioventricular septum separating the right atrium from the left ventricle, does not occur. Instead, a large septal defect is established that includes components at the atrioventricular and ventricular levels and in many cases at the atrial level (ostium primum) ,3.This congenital heart defect can be found in any position (situs) of the heart: situs solitus, situs inversus or in the isomeric positions of dextroisomerism or levoisomerism Fig. , 21. In It is noteworthy that the pathological stigmata of the two anatomic forms of atrioventricular septal defect are practically identical. When the atrioventricular leaflets are removed from the atrioventricular ring in the basal portion of the ventricular mass there is no way to determine whether the patient to whom the heart pertained had a so called "partial" or "complete" type of atrioventricular canal . This clIt is the relationships among the leaflets within the common atrioventricular junction that determine the number of atrioventricular orifices, while the relationships between the leaflets and the septal structures dictate the possible shunts that can be established between the two sides of the heart ,19-22.Other anatomic characteristics that can be determined by ultrasound involve the septal structures and their defects. Classic cases manifest a lack of separation between the left ventricle and the right atrium, as the term atrioventricular septal defect suggests. The absence of the membranous septum and an incomplete muscular ventricular septum create a large perimembranous and inflow tract ventricular septal defect in cases of atrioventricular septal defect with common atrioventricular valve that is obliterated when there are two atrioventricular valves. Finally, failure of closure of the ostium primum originates a large atrial septal defect, although in some cases the ostium primum is absent and the septum is complete . The conIn cases of atrioventricular septal defect with a common atrioventricular valve and a ventricular septal defect the mode of insertion of the right and left anterior leaflets determines the Rastelli type, a classification with significance for surgical correction. In fact, Rastelli types A, B and C constitute the most frequent sites of insertion in a spectrum of possibilities. In this study intermediate insertions between types A and B and mixed A and B insertions in the same heart are shown.As far as the dominance of the left Fig.  or rightOther key characteristics of this cardiac anomaly that are present in types with one and two valve orifices are the unwedged aorta with respect to the atrioventricular valves with a "goose neck" deformity of the left ventricular out flow tract. This is caused by displacement of the common atrioventricular fibrous ring and elongation and displacement of the distance between the apex and the aortic valve. With the echocardiographic 5-chamber apical image it is easy to identify the decrease in the ventricular inlet that reflects a disproportion between it and the elongated outlet: the greater shortening of the inlet greater the elongation of the outlet. This swan's neck shape constitutes a potential postoperative risk of obstruction ,23. On oThe problems of clinical diagnosis can be solved by cross-sectional echocardiography that shows the classic anatomic features of the atrioventricular defect ,19,24.In conclusion, this group of lesions is unified by the anatomical hallmarks of a common atrioventricular junction and deficient atrioventricular septation. An understanding of the anatomy of this congenital malformation is mandatory for clinical diagnosis by imaging. The anatomo-echocardiographic correlation between specimen hearts and echocardiograms of patients with equivalent findings demonstrates the high degree of precision that can be achieved with echocardiographic diagnosis. Validation by anatomic specimens is the defining reference for the imaging that is indispensable for diagnosis, treatment and follow-up of these patients.LMC and NEZ participated to the written manuscript, MKN made the photographs of the heart specimens and helped to draft the manuscript and CK helped to draft the manuscript and made the translation from Spanish into English. All the authors read and approved the final manuscript.


To evaluate if three-dimensional echocardiography (3-DE) is as accurate and reproducible as cine magnetic resonance imaging (cMR) in estimating left ventricular (LV) parameters in patients with and without wall motion abnormalities (WMA).83 patients (33 with WMA) underwent 3-DE and cMR. 3-DE datasets were analysed using a semi-automatic contour detection algorithm. The accuracy of 3-DE was tested against cMR in the two groups of patients. All measurements were made twice by two different observers.LV mass by 3-DE was similar to that obtained by cMR (149 (SD 42) g vs 148 (45) g, p = 0.67), with small bias (1 (28) g) and excellent interobserver agreement (−2 (31) g vs 4 (26) g). The two measurements were also highly correlated (r = 0.94), irrespective of WMA. End-diastolic and end-systolic LV volumes and ejection fraction by 3-DE and cMR were highly correlated . Yet, 3-DE underestimated cMR end-diastolic volumes (167 (68) ml vs 187 (70) ml, p<0.001) and end-systolic volumes (88 (56) ml vs 101 (65) ml, p<0.001), but yielded similar ejection fractions (50% (14%) vs 50% (16%), p = 0.23).3-DE permits accurate determination of LV mass and volumes irrespective of the presence or absence of WMA. LV parameters obtained by 3-DE are also as reproducible as those obtained by cMR. This suggests that 3-DE can be used to follow up patients with LV hypertrophy and/or remodelling. When used in combination with computerised endocardial contour tracking algorithms, it indeed permits faster, more accurate and less operator-dependent quantitation of LV volumes and function than 1-DE or 2-DE.8We have recently developed a computerised semi-automated volumetric contour tracking algorithm that enables both the endocardial and the epicardial contours to be detected with minimal interaction by the investigator. In this context, the aims of the present study were: (1) to validate this new border detection algorithm for quantitation of LV mass by using cMR as the reference method; (2) to assess the variability of 3-DE estimates of LV mass in patients with or without wall motion abnormalities (WMA); and (3) to evaluate the day-to-day variability of LV mass measurements obtained by 3-DE in comparison with those obtained by cMR.The study population consisted of 83 consecutive subjects  years, range 7–85 years) who underwent cMR and 3-DE in random order on the same day. There were 20 volunteers  years, range 7–50 years) and 63 patients with heart disease  years, range 37–85 years), of whom 20 had aortic valve disease, 10 severe mitral regurgitation and 33 a previous myocardial infarction. All subjects were in sinus rhythm. Patients with haemodynamic instability, constant arrhythmia  or any contraindication to cMR  were not considered for inclusion. In five volunteers, 3-DE and cMR were repeated twice, on two different days, to assess test-retest reproducibility. Ten volunteers and 11 patients with previous myocardial infarction also had 3-DE with and without LV opacification using the commercially available ultrasound contrast agent Sonoview . The study protocol was approved by the local ethics committee and all patients gave informed consent before inclusion into the study.A complete M-mode, two-dimensional and Doppler examination was performed using a Sonos 7500 system or IE33  with harmonic imaging. Images were acquired from standard parasternal and apical views, with short breath-holds if needed. The presence or absence of regional wall motion abnormalities (WMA) was assessed by using the 16-segments scoring model proposed by the American Society of Echocardiography . LV mass was derived from M-mode echocardiography as previously described.17Three-dimensional echocardiography was performed using a Sonos 7500 or a IE33 echocardiographic systems  equipped with a dedicated broadband, wide angle, matrix array transducer. Images were acquired from the apical window, with the patient in the left lateral decubitus position. Care was taken to include the entire LV cavity within the pyramidal scan volume. 3-DE datasets were acquired using a wide-angled acquisition (93×80°) mode in which four wedge-shaped subvolumes (93°×20° each) are obtained over four different cardiac cycles during a short breath-hold. Acquisition was triggered to the R wave of every other cardiac cycle to allow time for storage of each subvolume, resulting in a total acquisition time of eight heart beats.cMR imaging was performed on a 1.5 T magnet  using a five-element cardiac synergy coil for signal reception. After localisation of the heart using three-plane and oblique survey images, 10–12 contiguous short-axis cine images were prescribed to cover the entire left ventricle from base to apex. Images were acquired using a multislice cine vectocardiographic (VCG) gated balanced fast-field echo sequence with SENSE during serial breath-holds. Twenty cine phases were acquired using retrospective gating with a temporal resolution varying between 25–50 ms. Slice thickness was 8 mm and slice spacing was 2 mm. Imaging parameters were: repetition time 3.1 ms, echo time 1.6 ms, flip angle 60°, field of view 38 cm, image matrix of 160×128 pixels, SENSE acceleration factor 1.6, 12–20 lines acquired per phase.Images were transferred to dedicated workstations . Patient information was removed. In all patients, measurements were performed in duplicate by two blinded observers, who were both familiar with 3-DE and cMR. To avoid recalling the patient’s images, the blinded readers analysed 3-DE and cMR images on separate days. To assess intra-observer variability, in a randomly selected subgroup of 45 patients, measurements were repeated by one of the readers, one month after the first reading.Image quality of 3-DE images was graded semi-quantitatively by both readers on a five-point scale as 1  =  non-analysable, 2  =  fair, 3  =  moderate, 4  =  good and 5  =  excellent. Measurements of LV volumes, mass and EF were performed off-line. The 3-DE datasets were analysed with a prototype version of 3DQ-QLab software that allows for semi-automatic detection of endocardial and epicardial borders. Briefly, the 3D volume dataset is first displayed in three different cross-sections that can be modified interactively. For the detection algorithm to work properlycMR loops were analysed off-line with a commercial software (View Forum R4.1). In every end-diastolic short-axis slice, endocardial and epicardial contours were manually traced to calculate LV volumes, mass and EF, while including papillary muscles in the LV cavity.Statistical analysis was performed using SPSS version 11.5. Values are reported as mean (plus or minus 1 SD). The average of duplicate measurements was used for statistical analysis. Linear regression analysis and Bland-Altman plots with assessment of systematic bias and 95% confidence intervals, as well as two-way mixed intraclass correlation coefficient (ICC) were used to compare LV mass between 1-DE, 3-DE and cMR.The characteristics of the study population are summarised in None of the patients were excluded because of poor image quality or non-interpretable 3-DE images. Thirteen patients had excellent (16%), 29 good (35%), 27 moderate (32%) and 14 patients fair (17%) 3-DE image quality. Manual corrections were necessary to optimise endocardial and epicardial contours in all patients for the two imaging methods. With 3-DE, these corrections resulted in only minor changes in the estimated epicardial LV volumes (on average 10 (15) ml from 282 (67) ml before correction to 292 (71) ml after correction). Correction also tended to be larger in patients with normal wall motion (NWM) (17 (10) ml) than in those with WMA (7 (16) ml).Based on the 2-DE, 33 patients had wall motion abnormalities ). Subjects with NWM  served as a controls.Linear regression and Bland-Altman plots of LV mass by 1-DE versus cMR are shown in Mean LV mass values by 3-DE and cMR are reported in 3-DE and cMR yielded similar values for LV mass in patients with (167 (35) g vs 164 (37) g, p = 0.11) and without (137 (43) g vs 138 (47) g, p = 0.77) WMA. In both groups, 3-DE and cMR values were highly correlated (r  =  0.94 each) with only small absolute biases (3 (20) g vs −1 932) g, p = 0.25) and relative biases (5% (8%) vs 9% (14%), p = 0.17) .3-DE measurements of LV volumes and EF were also highly correlated with those obtained by cMR . Yet, volumes were significantly underestimated by 3-DE (167 (68) ml vs 187 (70) ml for EDV, p<0.001; 88 (56) ml vs 101 (65) ml for ESV, p<0.001), resulting in small negative biases  but did not affect the measurement of EF (48% (15%) vs 50% (15%), p = 0.62). As shown in Results of inter-observer and intra-observer reproducibility are summarised in The results of our study indicate that 3-D imaging of the LV with either 3-DE or cMR produces comparable results in terms of LV volumes, EF and mass in patients with or without wall motion abnormalities. Our data also show that 3-DE and cMR provide similarly low inter-observer variability for all LV measurements and well as similarly high test-retest reproducibility.–1521–Over the past 5 years, numerous studies have validated the use of 3-DE for assessment of LV volumes and EF, using various imaging methods as reference standards. All these studies have shown a greater accuracy of 3-DE compared with 1-DE or 2-DE.9–In the present study, we also evaluated the possibility of semi-automatically measuring LV mass using 3-DE. For this purpose, we modified the computerised contour-tracking algorithm used for endocardial border detection to allow for the estimation of the epicardial contours as well. Our data indicate that LV mass measurements obtained using this novel approach are highly accurate, even in patients whose LV cavity and walls are distorted by a previous myocardial infarction. So far, very few studies have examined the ability of 3-DE to measure LV mass. Although their results indicated that measurements of LV mass by 3-DE are feasible and correlate reasonably well with those obtained by cMR, most of these studies only recruited a limited number of patients (around 20 each), few investigated the accuracy of 3-DE in patients with distorted LV wall geometry25Previous studies using 3-DE to measure LV volumes and EF have shown that this technique was more reproducible than 2-DE and as reproducible as cMR.In contrast with the more widely reported parameters of intra-observer and inter-observer variability, which relate to the repeated measurement of a single dataset, test-retest variation involves repetition of the entire acquisition and analysis. Assessment of test-retest variations is important because both physiological and imaging factors contribute to variations in LV function and mass over time. Previous work found significant test-retest variations with respect to both the angulation and displacement of 2-DE imaging planes. These variations partially explain the variability in 2-DE measurements of LV mass that have been highlighted in several studies. The present study demonstrates that test-retest variations in 3-DE and cMR measurements of LV volumes, EF and mass are quite similar. This is consistent with previous studies.First, the 3-D algorithm used to draw the epicardial contours is not yet fully automatic and still requires manual adjustments. Future development of the software should thus aim at tracking the epicardial contours automatically. Second, because of its robustness and reproducibility, we used cMR as the standard to which 3-DE measurements of LV volumes and mass were compared. When doing so, one should not forget that the assessment of LV parameters by cMR has its own limitations, including partial volume effects, the presence of a chemical shift artefact at the muscle-cavity boundary and uncertainties about whether or not to include the most basal slice of the cMR dataset into the measurements of LV volumes. Including or excluding the most basal cMR slice from the measurements can indeed result in a plus or minus 10% variation in estimated volumes.The results of our study indicate that 3-DE, in combination with semi-automated border detection, allows for a robust, accurate and reproducible quantification of LV volumes, EF and mass. The data also show that the robustness, accuracy and reproducibility of 3-DE is equal to that of cMR and similar in patients with and without regional wall motion abnormalities.