History. We report about the course of central sleep apnea (CSA) in 3 patients (70.3 ± 15.2 years) with severe aortic stenosis (AS) . Investigations. Every patient received echocardiography, left/right-heart catheterization, and cardiorespiratory polygraphy before and 6 months after surgical aortic valve replacement (without right-heart catheterization during follow up). Course. Preoperatively all patients demonstrated reduced systolic left ventricular function (EF <55%). They had elevated pulmoraryarterialy pressures and severe CSA. After valve replacement left ventricular function and exercise capacity improved, as well as the severity of CSA. Conclusion. Patients with severe AS can develop CSA, which seems to improve after surgery. Patients with severe AS should be screened for CSA, because CSA might be an additional risk factor and hint that myocardial adaptation is exhausting. Sleep disordered breathing (SDB) has a prognostic impact on cardiac patients . DevelopPatient 1 reported about angina pectoris for 1-2 years (CCS II-III) and progredient dyspnea. Echocardiography revealed severe AS . Left ventricular systolic function was reduced to an ejection fraction of 50%. By heart catheterization an additional two-vessel coronary artery disease (CAD) was diagnosed. Patient 2 suffered from increasing dyspnea (NYHA III). Echocardiography identified severe AS . Left ventricular ejection fraction was reduced to 40%. CAD was excluded by catheterization.Patient 3 reported about moderate dyspnea (NYHA II). Echocardiography demonstrated severe AS . By catheterization a three-vessel CAD was found. Left ventricular ejection fraction was low with 22%. t-tests, in case of non-normality of distribution Wilcoxon-signed rank tests were used. A two-tailed P value < .05 was considered significant.All sleep apnea examinations were done with a mobile device during hospital stay . A standard classification was used for description of SDB . If thorP < .05) (P = .05). After valve replacement left ventricular ejection fraction improved considerably  and was associated with better exercise capacity .Before valve replacement all patients suffered from severe CSA and had a mean AHI of 34 ± 41.5 /h . Mean puP < .05) . PatientRecent studies demonstrated that occurrence and severity of CSA are correlated to the severity of systolic and diastolic left ventricular dysfunction . The occWe report about three patients with severe AS and CSA before surgical valve replacement. Patient 1 had the best systolic left ventricular function and demonstrated the lowest severity of CSA. Before valve replacement all patients had increased mean pulmonary artery and left end diastolic pressure.After valve replacement left ventricular function had been normalized in all three patients. Echocardiography revealed normal end diastolic and end systolic diameters and a decrease of left ventricular hypertrophy in all patients. All patients recovered from CSA postoperatively.The relation between occurrence and severity of CSA to occurrence and severity of pulmonary hypertension must be verified in further studies. CSA was decreased or resolved after improvement in cardiac function. This supports that CSA is another manifestation of cardiac dysfunction. Therefore, asymptomatic patients with moderate to severe AS should be screened for the presence of CSA. Yet it is unclear, if the development of CSA might be an early and sensitive marker to detect myocardial maladaptation in patients with so-far asymptomatic AS. Further controlled studies with pre- and postoperative evaluations are warranted.


Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a heart muscle disease clinically characterized by life-threatening ventricular arrhythmias. Its prevalence has been estimated to vary from 1:2,500 to 1:5,000. ARVC/D is a major cause of sudden death in the young and athletes. The pathology consists of a genetically determined dystrophy of the right ventricular myocardium with fibro-fatty replacement to such an extent that it leads to right ventricular aneurysms. The clinical picture may include: a subclinical phase without symptoms and with ventricular fibrillation being the first presentation; an electrical disorder with palpitations and syncope, due to tachyarrhythmias of right ventricular origin; right ventricular or biventricular pump failure, so severe as to require transplantation. The causative genes encode proteins of mechanical cell junctions  and account for intercalated disk remodeling. Familiar occurrence with an autosomal dominant pattern of inheritance and variable penetrance has been proven. Recessive variants associated with palmoplantar keratoderma and woolly hair have been also reported. Clinical diagnosis may be achieved by demonstrating functional and structural alterations of the right ventricle, depolarization and repolarization abnormalities, arrhythmias with the left bundle branch block morphology and fibro-fatty replacement through endomyocardial biopsy. Two dimensional echo, angiography and magnetic resonance are the imaging tools for visualizing structural-functional abnormalities. Electroanatomic mapping is able to detect areas of low voltage corresponding to myocardial atrophy with fibro-fatty replacement. The main differential diagnoses are idiopathic right ventricular outflow tract tachycardia, myocarditis, dialted cardiomyopathy and sarcoidosis. Only palliative therapy is available and consists of antiarrhythmic drugs, catheter ablation and implantable cardioverter defibrillator. Young age, family history of juvenile sudden death, QRS dispersion ≥ 40 ms, T-wave inversion, left ventricular involvement, ventricular tachycardia, syncope and previous cardiac arrest are the major risk factors for adverse prognosis. Preparticipation screening for sport eligibility has been proven to be effective in detecting asymptomatic patients and sport disqualification has been life-saving, substantially declining sudden death in young athletes. Arrhythmogenic right ventricular cardiomyopathy/dysplasiaARVC/DArrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a unique heart muscle disease, clinically characterized by non-ischemic ventricular arrhythmias originating from the right ventricle (RV), at risk of cardiac arrest. It is one of the major causes of sudden death in the young and in the athletes. The pathology consists of progressive dystrophy of the RV myocardium with fibro-fatty replacement.In the last 25 years, it was possible to identify the disease , to realThe prevalence of approximately 1 in 5,000 people has been estimated . The exaIn the Veneto Region, Italy, the prevalence of the disease has been estimated to vary from 1:2,000 to 1:5,000 [De Motu Cordis et Aneurysmatibus reported a family with disease recurrence in four generations: the affected members presented with palpitations, heart failure, dilation and aneurysms of the RV and sudden death [The disease was first described by Giovanni Maria Lancisi in 1736, who in his book en death .et al. in 1961 reported a patient with "auricularization" of the RV pressure curve, emphasizing the peculiar hemodynamic picture of this non-ischemic heart muscle disease with RV behaving like an atrium [Dalla Volta n atrium . Howevern atrium .et al. in 1982 reported the disease in adults, first emphasizing the origin of arrhythmias from the RV and the histopathological substrate consisting of fibro-fatty replacement of the RV free wall, accounting for epsilon wave and ventricular arrhythmias of RV origin with left bundle branch block (LBBB) morphology [Marcus et al. in 1987–1988 [Familiar occurrence with an autosomal dominant pattern of inheritance and variable penetrance was first proven by Nava 987–1988 ,11.et al. observed an impressive series of sudden deaths in the young (≤ 35 years), with pathology consisting of ARVC/D, mostly occurring during effort, and all characterized by inverted T-waves in the right precordial leads at electrocardiogram (ECG) and apparently benign ventricular arrhythmias of LBBB morphology [In 1988, Thiene  young ≤ 5 years, rphology .The diagnostic imaging was then implemented to visualize the RV, either non-invasively through echocardiogram  or invasSignal averaged ECG proved to be a sensitive tool to detect delay in the electric impulse transmission in the RV myocardium . ImproveARVD1) was found by Rampazzo et al. in 1994 at chromosome 14q23 [et al. in 1996, emphasizing the frequent left ventricular (LV) involvement and an inflammatory component [The first gene locus  classification  and progThe need of an International Registry of the disease was raised  and two Meanwhile, spontaneous occurrence of ARVC/D have been observed in cats  and dogsThe first gene defect was discovered in the recessive variant of the disease (identified since 1985) from the Naxos island and consisting of a cardiocutaneous syndrome  . A deletβ3 genes [Thereafter, other genes encoding cell junction proteins were found defective in the dominant, classical form of ARVC/D: desmoplakin , plakophβ3 genes .The discovery of these gene mutations allowed preliminary genotype-phenotype correlations to be made -37.The implantable cardioverter defibrillator (ICD) represented a major advance in therapy .Finally, electroanatomic mapping proved to be a sensitive tool for identifying areas of fibro-fatty replacement with low amplitude electrical activity .Study of ARVC/D in transgenic mice ,41 modelThe onset occurs usually after childhood, with palpitations and/or syncope.The following clinical pictures of the disease have been observed :1) Subclinical phase with concealed structural abnormalities, during which the affected patient presents no symptoms, and cardiac arrest may be the first and last manifestation of the disease. ARVC/D has been reported as the second cause of sudden death in the young [he young  Figs. 11 Subclinhe young . A subtl2) Overt electrical disorder, with palpitations and syncope. The most typical clinical presentation of the disease is symptomatic ventricular arrhythmias of RV origin, usually triggered by effort. Arrhythmias range from isolated premature ventricular beat to sustained ventricular tachycardia (VT) with LBBB morphology  RV failure. The progressive loss of the RV myocardium may impair the mechanical function of the RV and account for severe pump failure.4) Biventricular failure. When the disease involves the ventricular septum and the LV, congestive heart failure occurs, mimicking dilated cardiomyopathy. Endocavitary mural thrombosis may occur, especially within aneurysms or in the atrial appendages when heart failure is complicated by atrial fibrillation, as to account for thromboembolism. In such conditions, contractile dysfunction may be so severe as to require cardiac transplantation per se" ain vivo in endomyocardial biopsy specimens [The myocardial atrophy is progressive with time and by no way is present at birth, as seen in Uhl's disease, a congenital heart defect in which the RV myocardium failed to develop during embryonic life . Insteadpecimens .More than half of the hearts studied at post-mortem disclosed LV involvement, usually limited to the subepicardium of the postero-lateral free wall . The invHistology of the RV myocardium discloses severe atrophy of the myocardium, replaced by fibro-fatty tissue, which should be regarded as an healing phenomenon following myocyte deaths . FibrousDeath of single or multiple myocytes may be seen at histology, as proof of the acquired nature of myocardial atrophy, and may be associated with inflammatory infiltrates .Myocardial inflammation may be seen in up to 75% of hearts at autopsy, and probably it plays a role in triggering ventricular tachyarrhythmias . Nobody in vivo morphological demonstration of fibro-fatty myocardial replacement [Transvenous endomyocardial biopsy may be of great help for an lacement  Fig. 8)in vivo mlacement . HistomoIn vivo diagnosis may be achieved by demonstrating alterations of the RV function and structure, typical depolarization and repolarization abnormalities, arrhythmias of the LBBB morphology, fibro-fatty replacement of the myocardium and existence of a family history.Diagnostic criteria have been put forward  and diviMorphologic changes accounting for global and/or regional dysfunction are detectable by echocardiography, angiography, cardiac magnetic resonance imaging (MRI), or radionuclide scintigraphy. Major criteria consist of severe dilatation and reduction in systolic function of the RV with no (or only mild) impairment of the LV; localized RV aneurysms (akinesia or diskinetic areas with diastolic bulgings); and severe segmental dilatation of the RV. Minor criteria are mild global RV dilatation and/or reduction in ejection fraction with normal LV, mild segmental dilatation of the RV free wall and regional RV hypokinesia.RV angiography is usually reported as the gold standard for the diagnosis of ARVC/D Fig. . AngiogrEchocardiography is a non-invasive and widely used technique, and represents the first-line imaging approach for evaluating patients with suspected ARVC/D or for screening of family members Fig. . EchocarMRI is an attractive imaging tool because it is non-invasive and has the ability to characterize tissue by distinguishing fat from muscle Fig. . HoweverRadionuclide angiography is also an accurate non-invasive imaging technique for detection of global RV dysfunction and regional wall motion abnormalities. Its diagnostic concordance with RV angiography is nearly 90% .Tissue characterization of the RV free wall with fibro-fatty replacement of the myocardium, as demonstrated on endomyocardial biopsy Fig.  or surgiIn contrast, repolarization abnormalities consisting of inverted T-waves in right precordial leads V2 and V3) in the absence of RBBB, in individuals older than 12 years of age, are considered a minor criterion  or a family history of clinical diagnosis based on the present criteria.A modification of Task Force Criteria for the diagnosis of ARVC/D has been proposed in case of family members for early detection of the disease . In firsNew tools for improving diagnostic accuracy have been introduced in recent years. Among non-invasive investigations, MRI with gadolinium late enhancement has been demonstrated to be able to detect fibrosis in the RV and LV myocardium . Among iOf course, mutational analysis will help to establish with certainty who are the gene carriers, although asymptomatic. However, since these patients may not have the phenotypic expression of the disease, the Task Force criteria are critical to this assessment.et al. to chromosome 14q23 [i.e. desmosomes and fascia adherens, which provides continuous cell-to-cell connection. This explains why genes coding proteins of the intercellular junction became candidate genes. Intercalated discs contain three types of cell-cell connection: gap junction (or nexus), adherens junction, and desmosome. Gap junctions mediate ion transfer between cells and each gap junction channel is a composite of two hemi-channels, or connexons, located within the cytoplasmic membrane of adjacent cells. The connexon, in turn, is formed by an assembly of six connexin subunits, of which connexin 43 (Cx 43) is the principal subtype in the human heart, but also connexins 40 and 45 are expressed at lower levels.ARVC/D is heredo-familial in nearly 50% of cases, thus the ongoing myocardial atrophy may be genetically determined. The classical form is an autosomal dominant disease with variable penetrance ,11. In tme 14q23 . The canme 14q23 ,62. Notevia adheren junctions. The transmembrane component of an adherens junction, which establishes intercellular contact, is a cadherin, i.e. Ca2+-dependent glycoprotein. N-cadherin is the predominant isoform expressed in the human heart. Attached to the cytoplasmic tail of N-cadherin are β-catenin and plakoglobin (γ-catenin), both of which bind to α-catenin, which in turn, interacts directly with actin filaments within the sarcomere.Synchronous contraction requires transmission of force between cells, which is accomplished Finally, desmosomes, together with adheren junctions, provide mechanical attachment between cells. However, in contrast to adheren junctions, desmosomes are not linked to the actin network, but with intermediate filaments, namely desmin in the heart and keratin in the skin. Proteins from three separate families assemble to form desmosomes: the desmosomal cadherins, armadillo proteins, and plakins Fig. .i.e. plakoglobin and plakophilin. Noteworthy, plakoglobin is also found in adhering junctions together with its homologue β-catenin. β-catenin, conversely, is not a constituent of desmosomes as it binds specifically to the classical cadherins. However, β-catenin has an additional nonadhesive function as a regulator of transcription, and a similar role has been postulated for plakoglobin. The plakophilins are found in the nucleus as well as the desmosome, although their function therein remains speculative. Binding sites for both plakoglobin and plakophilin are situated in the N-terminal domain of desmoplakin. At its C-terminal, desmoplakin anchors desmin intermediate filaments to the cardiomocyte surface [The genes encoding the desmosomal cadherins are clustered on chromosome 18q12.1 and four desmogleins (DSG1-4) and three desmocollins (DSC1-3) are recognized. The desmosomal cadherins comprise the transmembrane component of the desmosomal complex. Their extracellular domains interface directly with their counterparts on neighboring cells. Besides their role in cell adhesion, the desmosomal cadherins may function as regulators of morphogenesis. The intracellular portions of the desmosomal cadherins interact with proteins of the armadillo family,  surface .A deletion in plakoglobin was first found in Naxos disease in 2000 , followeUltrastructural investigation in endomyocardial biopsy of patients with ARVC/D and cell junction gene mutations revealed intercalated disk remodeling with a decrease in the number and length of desmosomes and intercellular gap widening  Fig. 1414.Cell junction protein mutations may account for a final common pathway, namely disruption of intercellular junction, myocyte death and structural changes, which are the substrate of life-threatening ventricular arrhythmias .i.e. Carvajal disease [A recessive mutation of desmoplakin has been proven to explain another cardiocutaneous syndrome,  disease , charact disease , with diMoreover, remodeling of intercalated disc may lead to widening of myocyte gap junction, which may also contribute to the arrhythmogenicity of the disease and enhance the risk of sudden death .Two other gene defects have been reported to explain the disease so far. One is the gene encoding for the cardiac ryanodine receptor 2, which is located in the smooth sarcoplasmic reticulum and mediates calcium release for electroanatomical coupling (ARVD2 with polymorphic ventricular arrhythmias) . Similarβ gene [Another form of ARVC/D was found to be associated with regulatory mutations in the TGFβ gene . The genWith gene mutations available, transgenic mice are now being developed to gain an insight into the etiopathogenetic mechanisms of the disease, with possible therapeutic implications ,41.Young age, "malignant" family history, QRS dispersion ≥ 40 ms, T-wave inversion beyond V1, LV involvement, VT, syncope or previous cardiac arrest are considered the major determinants for adverse prognosis and impending sudden death .β-blockers, sotalol, amiodarone, verapamil alone or combinations. Wichter et al. reported the various efficacy rates by demonstrating that sotalol is superior with a complete or partial efficacy in 68% of patients vs 26% for amiodarone [Different antiarrhythmic drugs have been employed: sodium blockers, Catheter ablation has been accomplished in VT refractory to drug treatment . Althouget al. [ICD has been proven to be life-saving. Corrado et al.  found a In refractory congestive heart failure, cardiac transplantation is the only therapeutic option .An algorithm for management of ARVC/D has been proposed Fig. . In sympβ-blockers are recommended with follow-up control. If the form of ARVC/D is severe, electrophysiology-intracavitary testing is recommended. If negative, β-blockers and serial follow-up should be undertaken. If positive, ICD should be considered, as well as β-blockers and other antiarrhythmic drugs.In asymptomatic patients without a family history and a mild form of ARVC/D, In the absence of symptoms and a family history, it is controversial whether electrophysiologic testing should be carried out even in patients at low risk. Finally, it should be underlined that, at present, no curative therapy has been postulated and clearly the aforementioned treatments are palliative. Gene therapy is still far from being established  and no tCardiac arrest in ARVC/D is the consequence of a combination of various factors  and measures for prevention should focus on these various steps Fig. .etc. The availability of this tool even at home for families at risk, should be considered, provided it is accompanied by a life-support training.ICD aims to convert ventricular flutter/fibrillation into sinus rhythm for resuscitation from cardiac arrest. The device may be implanted in selected patients at risk or may be external, used on the spot in case of sudden cardiac arrest occurring in public sites, like sports courts, airports, schools, Drug therapy and ablation plays a fundamental role in the arrhythmic mechanism to prevent onset of life-threatening arrhythmias. The efficacy is, however, limited and the recurrence of arrhythmias quite high.Effort, by volume overload and stretching of the RV myocardium, is considered a major trigger. Sport activity increases 5 fold the risk of sudden death in the young  Fig. 2020. Thus,per se", regardless of the need of antiarrhythmic/ablation therapy or ICD. These are palliative, empiric treatments.A different life style may be safe "Curative therapy of the disease, the radical form of prevention of sudden death, may be accomplished in various ways:a) Heart replacement, in case of refractory congestive heart failure and/or arrhythmias, with cardiac transplantation.b) Some therapy to prevent myocyte death and inflammation, to block onset and progression of the disease at the pathobiological level. Nothing is available so far and transgenic animal models are ideal to investigate the pathogenesis of the diseases and to figure out curative therapies ,41.c) Repair of the defective genes at somatic level (gene therapy), a controversial and yet inconclusive approach.d) Genetic counseling and birth control.It must be underscored that the phenotypic expression of the gene defect, with the exception of cardiocutaneous syndromes, is only at the cardiac level and nowadays a series of effective measures are available to ensure normal life, with very low risk of premature death in affected patients.


Knowledge about age-specific normal values for left ventricular mass (LVM), end-diastolic volume (EDV), end-systolic volume (ESV), stroke volume (SV) and ejection fraction (EF) by cardiac magnetic resonance imaging (CMR) is of importance to differentiate between health and disease and to assess the severity of disease. The aims of the study were to determine age and gender specific normal reference values and to explore the normal physiological variation of these parameters from adolescence to late adulthood, in a cross sectional study.Gradient echo CMR was performed at 1.5 T in 96 healthy volunteers . Gender-specific analysis of parameters was undertaken in both absolute values and adjusted for body surface area (BSA).Age and gender specific normal ranges for LV volumes, mass and function are presented from the second through the eighth decade of life. LVM, ESV and EDV rose during adolescence and declined in adulthood. SV and EF decreased with age. Compared to adult females, adult males had higher BSA-adjusted values of EDV (p = 0.006) and ESV (p < 0.001), similar SV (p = 0.51) and lower EF (p = 0.014). No gender differences were seen in the youngest, 11–15 year, age range.LV volumes, mass and function vary over a broad age range in healthy individuals. LV volumes and mass both rise in adolescence and decline with age. EF showed a rapid decline in adolescence compared to changes throughout adulthood. These findings demonstrate the need for age and gender specific normal ranges for clinical use. Fundamental structural and functional properties of the left ventricle including left ventricular mass (LVM), volumes, and function are often assessed in the clinical setting using two dimensional echocardiography. The clinical use of cardiac magnetic resonance imaging (CMR) has increased lately and a consensus panel report has established clinical indications for cardiovascular magnetic resonance .It is of great clinical importance to be able to differentiate between normal and abnormal findings, but this may often prove difficult if adequate normal values are unknown. The need for age and gender specific normal values with CMR is therefore growing. Several studies have defined CMR normal ranges of LV volumes and function in limited age ranges -8, and nThe aim of the study was, therefore, to suggest clinically usable age and gender specific normal ranges for LV volumes and function using gradient echo CMR at 1.5 T for the second through the eighth decade of life. We also sought to explore, in a cross sectional study, the normal variation of LV volumes and function in strictly healthy subjects over a wide age range in order to examine the age variation of these parameters.The study population consisted of 96 healthy volunteers prospectively recruited by advertisement from the local community . No subjects were excluded because of poor image quality. All subjects had a normal electrocardiogram (ECG) and blood pressure (systolic blood pressure (SBP) ≤ 140 mmHg and diastolic blood pressure (DBP) ≤ 90 mmHg)  and had SBP and DBP were obtained in the supine or seated position by auscultation using a brachial cuff.All patients were imaged in the supine position using a 1.5 T system  with a 25 mT/m gradient system and a phased-array body coil. Standard scout images were used to locate the orthogonal planes of the heart. End-expiratory ECG triggered short-axis gradient echo cine loops were then acquired throughout the left ventricle from the base  to the apex. Typical imaging parameters were TR = 100 ms, and echo sharing gave an effective phase interval of 50 ms, TE = 4.8 ms, slice thickness 10 mm, field of view 350–420 mm, matrix 126 × 256, flip angle 20°. The number of cardiac phases per acquisition was determined as the integer obtained from the RR interval divided by TR. Nine to twelve slices were required to completely cover the left ventricle, depending on heart size.All measurements were undertaken manually without the aid of automated image analysis software. End-diastolic and end-systolic frames were identified according to ventricular blood pool area. Measurements of the left ventricular endocardial and epicardial areas  in each image frame were performed in the short-axis view. At the base of the left ventricle, the aortic outflow tract below the valve was included in volume measurements. The free papillary muscles were included for LVM assessment, and excluded for left ventricular volume assessment . In the 3). End-diastolic volume (EDV) and end-systolic volume (ESV) were calculated as the endocardial volume at end-diastole and end-systole respectively. Stroke volume (SV) was calculated as the difference between EDV and ESV. Ejection fraction (EF) was calculated as SV divided by EDV. All parameters were divided by body surface area (BSA) to achieve values adjusted for body size (e.g. LVM/BSA for left ventricular mass adjusted for BSA). In addition, a subset of 20 subjects were analyzed by 2 readers blinded to each others results in order to examine interobserver variability. Intraobserver variability was assessed by one observer who performed repeated blinded measurements in the same 20 subjects one month later.The difference in area between the endocardial and epicardial contours multiplied by the slice thickness (10 mm) represented myocardial volume for a given slice. Total myocardial mass was obtained by calculating the sum of all myocardial slice volumes and multiplying by the myocardial specific gravity  employing a two-way mixed model [2). BSA was calculated using a previously described technique [2 and lowest root mean square (RMS) of the error [SPSS (version 16) was used for statistical calculations. A ed model . The coeechnique . Curve ehe error . The preData from 94 of the 96 subjects have, in part, previously been published in a study of LVM and wall stress . Table 1Table Information on the age dependence of LV mass and wall stress in the majority of this population have been published and discussed previously . The cur2 vs. 77 ± 11 ml/m2, p = 0.95; ESV: 35 ± 8 ml vs. 32 ± 11 ml, p = 0.48; ESV/BSA: 22 ± 4 ml/m2 vs. 21 ± 7 ml/m2, p = 0.65). However, differences between genders became apparent for all ventricular volumes in the adult group with higher values in the male group .Ventricular volumes varied markedly between males and females Figure . Notably2 vs. 44 ± 9 ml/m2, p = 0.51; EF: 0.59 ± 0.07 vs. 0.64 ± 0.08, p = 0.014), SV and EF were similar between genders in the adolescent group . Notably, left ventricular EF in the adolescent group was higher  except for EDV/BSA (p = 0.13) and ESV/BSA (p = 0.06) in males, and EDV (p = 0.39), EDV/BSA (p = 0.15), ESV/BSA (p = 0.28), SV (p = 0.14) and LVM/BSA (p = 0.24) in females.Data on intra- and interobserver variability are presented in Table This study suggests age and gender specific normal values for LV mass, volumes and function measured in a healthy population over a large age range using gradient echo CMR at 1.5 T.One of the most important tasks in patient examination is to distinguish normal findings from those indicative of disease. Normal ranges for a given parameter encompassing 95% of the population constitute the mainstay of this procedure. With increasing use of cardiac magnetic resonance imaging, the need for age-specific ranges of LV mass, volumes and function for this modality is obvious. This is of specific importance in order to correctly exclude disease.The findings regarding left ventricular mass in the present study and its relation to earlier studies has been discussed earlier . Other net al used a gradient echo sequence and reported as much as 20–30% lower values for LV mass and volumes. Those authors chose to only measure LV volumes and mass in short axis slices which contained more than 50% of the circumference of the LV wall in both end-diastole and end-systole. This approach does not take into account the long axis movement of the basal parts of the left ventricle [et al, showed differences between subjects above and below the age of 35 [et al [Six CMR studies -5,7-9 haentricle , which ige of 35 , but conThe inotropy of the left ventricle has been shown to be related to growth hormone/IGF levels . AlthougThe reproducibility of CMR LV measures obtained in our study was consistent with previously reported studies of intra- and interobserver variability -21.Regurgitation in the aortic or mitral valve was only assessed visually in long axis CMR images, and this is a limitation. However, it is likely that this was sufficient to serve the purposes of this study in this population with otherwise unremarkable ECG, medical history and physical examination. The number of included subjects in each gender and decade is less than the minimally suggested n = 10  for the This study suggests normal reference values for left ventricular mass, dimensions, and function in healthy humans from early adolescence to the eighth decade according to gender using gradient echo CMR at 1.5 T. LV volumes, mass and function vary over a broad age range in healthy individuals. LV volumes and mass both rise in adolescence and decline with age. EF showed a rapid decline in adolescence compared to changes throughout adulthood. These findings further demonstrate the need for age and gender specific normal ranges for clinical cardiac MR examinations.(BSA): body surface area; (CMR): cardiac magnetic resonance; (DBP):diastolic blood pressure; (ECG): electrocardiogram; (EDV):end-diastolic volume; (EF): ejection fraction; (ESV): end-systolic volume; (ICC): intraclass correlation coefficient; (LV): left ventricle; (LVM): left ventricular mass; (SBP): systolic blood pressure; (SV): stroke volume; (T): Tesla; (TE): time to echo; (TR): time to repetition.The authors declare that they have no competing interests.PC performed data acquisition, data analysis, statistical analysis and drafted the manuscript. RA and AAJ performed data acquisition and data analysis, and participated in critically revising the manuscript for important intellectual content. EH performed data acquisition, data analysis, statistical analysis and helped to draft the manuscript. MU performed data acquisition, data analysis, statistical analysis and helped to draft the manuscript. PF conceived of the study, participated in its design and coordination, and participated in critically revising the manuscript for important intellectual content. HA conceived of the study, participated in its design and coordination, and helped to draft the manuscript. All authors' read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2342/9/2/prepub


Isolated ventricular non-compaction cardiomyopathy (IVNC) is a rare, morphologically distinct primary genetic cardiomyopathy, which is now gaining prominence as an important differential diagnosis in patients presenting with cardiac failure. We describe a case report of a Nigerian male with facial dysmorphism presenting with cardiac failure. This is followed by a review of the literature with focus on the diagnosis of this condition, which may be difficult especially in non-Caucasian populations. A 42-year-old Nigerian man presented with a three-month history of worsening dyspnoea, orthopnoea, and paroxysmal nocturnal dyspnoea. He had no associated chest pain. He had no significant past medical history, was a nonsmoker, and drank minimal alcohol. He had been living in Scotland for several years and there was no recent history of foreign travel. There was no family history of cardiomyopathy or sudden death. On examination he was afebrile with evidence of mild facial dysmorphism . He had He had a normal full blood count, urea, creatinine and electrolytes, liver function and thyroid function tests. He was HIV negative and his antinuclear factor was negative. His electrocardiogram showed a sinus tachycardia with voltage criteria for left ventricular hypertrophy. Troponin and CK levels were normal. His chest X-ray showed cardiomegaly with upper-lobe diversion. Echocardiographic images were suboptimal in quality but showed a dilated left ventricle, and severe global impairment of LV systolic function. Cardiac MRI (CMR) revealed similar findings but in addition demonstrated deep ventricular recesses and hypertrabeculation of the lateral wall extending down towards the apex . UnusualIn combination with his facial dysmorphism, these features were felt to be consistent with a diagnosis of LV noncompaction. Given the lack of anginal symptoms or risk factors, it was felt that coronary angiography was not necessary. Our patient did not describe any neurological symptoms. Furthermore complete examination of the nervous system and assessment of visual acuity were normal; therefore additional neurological investigations were not deemed necessary. Our patient was commenced on an ACE inhibitor, diuretic, beta blocker, and long-term anticoagulation. We advised him to contact his relatives back in Nigeria in order to arrange screening investigations. On followup, he has remained asymptomatic and has not required any further hospitalization. Holter monitoring has proved reassuring and repeat echocardiography suggested improvement in function, with the ejection fraction calculated at 45%. ICD insertion has therefore not been recommended at this time.Isolated ventricular non-compaction cardiomyopathy (IVNC) is a rare, morphologically distinct primary genetic cardiomyopathy, which was first described almost two decades ago. It is now gaining prominence as an important differential diagnosis in patients presenting with cardiac failure. It is characterized anatomically by prominent trabeculations in the ventricular wall and deep intertrabecular recesses which communicate with the main ventricular chamber . This reIVNC appears to be a genetically inherited condition that has been associated with many different gene mutations , 3. BothThe exact cause of IVNC, however, remains controversial. The congenital theory suggests that it is due to failure in the compaction of the myocardium, which usually occurs between weeks five and eight of embryonic life . This prNonisolated forms of non-compaction also exist which are associated with other forms of congenital heart disease including atrial septal defects, ventricular septal defects, aortic stenosis, pulmonary stenosis, tricuspid stenosis, and hypoplastic left ventricle. The aetiology of non-compaction in non-isolated forms may be different to IVNC . Since the very early reports of non-compaction, an association has been noted with different forms of facial dysmorphism, particularly when the diagnosis is made in children , 8–10. IDyspnoea is the most common symptom alongside other manifestations of cardiac failure. Cardiac arrhythmias are also frequent and may be life threatening. The incidence of thromboembolic events is particularly high. It is postulated that they are the result of thrombus formation within the deep intra-trabecular recesses. However these events are often observed in the context of other prothrombotic factors such as atrial fibrillation and impaired systolic function . In addiOtherwise management of IVNC focuses on conventional treatment of clinical manifestations. Patients with decreased ventricular systolic function should be treated with ACE inhibitors and beta blockers , 13. At Prognosis is variable; early studies suggested that it was poor  but moreGiven the significant morbidity and mortality associated with IVNC, prompt and accurate recognition by cardiac imaging techniques is essential. Until recently isolated IVNC was thought to be extremely rare with a prevalence in adults of less than 0.3% . HoweverEchocardiography is the first-line investigation for patients presenting with cardiac failure. Echocardiographic criteria for the diagnosis of IVNC have been established , with thCardiac MRI (CMR) is an alternative imaging technique, which offers superior contrast between the myocardium and the blood pool. Diagnosis of IVNC can be made using CMR and demonstration of a non-compacted:compacted ratio >2.3, in end diastole, on long-axis views, with a sensitivity of 86% and a specificity of 99% . In thisCMR studies have also led to the realization that a lesser degree of non-compaction may also be observed in normal ventricles . This isHypertrabeculation has also been reported in the context of other forms of cardiomyopathy, most commonly in hypertrophic cardiomyopathy and dilated cardiomyopathy. Interesting case reports have described families, in whom the same gene mutation has caused a dilated cardiomyopathy in some members and IVNC in others . It may Assessment of non-compaction is particularly difficult in black patients who display a greater degree of trabeculation of the ventricle than other groups. In one study 13% of healthy black control subjects fulfilled echo criteria for IVNC despite having no other features suggestive of a cardiomyopathy . There is in fact growing evidence that the prevalence of IVNC differs in different ethnic groups. An Australian study compared rates of cardiomyopathy between indigenous and nonindigenous children and showed a much higher incidence of IVNC in the indigenous population , whilst It is unclear whether these observations reflect a truly higher incidence of IVNC in these ethnic groups or are due to oversensitivity of the diagnostic criteria, which have been established in a predominantly Caucasian population.Our patient was a black African male, which made application of existing diagnostic criteria difficult. However, the degree of hypertrabeculation in combination with the established systolic impairment and facial dysmorphism was in our opinion sufficient to make a secure diagnosis of IVNC. It should be noted that it is unusual for the true apex not to be involved. However this has been described: Thuny et al. observed sparing of the true apex by CMR in 1 of the 16 patients studied. All 16 patients, however, had involvement of the lateral apical segment, which was the most clearly affected segment in our patient .In summary, the diagnosis of IVNC is currently made challenging because of the heterogeneity in the degree of trabeculation observed in patients with normal ventricles and other forms of cardiomyopathy. Current diagnostic criteria are based upon studies involving only a small number of patients and there are concerns that they are leading to the overdiagnosis of this condition. This is of particular concern in black patients and other racial groups. There is therefore an urgent need to establish more stringent criteria. However, before this is possible, large detailed studies are required to establish the normal range of trabeculation amongst healthy subjects of different ages and ethnicities. Only then can we interpret studies examining trabeculation amongst patients with different forms of cardiomyopathy.


Apical ballooning is a cardiac syndrome (Takotsubo Cardiomyopathy) described as a typical form of acute transient left ventricular dysfunction. While its onset has often been associated with emotionally or physically stressful situations, it has an overall favorable prognosis. We describe here a case of transient apical ballooning following permanent pacemaker implantation. Apical ballooning syndrome (ABS) is a novel cardiac syndrome described recently as a typical form of acute transient left ventricular (LV) dysfunction associated with chest pain, ECG changes, and mild enzyme elevation without coronary occlusion . While iAn 86 year old female, with no significant past medical history or cardiac symptoms, with the exception of a successful ablation of AVNRT 12 years ago, was admitted with recurrent syncope. She underwent a detailed evaluation, including an ECG and echocardiography, both of which were normal, with an estimated left ventricle ejection fraction (LVEF) of 60%. She was diagnosed by bedside monitoring to have intermittent complete AV block with ventricular escape rhythm of approximately 30 bpm.She underwent implantation of a dual chamber pacemaker. The procedure was smooth with no apparent complications. On the night following implantation she started complaining of dyspnea and orthopnea which resolved spontaneously. She was discharged the following morning after pacemaker evaluation and chest X-ray, both of which were normal. Several hours later, however, she developed acute pulmonary edema. Emergency room ECG showed atrial synchronous ventricular pacing of approximately 80bpm, prolongation of the QTc interval up to 540 ms and non-specific ST-T changes. Echocardiography revealed normal left ventricle size, normal basal segment contraction with severe global left ventricular dysfunction and an estimated LVEF of 20%. The echocardiographic picture was typical of apical ballooning . TroponiOver the next several days there was a dramatic improvement in her clinical condition, and a gradual recovery of LVEF on repeated echocardiograms. Repeated echocardiography after five days demonstrated an LVEF of 45%. She was discharged in stable condition and follow-up echocardiography demonstrated complete recovery of left ventricular function.Apical ballooning syndrome was first described in Japan in early 1990s , where iPacemaker implantation could be associated with a number of acute complications related to the procedure, the patient and/or the device itself. However, we are aware of only three previous cases of ABS reported in the literature following pacemaker implantation ,13. In o


Diagnostic and prognostic power of myocardial perfusion imaging in patients with coronary artery disease has been demonstrated with planar imaging which was further improvised with addition of gated SPECT and newer Technetium labeled myocardial perfusion tracers like SestaMIBI, Tetrofosmin. Myocardial perfusion abnormalities at rest and after stress are considered to be the best predictors of cardiac event–free survival in adults with ischemic heart disease. This article highlights various myocardial perfusion imaging (MPI) radiopharmaceuticals, exercise procedures, pharmacological stress protocols, indications for MPI and myocardial perfusion patterns in children with some of the common congenital and acquired heart diseases. However, not many centers practice pediatric nuclear cardiology because of less referrals, difficulties in stressing children, and inadequate exposure to image interpretation. Myocardial perfusion imaging (MPI) in children is an important modality for evaluation of myocardial ischemia and viability. Patients with Kawasaki disease (KD), anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), coronary ectasia, and complete transposition of the great arteries (TGA) after arterial switch operation (ASO) can be easily evaluated non-invasively by MPI. In addition, it is also useful in patients with primary or secondary cardiomyopathies to assess the extent of myocardial damage. Children presenting with right ventricular (RV) pressure and/or volume overload can also be assessed by MPI. The RV free wall is normally not visualized on MPI when the patient is at rest. Only when there is an underlying pressure overload affecting RV myocardium does the single photon emission computed tomography (SPECT) imaging reflect positively. Uptake of myocardial perfusion tracers like SestaMIBI (Methoxy Isobutyl Isonitrile) MIBI/Tl  in septum and RV free wall is directly proportional to pressure overload. An inverted D sign is charaet al. have useWith the growth of specialized pediatric cardiology centers and surgical techniques, there is a need for greater awareness of newer imaging procedures such as MPI in the diagnosis and prognostication of various pediatric cardiac problems. MPI is a useful physiological imaging modality in evaluation of myocardial viability and ischemia in children with congenital and acquired cardiac diseases that have associated coronary lesions.201Thallium (201Tl) and Technetium (99mTc) based radiopharmaceuticals such as SestaMIBI and Tetrofosmin. 201Tl, a potassium analogue, is used extensively in identification of myocardial viability, hibernation, and ischemia. Myocardial uptake of thallium is proportional to regional blood flow and depends on the presence of an active sodium potassium ATPase pump in the myocyte to facilitate its transport intracellularly.[Radiotracers used for myocardial perfusion SPECT imaging are llularly.99mTc-labeled tracers are found to be superior to 201Tl with regard to imaging physics, radiation safety, availability, and cost effectiveness. Mechanism of myocardial uptake for Technetium-labeled agents is by passive diffusion and is therefore proportional to regional blood flow. Unlike 201Tl, they do not have the property of redistribution, hence reinjection is necessary for ECG gated rest imaging.[However,  imaging.99mTc-labeled tracers in children is their significant hepatic accumulation due to slow liver tracer clearance [Technically, attenuation effects on LV tracer activity are almost negligible in pediatric patients . The main disadvantage of using learance . Vasodil99mTc-labelled agents by the genitals in children is from 0.1-10% less than that of 201Tl.[The minimum dosage of tracer to be administered intravenously in children is not well established so far. However,of 201Tl.To evaluate myocardial ischemia, MPI is often conducted along with physical exercise or with pharmacologic agents both augmenting regional flow heterogeneity. Every pharmacological agent has a unique effect on the coronary vasculature and influences the distribution of the radiolabeled perfusion agent. Children and their parents must be counseled prior to undertaking any form of stress procedures for maximum motivation.Although exercise testing is more physiological, it is technically difficult to stress children on a treadmill or bicycle ergometer as they are basically designed for adults. An ergometer is preferable for exercising children because it is difficult to quantify the amount of work done on a treadmill apart from its high cost, noise production, and large size. Patients do not need to be ambulatory for ergometer testing. It is also difficult to obtain accurate blood pressure estimation while the child is on the treadmill because of the noise and motion artifacts. Disadvantages of ergometer exercise are that it needs more coordination to perform the test correctly and it is difficult to achieve maximum physical exertion unless the patient is pretrained on the bicycle. However, a treadmill is useful in children over 3 years where fast walking or running needs no special motivation or training.The laboratory should be equipped with age and size-appropriate ergometers, emergency equipment, pediatric cuffs for the measurement of blood pressure, mouthpieces, and facemasks. It may be difficult to attain the maximum predicted heart rate in children, which is required to highlight abnormal coronary flow reserve. Serial evaluation might prove difficult in children, since exercise capacity significantly changes with physical growth during childhood. Symptom limited exercise protocols or modified pediatric protocols are followed with the aim of attaining the maximum possible heart rate. To increase workload, it is preferred to increase the grade of exercise rather than increasing the speed of the instrument.Pharmacological stress is preferred in children for assessing the presence of coronary stenosis or serial evaluation of the same child while observing the progression of coronary stenosis. The incidence of significant adverse reaction to pharmacological stress in the pediatric population is unknown. In contrast, exercise stress might be more suitable and reflects the physiological changes while evaluating older children with exercise-related abnormalities (positive ischemic changes on exercise ECG), documented coronary arterial stenosis, or those under anti-ischemic medication and post coronary artery bypass graft (CABG) surgery patients. CABG in children is commonly done for ischemic complications of KD, congenital left main coronary ostial stenosis, iatrogenic coronary cameral fistula, ALCAPA, and single coronary artery traversing between the great arteries in post cardiac transplantation settings. Asymptom8Similarly asymptomatic patients post ALCAPA repair and ASO are also at low risk for inducing myocardial ischemia at a good workload. High-risk patients for provoking ischemia are those with pulmonary hypertension, dilated or restrictive cardiomyopathy in cardiac failure, and those with exercise- induced malignant arrthymias.As per ACC/AHA/ESC 2006 guidelines (American College of Cardiology/American Heart Association/European Society of Cardiology) for manaSimilarly, patients with ventricular tachycardia (VT) and LV dysfunction due to prior myocardial infarction, cardiomypathy, bundle-branch reentry, and various forms of idiopathic VT planned for radiofrequency ablation also need to have documentation of ischemia with stress testing under strict supervision.Endpoints for treadmill or ergometer exercise in children as part of MPI imaging as per the AHA guidelines are as follows:Decrease in ventricular rate with increasing workload associated with extreme fatigue, dizziness, or other symptoms suggestive of insufficient cardiac outputFailure of heart rate to increase with exercise, and extreme fatigue, dizziness, or other symptoms suggestive of insufficient cardiac outputProgressive fall in systolic blood pressure with increasing workloadSevere hypertension, >250 mmHg systolic or 125 mmHg diastolic, or blood pressure higher than can be measured by the laboratory equipmentDyspnea that the patient finds intolerableSymptomatic tachycardia that the patient finds intolerableA progressive fall in oxygen saturation to <90% or a 10-point drop from resting saturation in a patient who is symptomaticPresence of ≥3 mm flat or downward sloping ST-segment depressionIncreasing ventricular ectopy with increasing workload, including a >3-beat runPatient requests termination of the studyConsequently, MPI stress methods in children should be individualized and adjusted according to the clinical purpose and basal conditions.Stressor agents and pharmacological stress procedure are the same as for adults. Pharmacologic stress agents fall into two categories: coronary vasodilators  and cardiac inotropic agents (e.g. dobutamine).Usually, vasodilators are preferred for MPI. Vasodilators work directly on the coronary vessels and increase myocardial blood flow three to five times above the resting level, whereas inotropic agents work indirectly by increasing myocardial workload leading to an increase in coronary blood flow. Diagnostic accuracies for adenosine are similar to those of dipyridamole.2 receptors in the vessels. It increases mean blood flow four to five times the baseline mean value. Dipyridamole inhibits the action of adenosine deaminase enzyme thereby inhibiting degradation of endogenously produced adenosine. It also blocks cellular reuptake of adenosine elevating endogenous adenosine causing vasodilation. Dipyridamole increases mean blood flow approximately three to four times of the baseline value.Adenosine promotes vasodilatation by activation of the ADobutamine is a synthetic catecholamine with a plasma half-life of 2 minutes that acts through the alpha-1 and beta-2 adrenoreceptors. Dobutamine mimics exercise physiology because it significantly increases the heart rate and blood pressure at doses greater than 20 μg/kg per minute. It artificially increases the cardiac output and myocardial oxygen consumption and therefore does not result in normal metabolic response to exercise.Adenosine is infused at a dose of 140 μg/kg body weight per minute by an infusion pump for 4 to 6 minutes . Abbreviated adenosine (3 to 4 minutes) protocols are also available. SimilarlDobutamine is administered intravenously in gradations with a starting dose of 10 to a maximal dose of 50 μg/kg body weight per minute in 3- to 5-minute stages. Atropine  is also used to enhance the heart rate, if required. In children, a dose of dobutamine of 50 μg/kg per minute is usually necessary to achieve the target heart rate. Atropine is needed in approximately two-thirds of patients if the target heart rate is not achieved by dobutamine alone. Esmolol (10 mg/mL dilution) at a dose of 0.5 mg/kg is the antidote used to reverse the side effect or development of ischemia during dobutamine infusion.14Children with bronchial asthma and heart blocks are contraindicated for vasodilator stress MPI (i.e. adenosine and dipyridamole stress).Symptomatic infants and young children with severe congenital abnormalities of the left main coronary artery, ALCAPA, and ostial atresia of the left coronary artery are also not normally stressed.Motionless acquisition remains the primary concern during a myocardial perfusion SPECT imaging. This can be achieved with intravenous midazolam  or pentobarbital in children younger than 5 years of age. Other conventional patient preparatory orders such as securing a patient IV line, 4 hours fasting prior to the test, and withholding beta-blockers and vasodilators remain the same as for adults.SPECT images of myocardium are generally acquired using a 180-degree rotation from the right anterior oblique to the left posterior oblique projection. A dual-head variable angle gamma camera is ideal for dedicated nuclear cardiology centers. After reconstruction of the transaxial plane, the long axis of the heart is defined and for visual analysis, images are displayed in serial slices at 90-degree angles relative to the long axis of the heart. This allows for an optimal display of the ventricular chambers and perfusion abnormalities to specific coronary arteries can be identified. Many types of softwares have been developed for the quantitative analysis to assist in interpretation.Kawasaki diseaseTetralogy of Fallot repairALCAPAPost arterial switch operationCardiomyopathies - hypertrophic cardiomyopathy, cardiomyopathy in Duchenne progressive muscular dystrophyCardiac transplantOthers: Estimation of right ventricular pressure overload, morphological RV as systemic ventricleIt is imperative to know that the normal distribution pattern of myocardial perfusion tracers in the LV is substantially different between children and adults. In children, the anterolateral segment of LV shows the least tracer uptake. There caMPI has mainly been utilized and studied extensively in pediatric cardiological practice for detecting myocardial ischemia in children with KD.14 Sudden13Stress MPI is useful in children with KD to study the appearance or aggravation of myocardial ischemia and assess the success of coronary revascularisation. Either a pharmacological or exercise MPI can detect angiographically proven coronary stenosis with 70-90% sensitivity.15 It can14The presence of reversible perfusion defect on dipyridamole-stress MPI is a powerful predictor of cardiac events during the chronic stage of KD Figure  and 5b.In tetralogy of Fallot, corrective surgery is usually performed above six months of age. Correction involves closure of the ventricular septal defect with a patch and relieving the pulmonary stenosis. An increasing number of patients have residual abnormalities of ventricular function and cardiovascular performance after right ventricular outflow tract (RVOT) repair. A myocardial perfusion SPECT may be useful in identifying residual or inducible ischemia, regional ejection fraction estimation, wall motion abnormality, and residual hypertrophy .With the recent availability of MDCT, conditions such as ALCAPA can be best diagnosed using MDCT. Practical difficulties of breath holding in newborns, infants, and children can be overcome with MPI where it can be easily performed with less radiation and better compliance.Patients with ALCAPA usually show severe perfusion defects at rest. Caution must be exerted to avoid inducing myocardial ischemia unnecessarily in these patients, which may be of less clinical relevance and may cause life threatening sudden cardiac events. Therefore, pre-surgical myocardial viability determined by a rest MPI might be adequate to determine if a patient will benefit from any revascularization procedure. Patients showing 50% and more MIBI or Thallium uptake in a LV segment is said to be viable.Stress MPI is also useful in treated or fairly asymptomatic older children and adults with ALCAPA as a follow-up to assess any fresh inducible myocardial ischemia . Such paet al.[For infants with ALCAPA syndrome, because the left main coronary artery territory is perfused retrogradely by collateral circulation from the right coronary artery, extensive perfusion abnormalities are more frequent at apical and inferior walls. Once reimplantation of the left coronary artery into the aorta is performed, studies indicate slow but significant improvement in myocardial dysfunction within the next year. Scintigret al. found a MPI is also useful in differentiating ALCAPA from congestive cardiomyopathy in infants. Patients with little collateralization become symptomatic early in life with cardiac failure due to ischemic heart lesions. Scintigraphic findings are characteristic in such patients with ALCAPA and are localized to high lateral, anterior, and lateral segments18 while Surgical management of D-TGA has evolved over several decades.‐22 Defin24The ASO is the current surgical procedure of choice in newborns and infants with transposition of arteries and involves mobilization and reimplantation of coronary arteries into the neo-aorta. Thus, thet al.[Several investigators have used MPI to study any variations in myocardial perfusion in these children after surgery. As in KDet al. also fouRecent evidence shows that in all new cases of dilated or hypertrophic cardiomyopathy, PET or scintigraphy should be performed to exclude coronary artery anomalies in children.‐31 Compret al.[Children diagnosed with hypertrophic cardiomyopathy (HCM) are at a higher risk of sudden death, which is usually due to inducible myocardial ischemia rather than ventricular arrhythmias. Theodoro, et al. from theIn HCM, there can be reversible perfusion abnormalities in anterior, septal, apical regions, or subendocardial ischemia shown as transient stress induced dilatation of the left ventricular cavity. The mechDuchenne muscular dystrophy (DMD) is associated with myocardial degeneration and fibrosis. The distribution of myocardial lesions is segmental and initially located throughout the basal inferior and contiguous lateral walls of the LV. In lateret al.[Today, pediatric heart transplantation covers an age range from the fetus listed for transplant at 36 weeks to the late adolescent. Diagnoses range in complexity from cardiomyopathy to the most complex of congenital heart malformations. The five-year survival rate for cardiac transplants is greater than 70%. As per Wigfield, et al. the actuIn post-transplant evaluations, there are conflicting reports of poor sensitivity of MPI to detect transplant vasculopathy (TV), which is99mTc Annexin V and 111In Pentetreotide. Although both agents are used to identify graft rejections, their mechanisms of action are different. 99mTc labeled Annexin V imaging[99mTc. This has a high Ca2+ dependent affinity towards phosphatidyl serine (PS). Apoptosis or cell death exposes a phospholipid, PS that is normally confined to the inner leaflet of the cell membrane bilayer. Annexin’s high affinity for binding to PS is the basis for apoptosis imaging. Somatostatin analogues like111In Pentetreotide[99mTc Annexin and 111In monoclonal antimyosin imaging hold promise in cardiac transplant patients but need more sample studies.Radiopharmaceuticals used in post cardiac transplant evaluations are V imaging is used etreotide are beinet al.[Normally, the RV is not visualized in MPI basically because of higher LV muscle mass. However, myocardial uptake of perfusion tracers by the RV wall is increased and marked in patients with pressure with or without volume overload of the RV. With reget al.Surgery for congenital heart disease may be divided into two types: Univentricular or biventricular. Biventricular repair encompasses situations wherein both ventricles are separately used to support the systemic and pulmonary circuits, such as repair of ventricular septal defect or tetralogy of Fallot. Univentricular palliation, also known as Fontan or Fontan-type repair may be net al.[201Tl and 123I-BMIPP (Iodinated fatty acids) have been undertaken in patients with a single RV to show that the myocardium is normally perfused and is hypertrophied at rest but metabolically compromised at apico-anterior regions with regional contractile dysfunction. Myocardial SPECT which highlights this contractile dysfunction in cyanotic heart disease is primarily linked to impaired free fatty acid metabolism rather than to a myocardial scar, an information which cannot be obtained from other imaging modalities. This also has prognostic implication in patient management as it signifies an ongoing microvascular or myocardial derrangement thus providing an opportunity for intracardiac repair.Therefore, in both these cases where morphological RV functions as a systemic ventricle, MPI can be performed to assess perfusion defects. Both reversible and fixed perfusion defects are observed more commonly in anterior, inferior, and septal walls rather than in lateral walls. The extent of fixed defects corresponds to the degree of depressed global RV function and reveet al. using 20Various imaging techniques are used to assess cardiac status. Imaging of the heart has been limited to cardiac catheterization, echocardiography, and nuclear medicine imaging. With new advances in both hardware and software in the last two decades, there is now a major change in the role of cross sectional imaging, i.e., CT and MRI, in the assessment of cardiac disease.et al.[Echocardiograms are universally available, cheap but operator dependent, and fail in cases of poor Echo window. A dobutamine stress echocardiography (DSE) exhibits a comparable accuracy with respect to MPI for diagnosing coronary artery disease, localizing coronary artery stenosis, and detecting regional myocardial abnormalities. The wallet al. As the tA multislice CT now provides retrospective cardiac gating and the entire procedure could be accomplished in one breath hold. With excellent spatial (0.625 mm) and temporal resolution (0.5 sec), CT imagiFor CAD evaluation, physiological significance of a known coronary lesion, collateralization of vessels, revascularization strategies  can only be answered by stress gated MPI/PET studies with excellent reproducibility. With the availability of SPECT-CT scanners, both MPI and multislice CT information obtained in the same sitting can be overlaid to provide better roadmaps to the cardiologist. DSE and stress thallium-201 MPI were compared for detecting coronary artery disease in 120 consecutive patients who underwent concomitant quantitative coronary angiography. Both tesThe DSE has been reported to be a safe and feasible pharmacological method for the evaluation of patients with suspected CAD. But in children with variable heart rates, MPI may become abnormal while WM is still normal, resulting in both higher sensitivity and diagnostic accuracy of MPI over WM analysis for detecting CAD. Another Standard MRI techniques do not allow real-time imaging for on-line assessment of a pharmacologically-induced myocardial contraction reserve, although this may become possible in the future with improved echo-planar techniques. Patients with a pacemaker and implants are contraindications for MRI. Moreover, quantitative assessment of wall thickness at rest and after dobutamine infusion is time-consuming and difficult to use for routine clinical purposes. However, faster imaging techniques used in Gold standard investigation in the assessment of myocardial viability as of now is FDG PET. Preserved glycolytic activity as estimated by regional FDG uptake in myocardial regions with impaired function has been reported to be accurate for the differentiation of viable myocardial tissue from scar. Myocardial perfusion imaging (MPI) with SPECT and flouro deoxy glucose (FDG) PET are well suited to provide all this information apart from quantification of these parameters. FDG-PET is superior to all existing imaging modalities as it provides metabolic status of myocardium prior to CABG. Although preservation of end-diastolic wall thickness as assessed by MRI in akinetic myocardial regions was proposed as an indicator of viable myocardium studies There is no investigation that can provide all information without fallacy. Today we are privileged to have multiple imaging modalities and fusion imaging at an affordable cost with 2D and 3D volume rendering reconstruction advancements. It is for us to choose the right blend of anatomical and physiological imaging techniques to optimize patient care.


A patient of severe chronic renal failure presented with hyperkalemic electrocardiographic (ECG) changes and hyperkalemia. Following a session of hemodialysis, when he reverted to normokalemia, the repeat ECG revealed left ventricular hypertrophy (LVH). Thus we confronted with a situation of hyperkalemia, masking the LVH on ECG initially and when the hyperkalemia was corrected with dialysis the LVH showed in ECG. The plausible explanation for this electrophysiological behaviour was offered. The electrocardiographic (ECG) changes suggesting left ventricular hypertrophy and hyperkalemia have been well established. However, there appears a paucity of understanding regarding the behavior of ECG in a compounding situation of hyperkalemia in the background of left ventricular hypertrophy. Keeping in view a similar situation encountered by us in a patient, we propose to submit this observation with a plausible scientific reasoning.3; serum creatinine, 9.9 mg/dl; blood urea, 206 mg/dl; serum sodium, 139 meq/l and serum potassium, 7 meq/l. Appropriate antihyperkalemic measures and hemodialysis was initiated. Following are the biochemistry of posthemodialysis serum: serum creatinine, 5.6 mg/dl; sodium, 136 meq/l; potassium, 5.3 meq/l and calcium, 8.9 mg/dl. A repeat ECG performed in the patient after hemodialysis revealed normal sinus rhythm, heart rate of 73/min, left axis deviation, reappearance of P waves, high-amplitude QRS complexes suggestive of left ventricular hypertrophy and a decrease in the T wave amplitude [A 55-year-old male presented to the emergency room with pulmonary edema. He did not provide the history of any comorbid illnesses and was not on any medication. On examination, he was found to have anemia orthopnea, cyanosis and anasarca. The patient exhibited bradycardia and hypotension. His ECG on admission revealed a heart rate of 63/min, absence of P waves, junctional rhythm and tall T waves . His hemmplitude . UltrasoPhysiologically, the electrical activation of the ventricles may be depicted in a simplified presentation as a small initial vector from the left to right through an interventricular septum, followed by a large vector from the left to the right through the free wall of the ventricle. Under condition of left ventricular hypertrophy, the abnormalities of the QRS complex noted were increased magnitude of QRS deflection and an increase in the left ventricular activation time. The increased magnitude of the left ventricular activation time was attributed to factors such as increased left ventricular muscle mass, increased left ventricular muscle surface and proximity of the left ventricle to the chest wall.12In hyperkalemia, a reduction occurs in the cardiac resting membrane potential due to decrease in the transmembrane potassium gradient, thereby inactivating sodium channels and decreasing the conduction velocity. This leads to inexcitability of the cardiac muscle fiber, resulting in broadening of QRS, with a decrease in the amplitude of P and QRS complexes. Eventually, sine wave and cardiac arrest in diastole occur.5In a compounding situation of hyperkalemia, manifesting in an already existing left ventricular hypertrophy, a possible explanation may be that when the conduction velocity and the excitability of the cardiac muscle decreases in hyperkalemia, the manifestation of increased amplitude of the QRS complex suggestive of left ventricular hypertrophy is masked. However, once hyperkalemia reverts to normokalemia, the hypertrophied left ventricle manifests its changes in the ECG.This case report highlights the interaction of two opposing coexisting situations such as hyperkalemia on the background of left ventricular hypertrophy, which can manifest initially with hyperkalemic changes in the ECG and after correcting to normokalemia via dialysis, the left ventricular hypertrophy may reappear in the ECG.


Heart failure is one of the major health care issues in the Western world. An increasing number of patients are affected, leading to a high rate of hospitalization and high costs. Even with administration of the best available medical treatment, mortality remains high. The increase in left ventricular volume after a myocardial infarction is a component of the remodeling process. Surgical Ventricular Restoration (SVR) has been introduced as an optional therapeutic strategy to reduce left ventricular volume and restore heart geometry. So far, it has been established that SVR improves cardiac function, clinical status, and survival in patients with ischemic, dilated cardiomyopathy and heart failure. Since its first description , SVR has been refined in an effort to standardize the procedure and to optimize the results. This review will discuss the rationale behind surgical reversal of LV remodeling, the SVR technique, its impact on cardiac function and survival, and future expectations. Heart failure (HF) remains a major public health problem. Almost 5 million patients in the United States are affected, and 30 to 40% of patients die from HF within 1 year after receiving the diagnosis , 3. HearLeft ventricular remodeling is the process by which mechanical, neurohormonal, and possibly genetic factors, alter ventricular size, shape, and function. Remodeling occurs in several clinical conditions, including myocardial infarction (MI), cardiomyopathy, hypertension, and valvular heart disease.Myocardial infarcts (MI), particularly large, transmural infarcts, result in a number of structural changes involving both the infarcted and non-infarcted zones . LV remoSVR has been developed with the goal of improving cardiac function through a reduction in LV wall tension in accordance with the principle of Laplace’s law. Since LV wall tension is directly proportional to the LV internal radius and pressure, and inversely proportional to wall thickness, any intervention to optimize this relationship would be beneficial in terms of improving wall compliance and reducing filling pressure. Optimization may also be beneficial in terms of enhancing the contractile performance of the LV by increasing the extent and velocity of systolic fiber shortening .2 for preoperative LVEDV < 150 mL/m2 or 70 mL/m2 for preoperative LVEDV > 150 mL/m2. It was removed before closure of the ventricle. Later, the procedure was adopted by many skilled and creative surgeons without real standardization, making results difficult to compare. McCarthy described a no-patch, double purse-string suture technique [TM, Chase Medical Richardson, TX), which is illustrated in Fig. (1) [After the first description of the linear suture by Cooley in 1958 , and theechnique ; MenicanFig. (1) . The man2) [The mannequin is removed before closure of the LV cavity, which is done with a direct suture if it is less than 3 cm large, or with an elliptical, synthetic patch if greater than 3 cm. Regardless, the mannequin is useful in giving the surgeon the correct position of the apex and avoiding sphericalization of the ventricle. The reconstruction of the apex can be difficult when the apical and inferior regions are severely dilated. To overcome this difficulty, Menicanti applied a modification of the Dor procedure that involves plication of the distal inferior wall before patch placement, thus placing the apex in a more anterior position as illustrated in Fig. (2) , 22.When indicated, the mitral valve is repaired through the ventricular opening with a double arm stitch running from one trigone to the other, embedding the two arms in the posterior annulus of the mitral valve. After that, the suture is tied to undersize the mitral orifice . 2. This study reported an improvement in EF from 29.6 ± 11.0% preoperatively to 39.5 ± 12.3% postoperatively (p < 0.001) and a decrease in LVESVI from 80.4 ± 51.4 ml/m2 preoperatively to 56.6 ± 34.3 ml/m2 postoperatively (p < 0.001). Thirty-day mortality after SVR was 5.3%, with this value being higher among patients in whom mitral valve repair was performed along with SVR (8.7%), versus patients in whom no mitral valve procedure was required . The overall five-year survival was 68.6 ± 2.8%. After five years, 78% of patients were not readmitted to the hospital for CHF. Surprisingly, in 2004, Mickleborough reported the results from a smaller group of patients (245) showing a lower in-hospital mortality rate (2.8%); one, five, and ten- year survivals were 92%, 82%, and 62% [1 shows a summary of the data from the literature.The first consistent results using SVR were reported by Dor and co-authors. They showed that the procedure improves LV function, NYHA functional class, and survival by reducing ventricular volumes and increasing the ejection fraction (EF). These results were observed not only in patients with classic dyskinetic aneurysms, but also in those with dilated ischemic cardiomyopathy and severe LV dysfunction. -26. More and 62% . PatientHow and when mitral regurgitation should be corrected during SVR, is still a topic of debate. In the failing heart, mitral regurgitation occurs secondarily to annular dilatation, altered left ventricular geometry, and papillary muscle dysfunction , 41. VolSVR has the potential to improve mitral functioning by reducing LV volumes, papillary muscles distances , and rebuilding a more normal heart geometry , 48, 49.Despite excellent contributions supporting SVR as a therapeutic strategy for patients with ischemic HF, few data are available on LV diastolic function (DF) in patient undergoing SVR. The ventricular remodeling following an acute myocardial infarction is accompanied by changes in diastolic properties of the LV due to scar formation. This, in turn, increases chamber stiffness and compensatory hypertrophy of the remote zone, which causes delayed relaxation . In turn3) [It is reasonable to assume that the geometric implications, as well as the volume of the residual LV cavity, may affect the diastolic function in patients undergoing SVR. We addressed this issue in a recent paper . One hun3)  and the The complex sequence of the events leading to LV remodeling involves the presence of electrically unexcitable scars. This predisposes the LV to electrical heterogeneity, which in turn, induces non-uniform contraction, relaxation, and filling with further deterioration of global systolic and diastolic function. Cardiac resynchronization therapy (CRT) has resulted in enhanced quality of life, improved symptoms, and improved survival in patients with refractory heart failure due to systolic dysfunction and mechanical dyssynchrony as defined by wide QRS , 61. AltWith an eye to the fact that SVR attempts to exclude the scarred tissue, our group reported data from a prospective study conducted on thirty patients undergoing SVR at the Cardiothoracic Center of Monaco . PatientThe increased risk for life-threatening arrhythmias after a myocardial infarction is related to the complex sequence of events leading to LV remodeling. These events include LV dilatation, which, in turns, increases wall stress and stretch (stretch is arrhythmogenic per sè), and to the presence of electrically unexcitable scars. Our group has previously demonstrated that patients with ventricular arrhythmias (spontaneous or inducible) have end-diastolic and end-systolic volumes significantly larger than those seen in non-inducible patients , 66. SVRBrain natriuretic peptide (BNP) and its precursor, N-terminal pro-brain natriuretic peptide (NT-pro-BNP), are synthesized in the myocytes and released during hemodynamic stress. This stress occurs when ventricles are dilated, hypertrophic, or subject to increased wall tension . The proPrevious studies have demonstrated that BNP is a valuable biomarker for LV remodeling as its activation is more pronounced in patients with functional MR rather that in those with organic MR, and it may predict mortality and morbidity in patients with chronic HF -74. UsinTo date, the data reported in the literature support the benefit of SVR in improving cardiac function and survival in patients with dilated ischemic cardiomyopathy and heart failure. However, the limitations of different studies are still considerable and make the results presently inconclusive. First, the majority of studies are retrospective and are not powered to address specific end-points either for the number of patients, the limited number of events, or for the length of the follow-up. Second, physiologic studies based on invasive measurements (pressure-volume relationships) have been conducted only on small series of patients. Also, the postoperative measurements were made immediately after the completion of the surgical procedure, or at most, ten days after surgery. Additional information will be provided from these studies performed at later time points. Third, the lack of a control group is one of the major limitations of these studies, and the combination of SVR and coronary artery bypass surgery in the majority of the study groups does not allow for evaluation of the specific role of each procedure. Fourth, different surgical techniques and operative protocol must be taken in account in interpreting the results.Surgical Treatment for Ischemic Heart Failure), sponsored by the National Institutes of Health, is a multicenter, international, randomized trial designed to assess the potential superiority of CABG over intensive medical therapy in improving long-term survival (“the revascularization hypothesis”). It also assesses the benefit of surgical ventricular restoration combined with CABG in improving survival free of hospitalization for cardiac cause compared to CABG alone in patients with LV dysfunction ( EF < 35%) and coronary artery disease suitable for surgical revascularization (“the reconstruction hypothesis”). Patients will undergo cardiac magnetic resonance imaging, echocardiography, neurohormonal and genetic profile, and radionuclide studies to ensure consistent testing practices and standardization of data collection. Testing will also identify eligible patients and address specific questions related to the primary hypotheses. Until the results of this study are available, a close collaboration between cardio-logists and cardiac surgeons in patient selection, perioperative management, and long-term follow-up is critical to definitively change the clinical decision making in the treatment of ischemic heart failure.The STICH Trial (


Patients on peritoneal and hemodialysis have accelerated atherosclerosis associated with an increase in cardiovascular morbidity and mortality. The atherosclerosis is associated with increased arterial stiffness, endothelial dysfunction and elevated oxidative stress and inflammation. The aims of this study are to investigate the effects of peritoneal and hemodialysis on arterial stiffness, vascular function, myocardial structure and function, oxidative stress and inflammation in incident patients with end stage kidney disease.2 isoprostanes and 3) inflammation measured by plasma pentraxin-3. Secondary outcomes will include additional measures of oxidative stress and inflammation, changes in vascular function assessed using the brachial artery reactivity technique, carotid artery intimal medial thickness, augmentation index and trans thoracic echocardiography to assess left ventricular geometry, and systolic and diastolic function. Patients will undergo these measures at baseline , then at six and 12 months after starting dialysis.This is an observational study. Eighty stage five CKD patients will be enrolled and followed for one-year. Primary outcome measures will be changes in 1) arterial stiffness measured by aortic pulse wave velocity, 2) oxidative stress assessed by plasma FThe results of this study may guide the choice of dialysis modality in the first year of treatment. It may also lead to a larger study prospectively assessing the effect of dialysis modality on cardiovascular morbidity and mortality.ACTRN12609000049279 End stage kidney disease (ESKD) patients undergoing dialysis have a substantially increased risk of cardiovascular morbidity and mortality In 2007,Oxidative stress and inflammation are associated with the development of cardiovascular disease in dialysis patients ,2. The mMeasures of arterial stiffness such as aortic pulse wave velocity (PWV) predict morbidity and mortality in patients with kidney disease . NumerouThis is a multi-centre, one-year prospective observational study utilising convenience sampling. Eighty stage five CKD patients, not yet on dialysis, will be enrolled. They will commence either haemodialysis or peritoneal dialysis according to clinical need and personal preference after undertaking pre-dialysis education. They will be followed for one year after the commencement of their treatment modality. Although this design may be a limitation it is the only ethical one for our aims.Patients will be recruited from the Royal Brisbane and Women's Hospital, Brisbane, and the Launceston General Hospital and Satellite Renal Unit Burnie which service populations of approximately 1.2 million and 250,000 respectively.2, 3) undertaken pre-dialysis education program, 4) provide written informed consent.Patients must satisfy the following criteria prior to entering the study: 1) age > 18 and < 85 years, 2) an estimated glomerular filtration rate (eGFR) of < 15 mL/min/1.73 mFailure to gain consent or inability to tolerate glyceryl trinitrate (GTN).The trial is utilising a sample of convenience recruiting participants whom will likely commence either haemodialysis or peritoneal dialysis within three-six months. Approximately six-eight weeks prior to commencing their treatment modality, all eligible patients will undergo baseline measurements of vascular and myocardial structure and function and blood will be collected for the oxidative stress and inflammatory measures. They will then undergo the same measurements at six and 12 months after commencing their chosen treatment.The nephrologists, trial co-ordinators and research assistants will be responsible for the selection of all eligible patients to be approached. Medical records will be screened prior to attendance at pre-dialysis education clinics. At this stage patient preference for dialysis treatment modality is discussed. After explanation of the study, the subject will be provided with a patient information sheet and informed consent form. The subject will then be asked to take this away with them and arrangements will be made to follow up via a telephone call. If the subject agrees to participate, they will sign the consent form.A trained phlebotomist will take 40 millilitres of blood during visits to their hospital pathology laboratory or taken on dialysis once this has started.The Tasmanian Statewide Scientific and Ethics Committee has approved the study. The Ethics Committee will be provided with annual reports of the trial progress and will promptly receive all adverse event reports.The primary objectives are to assess the effects of dialysis modality on arterial stiffness and oxidative stress and inflammation. The hypothesis is that peritoneal dialysis will significantly improve the rates of change in these measures compared with hemodialysis. The primary outcome measures are outlined below.The primary outcome for arterial stiffness will be aortic PWV. Carotid to femoral PWV will be derived by electrocardiography-gated sequential applanation tonometry  using the foot-to-foot method  . Testing2 isoprostane measured using gas chromatography mass spectrometry [The primary outcome measure for oxidative stress will plasma Ftrometry  and for trometry .Secondary objectives are to assess the effects of dialysis modality on vascular structure and function, myocardial structure and function and additional measures of arterial stiffness, oxidative stress and inflammation.Carotid Intimal Media Thickness (CIMT) will be used to measure structural alterations of the posterior wall of both the left and right common carotid arteries. CIMT refers to the combined thickness of the intimal and medial layers of the carotid arterial wall . This coBrachial artery reactivity (BAR) will be measured to assess vascular function. A 12 MHz linear array transducer and ultrasound scanner  will be used to record the dilator response of the brachial artery to increased blood flow generated during reactive hyperaemia of the downstream forearm. The measures will be performed on the non-fistula arm (if a fistula is present). A blood pressure cuff is placed on the upper aspect for the lower forearm. The brachial artery will be scanned in the longitudinal plane, acquiring a baseline image about five cm above the antecubital fossa. The transducer is left in this scanning position. The cuff is then inflated to 220 mmHg for four minutes. Following cuff deflation, images will be acquired at five and 10 seconds then at 10-second intervals up to 120 seconds. Following a 15-minute rest, this process is then repeated. The 15-minute interval allows the artery to return to its baseline state. The last phase of the BAR test involves the administration of sublingual GTN. Following a final 15-minute interval, a baseline image is acquired and then 300 μg of sublingual GTN will be administered. After three minutes, images of the brachial artery will be obtained as described above. All images will be stored for later offline analysis and archiving. The truest diameter of the brachial artery is indicated by the "double line sign", which is the crisp intima-media and media-adventitia boundaries on the near and far walls . These wi  with a phased-array ultrasound probe (S3) will be used for these measures. Patients will be positioned in the left lateral recumbent position.Transthoracic echocardiography (TTE) will be performed to assess left atrial and left ventricular (LV) geometry and myocardial systolic and diastolic function. The Vivid LV dimensions will be obtained using two-dimensional (2D) and M-Mode measurements from the parasternal long axis window. Measurements of the interventricular septal wall thickness during diastole (IVSTd), posterior wall thickness (PWT), LV internal dimension during diastole (LVIDd) and LV internal dimension during systole (LVIDs) will be measured to derive the Left Ventricular Mass (LVmass).LV mass will be calculated from LV linear dimensions using the ASE recommended formula:These measures will then be divided by body surface area to obtain Left Ventricular Mass Index (LVMI).Relative wall thickness (RWT) will be calculated using the American Society of Echocardiography (ASE) recommended formula :Patients will have concentric hypertrophy if RWT > 0.42 with increased LV mass; eccentric hypertrophy if RWT < 0.42 with increased LV mass and concentric remodelling if the LV mass normal and RWT was increased. Left at1 = Maximal LA area from A4C. A2 = Maximal LA area from A2C.Where L = shorter length from either A4C or A2C. ALV systolic function will be assessed by calculating the ejection fraction (EF) using the disc summation method (Modified biplane Simpson's rule) . ApplyinWhere LVEDV = left ventricular end diastolic volume, LVESV = left ventricular end systolic volume. For the purpose of this study, a normal EF will be ≥ 55% .LV Diastolic Function will be performed using pulsed Doppler interrogations of the LV inflow tract from the A4C view. This will allow for the measurement of peak flow velocity of early (E) and late (A) LV filling. Early diastolic flow (deceleration time DT) and mitral A wave duration. The isovolumic relaxation time (IVRT) will be recorded from the A5C view using continuous wave Doppler interrogation.Tissue Doppler Imaging (TDI) will measure the longitudinal velocities within the myocardium from the apical 4-chamber view, in pulse wave (PW) mode. This normally consists of a positive systolic wave (S') and 2 diastolic peaks, one during early diastole (E') and the second during atrial contraction (A'). The mitral E velocity and annular E' will be compared as a ratio (E/E'). An E/E' > 10 from the lateral wall is will be indicative of elevated LV filling pressures . RecordiPulse wave analysis (PWA) will be used to derive a central (ascending aortic) pressure waveform via applanation tonometry. This method involves using a generalised transfer function to generate a central pressure waveform, which we have shown to be valid . Is a reAugmentation index (AIx) is regarded as a measure of systemic arterial stiffness that will be derived from the central and radial pressure waveforms as previously described . The ratAdditional secondary outcome measures for oxidative stress and antioxidant status will include plasma protein carbonyls, total antioxidant capacity, plasma antioxidant enzyme activities, and for inflammation, C reactive protein and a cytokine panel consisting of IL-6, IL-8, IL-10 and TNF-alpha.Additional data to be collected will include self-reported health (SF-36 questionnaire), physical activity levels  nutritional status (four-day diet diary), adverse events including mortality, morbidity, hospitalizations and procedures performed within the day procedure unit (e.g. iron infusions and arterio-venous fistula formation).The study flow is summarized in Figure These visits occur at six months and one year after commencement of dialysis. An envelope containing a letter confirming appointment, pathology request forms, four-day diet diary, SF 36 and physical activity surveys will be mailed to patients. They will be asked to complete surveys and the diet diary and to attend the pathology laboratory seven days prior to visits. At these visits, data acquisition from measures and tests described above will be performed again. Patients will be examined on a non-haemodialysis day for hemodialysis patients and during the non-dwell phase period for peritoneal dialysis patients.Subjects will be withdrawn from the study at their request, without prejudice, as documented and explained at the time of consenting.Data from other studies indicates that pentraxin-3 has the highest variability of the three primary outcome measures  and therefore will be used to estimate the sample size required. We assume that a decrease of 10% in pentraxin-3 would be clinically significant. Therefore, with alpha = 0.05 and beta = (1–0.8), we would need 23 patients in one of the groups. It is expected with an initial enrolment of 80 patients, 60 will complete the one-year measures. Based on the current distribution patterns within the renal services we estimate that 37 of these patients will be receiving haemodialysis and 23 receiving peritoneal dialysis.All baseline continuous variables between groups will be compared using general linear modelling, whilst categorical variables will be compared using exact logistic regression. The rates of change will be estimated for each patient separately by linear regression. general linear modeling, will be used to compare the mean rate of change in primary outcome measures between groups, unadjusted and adjusted for actual and potential confounding variables. Mean differences, 95% confidence intervals and P-values will be corrected for repeated measures, and P-values corrected for multiple comparisons by the Holm method. All analyses will be performed using Stata/IC 10.1 for Windows .The objective of this study is to assess whether peritoneal dialysis will have less effect on arterial stiffness, vascular function, myocardial structure and function, oxidative stress and inflammation than hemodialysis. Comparing the effects of renal replacement therapy modalities on surrogate cardiovascular markers over time will provide unique important information to assist in the treatment of patients with ESKD. Data from this trial may also lead to a larger scale study assessing the effect of dialysis modality on major cardiovascular endpoints.The authors have no competing interests to declare. Baxter Healthcare, although providing a proportion of funding for the study through a competitive grant process, has no influence on the outcome. Although Baxter Healthcare is a provider of peritoneal dialysis therapy it is not the sole provider to the Hospitals included in this study. The outcome of the study will not impact on the balance of product use after completion of the study. However, the study outcome may influence the distribution of the use of peritoneal dialysis compared with hemodialysis in the first twelve months of therapy. This could result in an increased or decreased use of either dialysis therapy or in fact no alteration.RGF and JSC are responsible for the design of this clinical trial, the construction of the protocol and writing this manuscript. HH, DR and SR provided protocol advice and will be involved with recruitment and retention, manuscript review and data analysis. IKR has and will provide statistical advice. DPG provided technical advice and manuscript review. RD will perform measures of vascular and cardiac structure and function and provided protocol design elements related to these. JES provided advice on vascular measures and manuscript review. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2369/10/8/prepub


Fetal cardiac anomalies involving the ventricular and atrial septa, outflow tracts, chambers, and valves are often encountered in routine screening. However, the prenatal detection of a fetal left ventricular aneurysm is rare. This report describes the case of a left ventricular aneurysm that was diagnosed at 24 weeks of gestation; the diagnosis was later confirmed by postnatal echocardiography. This case is reported because of its rarity and the characteristic echocardiographic findings. An early specific antenatal USG diagnosis helps in prognostication and in counseling of the parents. We would like to report the case of a left ventricular aneurysm diagnosed at 24 weeks of gestation. The diagnosis was eventually confirmed on postnatal echocardiography.An asymptomatic young (26-year-old) primigravida was referred to us at 24 weeks of gestation for a routine antenatal USG. Her personal and family histories were noncontributory. She gave no history of exposure to any teratogen or of a recent infection. The USG showed a normal placenta, normal volume of liquor, and normal biometry corresponding to 24 weeks' gestation. The cardiac four-chamber view was abnormal because of the presence of a dilated left ventricle with a ‘boot-shaped’ appearance . We carrWith the advent of sophisticated machines and software, fetal echocardiography has become a very useful investigation. The detection rates of cardiac anomalies are increasing, as also the specificity of the diagnoses. In one rA ventricular diverticulum has to be differentiated from an aneurysm. A diverticulum is usually a small finger-like, synchronously contracting, narrow projection, which shows the same thickness and layering as the rest of the myocardium. Fetuses 7The prognosis of a fetal left ventricular aneurysm depends on the time of detection, the size and the progression of the aneurysm, the presence or absence of compression of the fetal lungs, involvement of the mitral opening, reversal of atrial shunt, associated cardiac failure, and the presence of myocardial damage or connective tissue disorders.101110This fetus was delivered by a Cesarian section for purely obstetric indications, and the postnatal detailed echocardiography confirmed the antenatal diagnosis.