Transposition of the great arteries (TGA), also referred to as complete transposition, is a congenital cardiac malformation characterised by atrioventricular concordance and ventriculoarterial (VA) discordance. The incidence is estimated at 1 in 3,500–5,000 live births, with a male-to-female ratio 1.5 to 3.2:1. In 50% of cases, the VA discordance is an isolated finding. In 10% of cases, TGA is associated with noncardiac malformations. The association with other cardiac malformations such as ventricular septal defect (VSD) and left ventricular outflow tract obstruction is frequent and dictates timing and clinical presentation, which consists of cyanosis with or without congestive heart failure. The onset and severity depend on anatomical and functional variants that influence the degree of mixing between the two circulations. If no obstructive lesions are present and there is a large VSD, cyanosis may go undetected and only be perceived during episodes of crying or agitation. In these cases, signs of congestive heart failure prevail. The exact aetiology remains unknown. Some associated risk factors  have been postulated. Mutations in growth differentiation factor-1 gene, the thyroid hormone receptor-associated protein-2 gene and the gene encoding the cryptic protein have been shown implicated in discordant VA connections, but they explain only a small minority of TGA cases.The diagnosis is confirmed by echocardiography, which also provides the morphological details required for future surgical management. Prenatal diagnosis by foetal echocardiography is possible and desirable, as it may improve the early neonatal management and reduce morbidity and mortality. Differential diagnosis includes other causes of central neonatal cyanosis. Palliative treatment with prostaglandin E1 and balloon atrial septostomy are usually required soon after birth. Surgical correction is performed at a later stage. Usually, the Jatene arterial switch operation is the procedure of choice. Whenever this operation is not feasible, adequate alternative surgical approach should be implemented. With the advent of newer and improved surgical techniques and post operative intensive care, the long-term survival is approximately 90% at 15 years of age. However, the exercise performance, cognitive function and quality of life may be impaired. Transposition of the great arteries; physiologically uncorrected transposition; complete transposition; atrioventricular concordance with ventriculoarterial discordance. The European paediatric cardiac code for this disease is 01.05.01.The Morbid Anatomy of Some of the Most Important Parts of the Human Body". However, the term transposition was only applied in 1814, by Farre, meaning that aorta and pulmonary trunk were placed (positio) across (trans) the ventricular septum.The transposition of the great arteries was first described by Mathew Baillie in 1797, in the second edition of the book "In fact, this congenital cardiac malformation is characterised by atrioventricular concordance and ventriculoarterial discordance. In other words, the morphological right atrium is connected to the morphological right ventricle which gives rise entirely to or most of the aorta; the morphological left atrium is connected to the morphological left ventricle from where the pulmonary trunk emerges .congenitally corrected transposition of the great arteries describes a different entity that conjugates atrioventricular and ventriculoarterial discordance [completed transposition was used to describe either congenitally corrected or uncorrected transposition. In both situations, the great arteries were completely or predominantly misplaced across the ventricular septum. The designation "incomplete transposition" was reserved to other types of malpositions of the great arteries, such as double outlet right and left ventricle. At present, the term complete transposition is usually used to describe only the physiologically uncorrected transposition.The term cordance . In the a-, d- and l- transposition describe the spatial relationship between the aorta and the pulmonary trunk, and should not be used to define this anomaly [The prefixes  anomaly . In d-tr anomaly . In congThese different classifications and nomenclatures have lead in some cases to the inadequate or imprecise use of certain terms. To avoid ambiguity, it is important to use a description based on segmental analysis, and therefore, in our opinion, transposition of the great arteries should be defined as concordant atrioventricular and discordant ventriculoarterial connections.The hearts with atrioventricular concordance and ventriculoarterial discordance represent 5–7% of all congenital heart diseases -8. In 10In the hearts with transposition of the great arteries, atriums and ventricles retain their typical configuration, and conduction tissue assumes a similar distribution. A right-sided subaortic infundibulum usually separates the tricuspid from the aortic valve, and fibrous continuity is present between the mitral and the pulmonary valve. Coronary arteries' anatomy may assume diverse patterns of epicardial distribution, but they invariably originate in the aortic sinuses facing the pulmonary trunk. This fact is constant and independent of the two great vessels' spatial relation.In 50% of the cases, the ventriculoarterial discordance is an isolated finding. This condition is designated as simple transposition. By contrast, complex transposition includes all the cases with coexisting malformations, such as ventricular septal defects, left ventricular outflow tract obstruction, aortic arch anomalies, and anomalous venous systemic return.Ventricular septal defects are particularly common, and their location and size are variable. They may be associated with a certain degree of malalignement between the outlet and trabecular septum. If an anterior and rightward deviation of the outlet septum is present, concomitant pulmonary overriding and subaortic stenosis can be expected. In these cases, hypoplasia, coarctation, or even interruption of the aortic arch may be encountered. On the other hand, if the outlet septum is deviated posteriorly and leftwards, it may lead to subpulmonary stenosis .In fact, left ventricular outflow tract obstruction is present in one-eighth to one-third of cases, being far more common in the presence of a ventricular septal defect than when the ventricular septum is intact. This impediment to the flow of blood can be caused by a wide spectrum of lesions, either at valvar or subvalvar level, and deviation of the muscular outlet septum is but one of them. Bulging of the septum, a fibrous shelf or fibromuscular tunnel, tissue tags, and anomalous attachment of the atrioventricular valvar tension apparatus can also be substrates for stenosis .A careful assessment of all these morphological variants is of the uttermost significance, particularly for surgical planning and in foreseeing eventual complications.In the hearts with concordant atrioventricular and discordant ventriculoarterial connections, the systemic and pulmonary circulations run in parallel, rather than in series. As such oxygenated blood flows through a closed circuit that involves the lungs and the left cardiac chambers. On the other hand, systemic blood flow is supplied by another closed circuit that begins and ends in the right cardiac chambers. In this setting, survival is only possible, if there is adequate mixing between the two circulations, be it between the septums or through the arterial duct.The parallel circulation just described results in a significant hypoxemic status that is observed clinically by central cyanosis. The bluish discoloration of the skin and mucous membranes is therefore the basic pattern of clinical presentation in transposition. Its onset and severity depend on anatomical and functional variants that influence the degree of mixing between the two circulations.Limited intercirculatory mixing, usually present if the ventricular septum is intact or the atrial septal defect is restrictive, is related to progressive and profound central cyanosis evident within the first hours of life. Likewise, associated left ventricular outflow tract obstruction or pulmonary obstructive disease can reduce the blood flow to the pulmonary vascular bed, and thus contributing to a marked cyanotic state. However, if no obstructive lesions are present, and there is a large ventricular septal defect that allows for satisfactory mixing between the two circulations, cyanosis may go undetected and only be perceived during episodes of crying or agitation. In these cases, signs of congestive heart failure prevail due to excessive ventricular workload. Tachypnoea, tachycardia, diaphoresis, poor weight gain, a gallop rhythm, and eventually hepatomegaly can be then detected later on during infancy.Heart murmurs associated with left outflow tract obstruction, persistent arterial duct or due to a septal defect may be heard, but they are not a constant finding.The exact aetiology of this disease is still unknown. However, some associated risk factors, namely gestational diabetes mellitus ,13, mateSignificant advances in the understanding of the underlying genetic mechanisms have been achieved over the last decade. Several mutations have been implicated as the cause of discordant ventriculoarterial connections. The genes involved so far are the growth differentiation factor-1 gene , the thyhyperoxia test are suggestive of a cyanotic congenital heart condition, further investigations are required.Diagnosis involves careful history taking and physical examination of the patient under good light condition. When these and the chest X-ray and an electrocardiogram may be helpful at this stage, but their respective findings are not specific. In transposition, a narrowed superior mediastinum gives to the cardiac silhouette a characteristic egg-shaped appearance on chest radiography. Cardiomegaly with increased pulmonary vascular markings may be found if a ventricular septal defect is present. The main electrocardiographic features are a rightward deviation of the QRS complex axis associated with right ventricular hypertrophy; biventricular hypertrophy is evident in conditions that lead to a left ventricular overload.A echocardiography. This imaging modality provides accurate morphological and functional assessment of the heart, being able to show the specific features of the transposition of the great arteries. In the four-chamber view, one assesses atrioventricular concordance, but the ventriculoarterial discordance is better observed using other incidences. In the five-chamber, parasternal long-axis or even subcostal view, the vessel arising from the morphologically left ventricle has a posterior course and bifurcates immediately, being identified as the pulmonary trunk  leading to excessive alveolar distension should be avoided so that intercirculatory shunts are not disturbed.The correction of metabolic acidosis with bicarbonate is important, as it may compromise myocardial function and in case of cardiac failure, inotropic agents or diuretics may be necessary.arterial switch operation is the procedure of choice used to achieve complete physiological and anatomical repair. Its supremacy has been corroborated by long-term results that show preservation of good left ventricular function, sinus rhythm and a low mortality with a survival rate of 88% at both 10 and 15 years. The rare post operative complications are mainly related to prolonged peri-operative ischaemia times, aortic regurgitation and coronary artery obstruction, which may result in myocardial ischaemia or even infarction. A low reoperation rate has also been reported, pulmonary stenosis at the site of reconstruction being the most common cause [The on cause .In this surgery, the aorta and pulmonary trunks are sectioned and their distal extremities are transposed and anastomosed; coronary arteries are then translocated to the neo-aorta Fig. . The corIn the specific context of transposition with intact septum, the newborn should be operated preferably within the first two-weeks of life. This would ensure that left ventricle has not suffered significant involution, and its contractility is still able to support the systemic circulation. In older neonates and young infants, the left ventricle may need to be retrained, before the arterial switch is attempted, by pulmonary artery banding, with or without an associated Blalock-Taussig shunt. This intermediate step should establish a left/right ventricular pressure ratio between 0.6/0.75 without compromising the required pulmonary blood flow . This prIn complex transposition, the arterial switch procedure should be tailored towards the individual morphological aspects and complementary interventions may be necessary to repair concomitant malformations. In fact, an associated atrial defect, or more commonly the septostomy defect, can usually be closed by direct suture. Closure of the ventricular septal defect may require the use of a patch to close the communication or, if very small, may just be left open. An obstruction within the aortic arch is best repaired concomitantly, if necessary using a pulmonary homograft to enlarge the aorta . Some leTwo morphologic features may preclude however the use of this operation: an outflow tract obstruction lesion that can not be satisfactory relieved by resection and certain coronary artery patterns that may pose difficulties during coronary transfer.Whenever the arterial switch is not feasible, alternative approaches are required – Fig. In the presence of a ventricular septal defect, the most used options are the REV procedure or its modification and the Rastelli operation. In both, an intraventricular tunnel passing through the septal defect is created to connect the left ventricle to the aorta. In the Rastelli procedure, an extracardiac conduit is placed connecting the right ventricle to the pulmonary artery Fig. . HoweverThe Nikaidoh's procedure is also an option in the setting of discordant ventriculoarterial connections, ventricular septal defect and left outflow tract obstruction, especially if inadequate anatomy for a REV or Rastelli is present . The repUntil mid twentieth century, the treatment of transposition was restricted to few palliative measures and the natural history of the disease with its poor prognosis was an undeniable reality. By that time, the average life expectancy for a newborn with transposition was 0.65 years and the mortality rate at one year was 89.3% . With thThe authors declare that they have no competing interests.The authors equally contributed to this review article. They read and approved the final version of the manuscript.


We treated a 10 year 11 month old girl with severe mitral valve regurgitation, stenosis and dilated cardiomyopathy, presented with New York Heart Association (NYHA) functional classification IV. She acutely developed cardiogenic shock with a dyskinetic anterior-septal left ventricle and entered a shock state during our consultation about heart transplantation. Septal-anterior ventricular exclusion and mitral valve replacement were performed emergently. She successfully recovered from cardiogenic shock. Left ventricular end-diastolic diameter and fractional shortening improved from 71.5 mm  to 62.5 mm  and 7.6% to 18.3% respectively. Furthermore, her serum BNP decreased from 2217.5 pg/ml to 112.0 pg/ml. Her cardiac function has remained stable for 7 years since the procedures were performed. Dilated cardiomyopathy (DCM) is one of the most serious prognostic factors in heart disease ,2. BatisIn November 2001, a 10 year, 11 month old girl was admitted to our hospital with dyspnea on mild exertion and pretibial and palpebral edema.At 2 months, a heart murmur was detected. One year later, she was diagnosed with congenital mitral valve stenosis (MS) and mitral valve regurgitation (MR) by cardiac echogram and catheterization. Despite treatment with digitoxin and diuretics, her left ventricular end-diastolic diameter (LVDd) gradually increased and MR worsened. She received mitral valve replacement (MVR) at age 6, but her cardiac function continued to worsen and her LVDd increased despite of 9 years optimal medical treatment.201Tl and 123I-MIBG were detected in the anterior IVS and anterior LV wall by cardiac scintigraphy . Echocardiogram showed dilated LVDd, of 71.5 mm , reduced left ventricular fractional shortening (LVFS) (7.6%) and closure of one of the artificial mechanical valves. Left ventricular ejection fraction (LVEF) was also measured by cardiac catheterization, and the LVEF was 11.0% at this time. Serum BNP was elevated at 2217.5 pg/ml. Decreased up-takes of 2, respectively) and 7 years after  the SAVE procedure. Although single and monofocal premature ventricular conductions are occasionaly recorded on electrocardiography, her condition is stable and she is able to attend high school daily by wheelchair.Despite of treatment with bed-rest, diuretics and cardiotonic agents, her condition continued to worsen. While preparing to place her on the heart transplant waiting list, she went into a cardiogenic shock requiring mechanical ventilation and placement of an intra-aortic balloon pumping (IABP). Soon after the onset of the shock, SAVE procedure and the second MVR were performed emergently. We replaced a 23 mm diameter St. Jude Medical mechanical valve and tied up and patched the thin area of her anterior IVS and anterior LV wall with a sheet of patch after a close examination of her LV wall by intra-operative echocardiogram. Her LVDd decreased to 52.8 mm  after 1 and 62.5 mm  after 7 years of the SAVE procedure. Her LVFS elevated to 15.4% after 1 and 18.3% after 7 years of the SAVE procedure. Serum BNP remarkably decreased to 129.3 pg/ml after 1 and 112.0 pg/ml after 7 years of the SAVE procedure. Upon cardiac catheterization, LVEF had increased and LV volume index had not changed between 2 months after (16.6% and 180.6 ml/mSevere heart failure in children is commonly treated with diuretics, ACE inhibitors, calcium blockers, β-blockers and vasodilators ,11. PatiRandas Batista et al. described techniques to improve cardiac contraction and reduce LV diameter . But damLV diameter reduction has been performed worldwide in adults and has been shown to improve LV function in mid-term follow-up studies. However, long-term follow-up after the SAVE procedure , especiaIn conclusion, we report good long-term outcome in a child with DCM and large non-functional LV area treated with the SAVE procedure. She recovered from cardiogenic shock and her cardiac function has now been stable for more than 7 years after the SAVE procedure.NYHA: New York Heart Association; DCM: dilated cardiomyopathy; IVS: intraventricular septum; LV: left ventricular or left ventricle; SAVE: Septal Anterior Ventricular Exclusion; MS: mitral valve stenosis; MR: mitral valve regurgitation; LVDd: left ventricular end-diastolic diameter; MVR: mitral valve replacement; LVFS: left ventricular fractional shortening; IABP: intra-aortic balloon pumping.The authors declare that they have no competing interests.SB was an attending physician in the pediatric ward in Kyoto university hospital, and wrote most part of this manuscript. HD is an attending physician in the pediatric outpatient clinic in Kyoto university hospital, and gave some comments for this manuscript. TI was an assistant operator in the SAVE operation. MK is a chief operator in the SAVE operation. TN is a general supervisor of this manuscript.SB is an assistant professor and a pediatric cardiologist in charge in a pediatric ward of Kyoto university hospital. HD is an assistant professor and a pediatric cardiologist in charge in a pediatric ward and an outpatient clinic of Kyoto university hospital. TI is an associate professor in the department of cardiovascular surgery in Kyoto university hospital. MK is a previous professor of the department of cardiovascular surgery in Koyto university hospital. Now he works as a cardiovascular surgeon in Toyohashi heart center. TN is a professor of the pediatrics department in Kyoto university hospital. He is a supervisor of this manuscript.Written informed consent was obtained from this patient and her mother for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Muscular ventricular septal defects (mVSDs) account for approximately 10-15% of all VSDs. In addit3513In this article, we review the technique of device closure of mVSDs using the perventricular or hybrid approach off-pump without cardiopulmonary bypass (CPB). We discuss the indications, the selection criteria, the equipment required, and the potential complications encountered.Patients with significant mVSDs, who meet the criteria for closure of their defects, are considered for the perventricular or hybrid approach in the following conditions: (i) patients with contraindications for percutaneous device closure, which include small infants (<5.0 kg), in whom using large sheaths would be associated with significant morbidity, and patients with poor vascular access; (ii) patients with mVSDs and other cardiac anomalies requiring concomitant surgical repair ; (iii) patients with multiple or Swiss cheese VSDs in whom surgical repair would provide suboptimal results and in whom percutaneous closure is highly challenging; (iv) patients with multiple mVSDs who had previously undergone pulmonary artery (PA) banding until they gain enough weight to have their VSDs closed.Prior to the procedure, these patients should undergo detailed echocardiographic assessment of their defects. This provides essential information regarding the number, location, and size of the defects as well as any associated cardiac lesions. The initial assessment is by transthoracic echocardiography (TTE), where the four-chamber and parasternal long-axis and short-axis views provide adequate planes to determine the location of mVSDs. In particular, the parasternal short-axis view near the tip of the mitral valve well delineates the location of these defects: inlet defects appear between 7 and 9 o'clock, mid-muscular defects between 9 and 12 o'clock, and anterior defects between 12 and 1 o'clock.Additional imaging is usually performed intraoperatively by transesophageal echocardiography (TEE) to further plan and guide the procedure as detailed below. In some patients, the use of real-time 3-D echocardiography or cardiac CT imaging of the muscular septum, as part of the pre-evaluation procedure, can help demonstrate the exact morphology, size, and location of mVSDs. The applet al. in 1998 and first used in humans by Thanapoulos et al. in 1999. Thi Thiet alAfter full evaluation, the surgeon and the echocardiographer need to determine the best location for puncturing the RV free wall. This is done by the surgeon by tapping the fingers or a hemostat on the RV free wall under TEE guidance. The location should be chosen away from the papillary muscles and moderator band, but perpendicular to the septum . The surIn patients with multiple mVSDs without additional cardiac lesions requiring repair, it is sometimes advantageous to perform the procedure in the catheterization laboratory as, on occasions, it is difficult to cross other defects after deploying the first device. In such cases, combining steps from both percutaneous and perventricular techniques may be particularly helpful to close the remaining VSDs . PercutaThe main advantages of this technique are avoiding CPB in the absence of associated cardiac lesions, and reducing CPB time in patients with additional cardiac anomalies requiring surgical repair. This is particularly important in patients who had or might need long cross-clamp time for repair of associated lesions or those who already have evidence of myocardial dysfunction. The procedure only requires a minimal incision in patients without additional lesions. Other advantages include avoidance of any ventricular incisions and avoidance of transection of RV muscle bundles. Unlike the percutaneous approach, it is not limited by weight or need for vascular access: mVSDs were closed in infants weighing as less as 3.2 kg using this approach. It also In particular, this technique provides a major advantage to patients who present in the neonatal period for repair of associated cardiac lesions, such as newborns with coarctation of the aorta with significant mVSDs. This allows total repair in one setting for these patients. Using this technique, the indication for pulmonary artery banding for patients with multiple mVSDs would also be significantly decreased, and patients who had PA banding can have it debanded and the mVSDs closed intraoperatively.The technique allows the immediate confirmation of adequate closure since it is done under TEE guidance, and any additional mVSDs can be easily detected and closed in the same sitting.Initial attempts at intraoperative device closure of mVSDs in patients, in whom percutaneous closure was contraindicated , had unsatisfactory results with mortality and failure rates as high as 14-25% and 20-40%, respectively.–24 Theseet al.[The first successful case of intraoperative perventricular mVSD device closure without CPB on a beating heart was reported in a baby in 1998 by Amin et al. Few seriet al.–29 TableAs mentioned above, detailed echocardiographic (TTE and TEE) evaluation of the defect and associated structures is a major step in planning the perventricular closure. Once planned appropriately, the procedure itself is significantly less complex than the percutaneous approach, as it involves fewer steps and less extensive manipulation of wires, catheters, and sheaths. To date, no significant major complications have been reported in the clinical studies involving the perventricular approach, when applied off-pump without CPB, despite the limited number of these studies. The interventionalist should, however, be meticulous in handling the equipment to avoid potential complications.Two major potential complications are cardiac perforation and device embolization. Perforation is a rare but serious complication of the percutaneous technique and could occur with the perventricular approach, although it has not been reported to date. It may occur when introducing the delivery sheath and its dilator across the VSD into the LV as the dilator is stiff enough to puncture the LV if pushed too far. This is preventable by making sure the delivery sheath is positioned in the LV cavity not too close to the LV free wall by monitoring its position with TEE.Device embolization is another major potential complication that could occur if the device is released prematurely or in an inappropriate position. The device can then embolize to the LV, ascending aorta, RV or pulmonary arteries. This has been reported in the literature in one case, in which the mVSD size was underestimated by TEE, resulting in the use of a small device that embolized into the LV cavity. This mayAnother potential challenge that might result in unsuccessful closure is the inability to deploy the RV disk of the device, when there is an apical mVSD and there are heavy RV trabeculations.2728 This27Valvular regurgitation can be a serious complication if the device impinges on the valve apparatus. This may potentially occur in some challenging mVSDs, such as high posterior mVSDs close to the atrioventricular valves. Thus, it is essential to measure the distance from the defect to the various valvar structures in order to select the appropriate device size and to monitor valve function by TEE prior to device release. Minimal valve regurgitation that is not hemodynamically significant can be followed up medically; however, if the device causes significant impingement on the valve apparatus after release, it should be removed surgically.Hemolysis has been rarely reported with percutaneous device closure of mVSDs and could potentially occur with the perventricular technique, as it results from residual shunting. By usingAir embolism is another serious complication. As in the percutaneous approach, the device should be loaded onto the delivery cable under water or blood seal to prevent such rare complications.Conduction disturbances can be seen after mVSD device closure. Both right bundle branch block and complete heart block have been reported after percutaneous mVSD device closure, althoughPerventricular device closure of mVSDs is effective, safe, and provides a true hybrid approach to a challenging lesion by combining interventional and surgical techniques. Further experience with this technique will probably make it the approach of choice for small infants with significant mVSDs, patients with associated cardiac lesions undergoing surgical repair, and those with poor vascular access. Refinements of this technique might even expand its application to closing paramembranous VSDs in the future.


Heart failure is one of the most common, costly, disabling and deadly diseases. During the last decade, several different indices reflecting renal function such as creatinine-based glomerular filtration rate, circulating levels of cystatin C and low-grade albuminuria have been demonstrated to be independent risk factors for heart failure. This review summarizes our current knowledge of the relationship between diminished renal function and the incidence of heart failure in the community, and also in individuals with increased risk of heart failure such as patients with overt cardiovascular disease, hypertension or diabetes. This review will also put forward important areas of future research in this field. It 1) -9. A rec1)  showed tThe temporal relationship between heart failure and renal failure appears to be complex, however, and the opposite chain of events is also common: patients with chronic kidney disease are at substantially higher risk of developing cardiovascular disease and heart failure. In fact, heart failure is about 15 times more common in patients with chronic kidney disease than in patients with normal renal function  and indiAlthough the close relationship between cardiovascular and renal pathologies is well recognized in advanced nephropathy and heart failure, it has received less attention in early disease. To date, several studies have suggested that diminished renal function may be involved in the development of heart failure at an early stage, prior to the development of clinically overt heart failure and kidney disease. The present review will focus on studies that have reported the longitudinal relationship between different indices of renal function  and the development of heart failure in individuals without heart failure at baseline.The serum levels of creatinine and creatinine-based equations of glomerular filtration rate have been used extensively in clinical practice, as well as in research, as indirect estimates of glomerular filtration rate. Although widely used, creatinine-based equations of glomerular filtration rate such as Cockroft-Gault  or the MThe association between elevated serum levels of creatinine and the incidence of heart failure in the community was first reported by Gottdiener and co-workers in the Cardiovascular Health Study . In thatSerum creatinine levels as a predictor of heart failure has also been evaluated in individuals with high risk of heart failure such as patients with prevalent cardiovascular disease, hypertension, and/or diabetes. In most of these studies -23, howeIn a report from the Heart and Estrogen/Progestin Replacement Study (HERS), the primary aim was to identify predictors of heart failure in post-menopausal women with coronary heart disease . BibbinsCirculating cystatin C concentration is a relatively novel measure of kidney function that has been suggested to overcome many of the limitations of using creatinine-based estimates, as the relationship between kidney function and cystatin C concentration does not appear to vary with age, sex, and body mass , 27, 28.The association between serum cystatin C and development of heart failure in the community was first reported by Sarnak and co-workers in the Cardiovascular Health Study . Serum cIn secondary analyses in the Cardiovascular Health study, slightly stronger associations between cystatin C levels and heart failure were seen in blacks as compared to whites . The queIn a recent report from the Physicians’ Health study, Djousse and co-workers demonstrated that higher levels of cystatin C were associated with an increased risk of heart failure and that this association was evident in hypertensive individuals only . This reOne major limitation of most studies investigating the relationship between renal impairment and incidence of heart failure has been the lack of differentiation between systolic and diastolic heart failure. This important issue was recently addressed by Moran and co-workers in another report from the Cardiovascular Health Study  In that Several previous studies have indicated that elevated cystatin C levels are predictive of cardiovascular morbidity and mortality in patients with prevalent coronary heart disease , 38, yetAlthough low-grade albuminuria has traditionally been considered to be an indicator of early renal pathology, there is an emerging view that albuminuria is also a marker of generalized vascular pathology such as endothelial dysfunction (the STENO hypothesis). Many prThere have been a few community-based studies investigating the relationship between albuminuria and incidence of heart failure. In a study by Kistorp and co-workers , an incrIn contrast, Ingelsson et al. reported that the urinary albumin excretion rate predicted heart failure incidence independently of both established risk factors for heart failure and other indices of renal function (creatinine-based glomerular filtration rate (MDRD) and serum cystatin C) in a community-based sample of elderly men . FurtherSeveral authors have reported an association between albuminuria and heart failure in patients with diabetes. For instance, in the Appropriate Blood Pressure Control in Diabetes (ABCD) trial, patients with type 2 diabetes and macroalbuminuria were found to have a greater than threefold risk of heart failure as compared to diabetics with normo- or microalbuminuria . MoreoveThe predictive importance of low-grade albuminuria in hypertensive individuals with left ventricular hypertrophy was shown in the LIFE study , where aIt is important to note that although there are convincing data to show that low-grade albuminuria is an independent predictor of heart failure, this association should not necessarily be construed to imply that there is a direct benefit in lowering of the albuminuria level. This essential issue was addressed in the landmark study by deZeeuw and co-workers in a report from the RENAAL trial. In that study, low-grade albuminuria was found to be an independent predictor of future heart failure in individuals with diabetic nephropathy, but more importantly, a 50% reduction in albuminuria after 6 months of antihypertensive treatment was associated with a 27% reduction in heart failure risk, supporting the idea of lowering of albuminuria as a treatment goal in order to achieve cardiovascular protection .Even though several previous studies have shown that indices of glomerular filtration rate and albuminuria predict heart failure independently of each other, the conjoint effect of glomerular filtration rate and albuminuria for the prediction of heart failure is less reported. In a secondary analysis in the Cholesterol And Recurrent Events (CARE) trial , myocardDespite the relatively large number of studies, several important issues regarding the prognostic value of the indices of renal dysfunction and the development of heart failure remain unresolved. First, there have been no studies evaluating whether adding the indices of diminished renal function to a model with established risk factors for heart failure will improve the model discrimination, calibration, and global model fit for the prediction of heart failure -54. It iThere is a considerable body of data to support the notion that diminished renal function, as evaluated by reduced glomerular filtration rate or low-grade albuminuria, is an independent risk factor for subsequent heart failure both in high risk populations such as patients with overt cardiovascular disease, hypertension or diabetes, and in the community. Cystatin C appears to be a superior marker for future risk of heart failure compared to serum levels of creatinine or creatinine-based equations of glomerular filtration rate. Moreover, the risk of heart failure appears to begin at levels of glomerular filtration rate and albuminuria that are considered to be in the normal range according to current clinical practice. Some data suggest that the relative importance of renal dysfunction for the development of heart failure is different in different ethnic groups, an issue that merits further studies. Thus, based on the studies presented in this review, the close association between renal and cardiac dysfunction already appears to be present before the development of clinically overt renal and cardiovascular disease, an observation that challenges the current definition of cardiorenal syndrome .


Temporary epicardial pacing is commonly used for the diagnosis and treatment of arrhythmias in the acute post operative period after surgery for congenital heart disease. Temporary epicardial pacemakers have become increasingly sophisticated over the years and have evolved from simple single chamber devices with few programmable parameters to complex dual chamber devices capable of adjustable parameters similar to permanent pacemakers. This review will describe the various indications for temporary pacing, technical considerations for both the choice of pacing wires and pacemaker modes, complications with temporary pacing and our current practice with temporary pacing. Postoperative arrhythmias are a major cause of mortality and morbidity after cardiac surgery for congenital heart disease (CHD). Patients2Atrial pacemaker wires can be used to create an atrial electrogram (AEG) with augmented P waves. The recording is done by connecting the atrial pacemaker wires to the left and right arm leads. This creates a bipolar AEG in lead I and a unipolar AEG in the remainder of the limb leads. An alternative option is to attach the pacing lead to one of the precordial chest leads.Diagnostic AEGs are useful when P waves are not clearly visible on a surface electrocardiogram (ECG) . They alSinus node dysfunction, junctional ectopic tachycardia, supraventricular tachycardia , and atrioventricular block are common postoperative arrhythmias3 TemporaIn patients with junctional ectopic tachycardia, temporary dual chamber pacemakers can be used to establish AV synchrony by pacing the atrium at rates faster than the junctional rate.Sinus node dysfunction and high grade heart block are not so uncommon complications of surgery for CHD. Over half these patients with conduction abnormalities will have normalization of conduction within 10 days from cardiac surgery. Temporary epicardial pacing can be used to bridge this interval between post operative heart block and either recovery of spontaneous conduction or placement of a permanent pacemaker. In extremely low-birth-weight infants with complete heart block, patient size may preclude implantation of a permanent pacemaker. Extrathoracic temporary epicardial pacing may provide support to these patients for up to three months at which time permanent, epicardial-pacing leads can be implanted.et al, evaluated the effect of optimizing temporary dual chamber pacing in patients after surgery for congenital heart disease and demonstrated several techniques of individually optimized temporary dual chamber pacing to improve hemodynamics  by optimizing AV synchrony and/or synchronous ventricular contraction.[Disturbance of normal AV synchrony and dyssynchronous ventricular contraction may be deleterious in patients with CHD and compromised hemodynamics. Janousek traction.The pacing mode is classified using the Heart Rhythm Society and British Pacing and Electrophysiology Group generic code .7] The  The 7] TThe single-chamber external pulse generators are easy to use and can be programmed in the VVI, VOO, AAI, or AOO mode. They also have an increased sensitivity range (0.5 to 20 mV) to provide increased ability to sense P- and R-waves over other devices. Modern day dual chamber temporary pacemakers allow adjustment of parameters such as mode of pacing, lower and upper rate limits, AV interval, and post ventricular atrial refractory period. The most important of these are rate, atrial output and ventricular output . Making appropriate adjustments of these three parameters will provide effective pacing in most clinical situations. Changing the pacing rate automatically adjusts other dual-chamber temporary pacing parameters. Pediatric patients may need a pacing rate up to 200 ppm. A patient's high stimulation threshold may require a ventricular output of 25 mA. For managing atrial tachyarrhythmias, a rapid atrial pacing rate up to 800 ppm is possible but rates beyond 400/min are rarely used.The optimal sensing and pacing parameters need to be adjusted based on the thresholds. The capture threshold is the minimum pacemaker output (current intensity measured as voltage or amperage) required to stimulate an action potential in the myocardium. Only the current amplitude (volts/amperes) is altered in measuring threshold since temporary pacemakers come with a fixed current duration . This should be checked on a daily basis especially in patients who are dependent on the temporary pacing. Typical default settings are to set the voltage outputs at twice threshold in both atrium and ventricle to allow for a margin of safety. This however may not be possible if the capture threshold is > 10 mA. The sensing threshold is the minimum current the pacemaker is able to sense. It is generally recommended to set the sensitivity at half the minimum current sensed. If sensing is set too high (insensitive) this will make the pacemaker fail to sense intrinsic events, thereby converting it from a demand to a fixed-rate pacemaker. Setting the sensitivity too low (over-sensitive) will make the pacemaker potentially sense muscle noise, and other ambient events common to the intensive care situation  as intrinsic events leading to inappropriate loss of pacing. The upper and lower rate limits and AV intervals vary with age and should be set to maximize cardiac output within the range of age appropriate physiological parameters. In general, the AV delay is set between 100-140 ms in most children.Newer applications of temporary pacemakers include their use for the simultaneous pacing of both ventricles. Both ventricles can be simultaneously paced by placing a right and a left ventricular lead at implant and connecting both wires to the same output terminal of the pulse generator. There is evidence to show that biventricular pacing can augment cardiac performance in patients after repair of CHD and that this form of pacing may be better than conventional RV pacing in patients with normal interventricular conduction.–10 On a Epicardial wires come in two forms: unipolar and bipolar. A unipolar system  consistsTemporary epicardial pacing leads are typically placed in the operating room after the cardiac procedure is completed and before chest closure. Epicardial pacing wires were historically placed only on the right ventricle. This allowed for management of severe bradyarrhythmias with a more stable ventricular rate but at the expense of AV synchrony. As patients with CHD are often dependent on the atrial contraction for better preload and cardiac output, most institutions currently place both atrial and ventricular pacing wires. The atrial wires are placed on the right atrium.By informal convention, wires placed on the right atrium are brought out through the skin on the right of the sternum, and those on the right ventricle are brought out on the left of the sternum. The wire connected to the negative terminal is marked by color coding.An implanted pacing lead forms a direct current path to the myocardium. During pacing lead insertion and testing, only battery-powered equipment specially designed for this purpose should be used to protect against fibrillation that may be caused by alternating currents. Line-powered equipment used in the vicinity of the patient must be properly grounded. Pacing lead connector pins must be insulated from any leakage currents that may arise from line-powered equipment. Care should be taken to avoid the possibility of unintentional contact between the pacing lead(s), including extension cable, and any equipment used as well as any conductive surface contact.Although both atrial and ventricular temporary epicardial leads are reliable for short-term use, their function deteriorates on a daily basis. These leads are intended for temporary atrial and ventricular pacing and sensing for contemplated implant duration of 7 days or less. Increase in stimulation threshold typically occurs after 4 days in both the atrial and ventricular wires. The reas12There is a small but definite risk of epicardial pacing. Complications of epicardial wires include infection, myocardial damage, ventricular arrhythmias, perforation, and tamponade.15 While The use of MRI is currently not recommended in patients dependent on temporary epicardial pacing because of the potential risk of precipitating an arrhythmia or causiIn certain cases where epicardial pacing wires are not available or fail to function, alternative means of pacing need to be considered. These include pacing through transcutaneous patches, a transvenous lead or a transesophageal lead. Transcutaneous patches are readily available in the intensive care units and can be used to initiate asynchronous ventricular pacing in a relatively short period of time. Transcutaneous pacing has many limitations including making the patient extremely uncomfortable, requiring high energies for capture, and is generally not recommended for periods over 24 hours. A transvenous pacing lead can be inserted using fluoroscopic guidance and typically used to stimulate ventricular depolarization. Although more reliable than the transcutaneous patches, there is a risk of lead dislodgement and ventricular perforation Transesophageal pacing is used for brief bursts to overdrive the atrium in order to terminate atrial tachycardias. Ventricular capture is not possible and hence this form of pacing is not useful for heart block. There is also the risk of esophageal damage when used for longer periods of pacing at high output. There haOur current practice involves placement of atrial and ventricular unipolar pediatric pacing wires in all patients undergoing surgery for CHD who have undergone cardiopulmonary bypass. The risk of arrhythmias is considered low in patients that do not undergo cardiopulmonary bypass and hence temporary pacing wires are routinely not placed in these patients. Temporary pacing is undertaken to treat dysrhythmias and to improve hemodynamics in select patients with heart failure. Patients with complete heart block are paced using the temporary wires for 10-14 days for their underlying rhythm to return before placing a permanent pacemaker. The temporary epicardial leads are removed just before the patient is discharged from the intensive care unit.et al,[Fishberger et al, have rec


The study aimed at defining reference values for electrocardiographic (ECG) and echocardiographic parameters as well as macroscopic dimensions of the heart and microscopic dimensions of cardiomyocytes in the European brown hare. The studies were conducted on 30 adult, clinically healthy hares of either sex caught in Poland. ECG and echocardiography were performed supravitally on anaesthetized hares. After euthanasia, gross and microscopic myocardial and cardiomyocyte dimensions were determined. Heart rate amounted to 140 ± 37.5 beats/min, the leading rhythm involved the sinus rhythm. P wave time was 26 ± 5 ms, PQ time was 80 ms, QRS time was 29 ± 3.5 ms, and ST was 97.5 ± 7 ms. Echocardiography determined a left ventricular wall end-diastolic diameter of 8.6 ± 2.0 mm and an intraventricular septum end-diastolic diameter of 5.75 ± 1.0 mm. The thickness of the interventricular septum corresponded to that of the free wall of the left ventricle, a finding consistent with physiological hypertrophy. Preliminary reference values were established for echocardiography. The findings were similar to those obtained at necropsy. The ECG and echocardiographic studies represent the first supravital examination of cardiac function in the hare. The obtained results illustrate adaptation of hare's myocardium to its mode of life. The cardiac findings resemble the athlete's heart syndrome described in humans. The findings may prove useful in further studies on the physiology of the cardio-vascular system in the hare. Lepus europaeus) have focused on organ morphology, blood biochemical parameters, methods of blood sampling, coagulation parameters and cardiovascular disorders [Studies on the physiology of the European brown hare  3.2 ± 0.54 kg), clinically healthy hares  out of 96 hares caught in south-eastern Poland. The 30 hares were selected randomly among the 96 hares by selecting every third clinically normal hare. Two hares were omitted from the sampling population due to low age and low BW, respectively. Examination of cardiac morphometry was done in 42 hares, including the 30 hares mentioned above, 8 hares euthanatized due to injuries to extremities, which had developed during transport and 4 hares, which died during the transport. The hares were euthanatized by phenobarbitaladministered intracardially.nd Local Ethical Commission, No.87/2006 . ECG and echocardiography were conducted following anesthesia by a mixture of xylazine  3 mg/kg BW and ketamine  10 mg/kg BW, administered intramuscularly.The studies obtained consent of the 2ECG was conducted on animals positioned on their right flank, using a three-channel Sheiler AT-1 apparatus at the pass of 50 mm/s. On extremities the electrodes were placed in line with the generally accepted standards for small animals Fig. . The meaThe echocardiographic examination was performed using an Aloka 8000 apparatus equipped with a 7.5–10 Mzh head. Left ventricular end-systolic diameter, left ventricular end-diastolic diameter (LVEDd), left ventricular wall end-diastolic diameter (LWDd) and left ventricular wall end-systolic diameter in diastole as well as intraventricular septum end-diastolic diameter (IVSDd) and intraventricular septum end-systolic diameter were measured. The measurements were taken in parasternal projection in the short axis, from the right hand side, and the probe was placed in the third and fourth intercostal space above the sternum [Post mortem examination of the cardiac morphometry included measurements of height and width of right and left atrium, right and left ventricle, thickness of the interventricular septum and of the free wall in the left and right ventricle below the atrio-ventricular valves (similar to the site of measurement in echocardiographic examinations), and diameters of atrioventricular and arterial ostia. The locations are illustrated in additional file versus euthanasia).Tissue specimens of left ventricular free wall myocardium were fixed for 24 h in buffered 7% formalin, prepared by routine methods for histology, embedded in paraffin and sectioned at 4 μm. Sections were stained by hematoxylin and eosin and subjected to computer-assisted image analysis and morphometric measurements in a setup consisting of a computer connected to an Axiophot optical microscope (Carl Zeiss) equipped with a camera . The entire set had the potential of recording images and of their digital analysis. The measurements took advantage of MultiScaneBase V 14.02 p software, working in Windows environment. In every of 4 sections 10 optical fields (115 × 150 μm) were examined at 40× magnification. The morphometric analysis included measurements based on transverse as well as cross sections and included number of fibers per optical fields, diameter of cardiomyocytes and length and diameter of cardiomyocyte nuclei  transverse dimension, b) longitudinal dimension, c) right ventricle diameter, d) intraventricular septum diameter and e) left ventricle diameter.Click here for fileMicrographs showing the way cardiomyocytes were measured. a) cross-section and b) longitudinal section. Hematoxylin and eosin. Obj. ×40Click here for file


Premature ventricular contractions (PVCs) and non-sustained ventricular tachycardia (NSVT) are frequently encountered and a marker of electrocardiomyopathy. In some instances, they increase the risk for sustained ventricular tachycardia, ventricular fibrillation, and sudden cardiac death. While often associated with a primary cardiomyopathy, they have also been known to cause tachycardia-induced cardiomyopathy in patients without preceding structural heart disease. Medical therapy including beta-blockers and class III anti-arrhythmic agents can be effective while implantable cardiac defibrillators (ICD) are indicated in certain patients. Radiofrequency ablation (RFA) is the preferred, definitive treatment in those patients that improve with anti-arrhythmic therapy, have tachycardia-induced cardiomyopathy, or have certain subtypes of PVCs/NSVT. We present a review of PVCs and NSVT coupled with case presentations on RFA of fascicular ventricular tachycardia, left-ventricular outflow tract ventricular tachycardia, and Purkinje arrhythmia leading to polymorphic ventricular tachycardia. Heart disease remains the leading cause of death in the United States .  Most oThe presence of premature ventricular contractions (PVCs) and non-sustained ventricular tachycardia (NSVT) can be a marker of electrocardiomyopathy. While often considered benign, these types of ventricular ectopy can have negative consequences through an increased risk of SCD, underlying cardiomyopathy, or induction of cardiomyopathy. We discuss the relationship of PVCs and NSVT to SCD and cardiomyopathy, risk stratification strategies, and approaches to management including common scenarios for ablation.The estimated incidence of SCD in the United States is 300,000 deaths annually . CommonlThe overall incidence of SCD appears to be decreasing . EvidencSince our currently identified risk factors fail to account for much of the population at risk for SCD and the incidence of SCD may be increasing in patients without ischemic heart disease, we must refocus our attention on this group, thus the need to identify new risk factors to capture a larger segment of those at risk.  Furthermore, we must recognize clues suggesting subtle electrocardiomyopathy.PVCs are early depolarizations originating in the ventricle due to increased automaticity. NSVT occurs when three or more consecutive PVCs occur at a rate greater than 100 beats-per-minute . They maThe incidence of PVCs and NSVT in the adult population varies. Ambulatory 24-hour ECG monitoring in male medical students identified PVCs in 50% of the cohort, but only 2% had more than 50 PVCs in 24 hours . A FramiThe presentation of PVCs and NSVT varies and may include palpitations, chest pain, pre-syncope, syncope, or heart failure. Dysrhythmias occur with increased automaticity, reentry, or triggered automaticity.  PVCs arise from tissue with increased automaticity. This is influenced by autonomic tone, ischemia/reperfusion, systemic abnormalities such as electrolyte disturbances, and cardiotoxic factors . MonomorThere are three major concerns regarding the presence of PVCs and NSVT. The substrate often associated with monomorphic VT increases the risk for sustained VT. Another concern, particularly with increased automaticity and polymorphic VT, is that a ventricular beat may be coupled closely with the preceding QRS complex and produce ventricular fibrillation. In addition, frequent ventricular ectopy is known to adversely affect cardiac function itself, namely tachycardia-induced cardiomyopathy.It can be difficult to determine whether a primary cardiomyopathy is present resulting in ventricular ectopy or a primary arrhythmia causing tachycardia-induced cardiomyopathy  9]. Whi. Whi9]. Certain factors are helpful in determining the primary disorder  9]. Clu. Clu9]. An evaluation of patients with PVCs and NSVT should determine the extent of symptoms, evidence of underlying cardiomyopathy, the risk for tachycardia-induced cardiomyopathy, and the risk for sudden cardiac death. This begins with a focused history including associated symptoms, frequency, duration, triggers, family history, and risk factors for coronary artery disease. The physical exam should evaluate for evidence of systemic disease .Routine tests include a resting ECG, 24-hour ambulatory electrocardiography, and an echocardiogram. The ambulatory electrocardiogram can establish the frequency, duration, and morphology of ectopy. Early repolarization on electrocardiogram is prognostic of increased risk of death from cardiac causes . The echNoninvasive and invasive methods of risk stratification for SCD are available. The most recognized risk factor is diminished left ventricular function as determined by the ejection fraction. This, however, fails to account for the majority of SCD cases . EvaluatICDs are recognized as the principal method of preventing sudden cardiac death in the highest-risk population. The AVID study in patients with history of sudden cardiac arrest (secondary prevention) showed a reduction of overall mortality and SCD incidence with ICDs as compared with amiodarone . MADIT IPVCs and NSVT increase the risk for sudden cardiac death. There is, however, little evidence evaluating the efficacy of ICDs in this population. The MADIT I study showed a survival benefit with ICD therapy versus conventional therapy in patients with asymptomatic NSVT and symptomatic ischemic cardiomyopathy with an ejection fraction of less than 35% . The DEFEconomic constraints and quality of life implications must be entertained when considering ICD therapy. A reduction in the incidence of sudden cardiac death may not equate to survival with meaningful quality of life . FurtherThe goal of therapy for PVCs and NSVT is to 1) alleviate symptoms; 2) suppress ventricular tachyarrhythmias and PVCs; 3) prevent or reverse tachycardia-induced cardiomyopathy; and 4) decrease shocks in patients with ICDs. Lifestyle modifications such as reducing caffeine and alcohol intake can be recommended initially, although neither moderate use nor reduction in intake has a major effect on ventricular ectopy ,17.Beta-blockers can be considered for treatment of ventricular tachyarrhythmias. They decrease mortality in patients post-myocardial infarction  and in hThe role of antiarrhythmic drugs in the management of PVCs and NSVT is best assessed by evaluating their efficacies versus side-effect profiles, including the propensity for pro-arrhythmic effects. Selection of antiarrhythmic drugs may be empiric, guided by the use of electrophysiology study with stimulation testing, or guided by ambulatory/exercise ECG monitoring .Arrhythmia suppression with class I antiarrhythmic drugs is not advised due to the results of the CAST trial . AlthougClass III antiarrhythmic drugs have been evaluated in patients at risk for SCD. The Amiodarone Trials Meta-Analysis Investigators showed a 13% reduction in mortality and 29% reduction in SCD in patients with recent myocardial infarction or congestive heart failure treated with amiodarone versus placebo . The SCDAnother class III drug, dofetilide, has been studied in the setting of inducible sustained ventricular tachycardia. It appears to be as efficacious as sotalol and better tolerated, possibly due to less beta-blockade . DofetilGiven the pro-arrhythmic effects and adverse side effect profiles of most antiarrhythmic drugs, it is often impractical to use them for primary prevention of SCD. Nevertheless, in patients with structural heart disease and symptomatic or frequent ventricular ectopy, amiodarone or dofetilide could be used with caution. They may also be utilized short-term in the setting of suspected tachycardia-induced cardiomyopathy to evaluate for improvement of ejection fraction that might be sustained with radiofrequency ablation.RFA is an additional management option for PVCs and NSVT.  Determining the target site for ablation depends on the mechanism of the arrhythmia. Activation mapping is used with PVCs to identify the earliest activation point, representing the automatic tissue targeted for ablation. Alternatively, pace mapping can be utilized to find the paced site that matches spontaneous premature ventricular depolarization . Common RFA has been successful in alleviating symptomatic ventricular ectopy , reducinMonomorphic ectopic complexes strongly suggest a primary arrhythmic propensity as seen in the following illustrative case. A 19 year-old female presented with fatigue and dyspnea on exertion. Her electrocardiogram  demonstrShe had a reduced left ventricular ejection fraction (32%) without evidence of obstructive coronary disease or anomalous anatomy. High dose beta-blocker therapy was poorly tolerated and ineffective at controlling her PVCs. Similarly, sotalol 120 mg by mouth twice daily caused fatigue and only minimal reduction in her PVCs.Since medical therapy was not effective for her, she was referred for an EP study. The morphology of her PVCs suggested an outflow tract origin given they were strongly positive in the inferior leads  and strongly negative in aVL and aVR.  The right bundle branch morphology (positive in V1) and S wave in lead I suggested a left-sided focus such as from the left ventricular outflow tract (LVOT) [38]. During the EP study, a lasso catheter was placed in the LVOT where the focus was localized based on activation at the Sinus of Valsalva . She hadThe outflow tracts are common sources of PVCs and have classic ECG patterns. The key electrocardiographic findings for an LVOT focus are illustrated above. An RVOT focus similarly has strongly inferior vector  but with a left bundle branch block pattern (positive in V1). Arrhymothogenic right ventricular cardiomyopathy  is imporFascicular ventricular tachycardia  is a speThese two cases examples demonstrate that primary arrhythmias may develop in patients who do not have a traditional cardiomyopathy. The astute clinician is mindful of the uncommon cardiomyopathies manifesting rhythm abnormalities.Polymorphic ventricular tachycardia can, in specific circumstances, be treated by ablation. A 31 year-old female collapsed after exercising and had a second episode while shopping. Polymorphic ventricular tachycardia / ventricular fibrillation was diagnosed, and her electrocardiogram had a borderline QT interval. She had no structural heart disease by echocardiogram and she did not provoke any evidence of arrhythmia during an exercise test. An ambulatory Holter monitor demonstrated repeated runs of minimally-symptomatic polymorphic VT with a normal QT interval. An ICD was implanted and subsequent interrogation demonstrated similar episodes with frequent detections and shocks. EP study found Purkinje potentials preceding PVCs  that indPVCs and NSVT are frequently encountered in clinical practice. Their presence may increase the risk for more malignant dysrhythmias such as sustained VT or ventricular fibrillation that can cause sudden cardiac death. PVCs and NSVT can be associated with a reversible tachycardia-induced cardiomyopathy or a primary cardiomyopathy. A focused evaluation and implementation of risk stratification strategies are helpful in treatment selection. RFA is effective in alleviating symptoms and reducing ICD shocks, especially in the setting of outflow tract or fascicular tachycardia. The presented cases highlight key points in the approach to RFA for PVCs and NSVT.


Atrial fibrillation is a multivariable disease  . It is g39 year healthy male with 20 year history of atrial fibrillation (AF).  Left ventricular systolic function was reduced (LVEF 30%). Despite long-standing AF and structural heart disease pulmonary vein isolation resulted in excellent benefit with subsequent normalization of EF (56% at 3 months follow up) .52-year-old female with diabetes, obesity and paroxysmal AF.  Systolic ventricular function was normal. Radiofrequency ablation was required to isolate the pulmonary veins, as well as additional focal sources of arrhythmia in the superior vena cava and coronary sinus .2). Despite pulmonary vein isolation multiple atrial flutters were noted necessitating extensive liner ablation with eventual control that required pace termination and adjunctive medical therapy . Despite wide-area circumferential ablation around the pulmonary veins and adjunctive medical therapy a modified right and left sided endocardial maze procedure control of arrhythmia has been suboptimal with subsequent requirement of permanent pacemaker implantation (56-year-old with chronic atrial fibrillation restrictive cardiomyopathy and moderate tricuspid regurgitation. Echo exam showed severe diastolic dysfunction and marked atrial enlargement (LAVI 73 ml/mantation .A collage of four cases demonstrating a spectrum of correlation between echo Doppler variables and intracardiac electrograms that accurately defined the electropathy and predicted outcomes of ablation are presented.  Further studies will help define whether echo based assessment of diastolic function will help guide patient choice for invasive electrophysiology procedures.


USG performed with a high-end machine, using a good cine-loop facility is extremely helpful in the diagnosis of fetal cardiac anomalies. In fetal echocardiography, the four-chamber view and the outflow-tract view are used to diagnose cardiac anomalies. The most important objective during a targeted anomaly scan is to identify those cases that need a dedicated fetal echocardiogram. Associated truncal and chromosomal anomalies need to be identified. This review shows how fetal echocardiography, apart from identifying structural defects in the fetal heart, can be used to look at rhythm abnormalities and other functional aspects of the fetal heart. Congenital heart disease (CHD) is a common anomaly in newborns. Improvements in the antenatal diagnosis of cardiac anomalies have resulted in a significant reduction in neonatal morbidity and mortality.2 With eaWith improvement in operator skills and the availability of high-end machines with newer imaging techniques like spatio-temporal image correlation (STIC), multiplanar imaging, X-plane, full-volume fetal echo, and tissue Doppler imaging, the pick-up rate for cardiac anomalies has increased significantly.The incidence of CHD is 8 per 1000 live births.5 HoweverIf a previous child was born with a CHD, the probability of a subsequent child being born with a CHD is 1:20 to 1:100.If two previous children were born with CHD, the risk is 1:10 to 1:20.If the mother has CHD, the risk is as high as 1:5 to 1:20If the father has CHD, the risk is 1:30Cardiac anomalies are known to cluster in families; the risk of having a child with a cardiac anomaly is as follows:DiabetesAutoimmune disorders like systemic lupus erythematosus (SLE) or Sjogren's syndromeUse of drugs, e.g., antiepileptics or antipsychotics like lithium, etc.CHD in the motherHistory of a sibling with a cardiac anomalyProminent nuchal translucency or increased nuchal fold thicknessStructural defect in other systemsFetal infectionsIUGR in the mid-trimesterChamber asymmetryAltered cardiac axisAltered position of the fetal heartEnlarged fetal heartArrhythmiaThe fetal heart can be evaluated at any time during the gestation period when USG is done. In the first trimester (11–14 weeks), cardiac details may not be elicited well, but the presence of a pulsatile ductus venosus or tricuspid regurgitation can be a very strong marker for cardiac and chromosomal anomalies.The fetal heart can be evaluated in the third trimester, but there are a lot of limitations due to oligoamnios and shadowing from the fetal spine, ribs, and limbs.The best time to evaluate the fetal heart is between 18–22 weeks gestation.The equipment used for performance of fetal echocardiography needs to have an excellent B-mode, with a good cine-loop facility so that one can scroll back frame by frame and capture the frame of interest. The spatial and temporal resolution needs to be good. The system should have color Doppler, pulsed Doppler, and continuous wave Doppler. STIC, tissue Doppler, and multiplanar imaging are added advantages.A special pre-set for fetal heart evaluation needs to be created with a high frame rate, decreased persistence, and increased compression. The system should have the ability to zoom the image without causing deterioration of image quality. A higher pulse repetition frequency (PRF) is required for color Doppler in the fetus as compared to the settings used for routine obstetric color Doppler.The heart may be completely or partially outside the thorax (ectopia cordis) . The posThe cardiac axis is the angle the interventricular septum makes with the anteroposterior diameter of the thorax. The normal cardiac axis is 45± 15°. The heart is normally deviated to the left. It is almost entirely within the left chest. An altered cardiac axis is a pointer toward an anomaly. Altered axis is often associated with outflow tract anomalies .In order to judge the situs of the fetus, it is essential to figure out which the left side of the fetus is by looking at its presentation and position. After this, one has to confirm that the fetal stomach is to the left. Once the abdominal situs is confirmed as being normal, the transducer is angled cephalad to see if the apex of the heart points to the same side as the stomach.The normal situs or situs solitus has all the left-sided landmarks, such as the stomach, spleen, the abdominal aorta, and the cardiac apex, to the left of fetus, while the liver and the inferior vena cava (IVC) are to the right of the fetus. A mirror image of this arrangement is called situs inversus. The incidence of cardiac anomalies in the presence of situs inversus is 2%.Any situs anomaly between these two ends of the spectrum can be labeled as a heterotaxy syndrome. These syndromes have a very high incidence of cardiac anomalies– up to 75–90%. In these patients, it is important to look for an interrupted IVC, presence of an azygos system, a midline liver, and splenic anomalies .must be seen and documented, and on each view, there are certain observations that must be made. These views are:There are certain views that Number of chambers. Normally four chambers are seen.Comparison of chamber sizes. Normally both atria are of the same size. The ventricles should also be identical in size, with no evidence of wall thickening.a Identification of chambers can be done on the basis of:b Position – the chamber closest to the spine is the left atrium and the most anterior chamber is the right ventricle.Morphological features – the echogenic moderator band is present in the right ventricle. The flap of the foramen ovale opens into the left atrium.The crux of the heart is formed by the membranous part of the ventricular septum, the septum primum of the atrial septum, and the septal leaflets of the mitral and tricuspid valves.The septal leaflet of the tricuspid valve is inserted into the septum closer to the apex than that of the mitral valve. This ‘offset’ is normally about 3 mm. Abnormalities of alignment of the valves could be a pointer towards atrioventricular septal defects.The ventricular septum has to be examined right from the apex to the crux for any defects. An echo drop-out is often seen in the interventricular septum, especially when the sound beam is parallel to the interventricular septum. In this case, an angle needs to be created between the interventricular septum and the sound beam, upon which this echo drop-out disappears; however, any change due to a ventricular septal defect (VSD) will persist. A true VSD generally has bright margins.The two atrioventricular valves—the mitral and tricuspid—should be identified. Opening and closing of these valves has to be evaluated in real time.Flow across the atrioventricular valves has to be examined with color Doppler. The direction of flow is from the atria to the ventricles. It is the same across both valves. No aliasing should be seen.Area behind the heart: only one vessel, the descending aorta, should be seen between the left atrium and the spine.This view is technically very easy to obtain. It is taken in the transverse section of the thorax. First, a good abdomen perimeter section is obtained; then the probe is slid cephalad to obtain the four-chamber view . There aTwo-chambered heart: This is a defect of septation. The septum that divides the primitive heart into the left and right sides fails to develop. There is a single atrium, a single ventricle, and a single AV valve. This condition is often also associated with a single outflow tract—the truncus. Chromosomal anomalies are known to be associated .Hypoplastic left heart : This isEbstein's anomaly : This isVentricular septal defects : The defAtrioventricular septal defect : This caThis view, also, is technically easy to obtain. From the position for a four-chamber view, the probe is slid cephalad for the three-vessel view. The three vessels seen on this view are the pulmonary artery in longitudinal section, seen anteriorly and to the left; the aorta in transverse section, seen in the center; and the superior vena cava (SVC) in transverse section, seen to the right.From the position for a four-chamber view, the probe is slid cephalad and rotated towards the left fetal shoulder to obtain the RVOT view. This reveals the RVOT in its long axis. The pulmonary valve motion can be appreciated within the RVOT. On rotation toward the left, the bifurcation of the pulmonary artery can be seen.Color and pulsed Doppler evaluation of the RVOT is done to look for aliasing at the pulmonary valve. The peak systolic velocity in the pulmonary artery can be obtained with pulsed Doppler.From the four-chamber view position, the transducer is angled towards the right shoulder of the fetus to visualize the LVOT. The LVOT view can also be opened from the lateral four-chamber view. In this view, 1) the septoaortic continuity needs to be confirmed and 2) the aortic valve motion has to be observed.The two outflow tracts cross each other at their origins. On color Doppler they reveal opposite color signals at the origin.The arch of aorta has to be visualized in a sagittal view. It is narrow and round. The great vessels arise from this arch. On color Doppler, the direction of flow can be seen to be from the ascending aorta to the arch and then to the descending aorta.The ductal arch is flat and wide. It can be seen by angulating the transducer anteriorly from the position for the arch of aorta section. The ductal arch starts anteriorly behind the sternum. On color Doppler, there is an area of aliasing in the arch; this corresponds to the area of the ductus arteriosus. The peak systolic velocity in the ductus arteriosus can be measured with pulsed Doppler.The venoatrial connections on the right side are visualized on a right parasagittal view. This is popularly known as the ‘hammock view.’ The SVC and IVC are seen to open into the right atrium. On the left side, the pulmonary veins are seen to open into the left atrium. These are best seen in a four-chamber view. Of the four pulmonary veins only two or three may be visualized. On color Doppler, the direction of flow is toward the left atrium.There is loss of septoaortic continuity. The aorta is seen to override the interventricular septum. There is turbulence at the root of the aorta on color Doppler. The RVOT is relatively narrow and reveals poor or no forward flow.Both the outflows are parallel to each other at the origin. They show similar color flows, indicating that the direction of flow in both the great vessels is the same.The right ventricle is large and reveals a moderator band. The left ventricle is rudimentary. Both outflow tracts are parallel to each other at the origin and are usually good sized. It is often associated with situs anomalies.The pulmonary artery is very narrow or may reveal poststenotic dilatation. On real-time echocardiography, the pulmonary valve is not seen to open adequately. On color Doppler, aliasing may be seen. On pulsed Doppler, very high velocities, over 180 cm/s, are noted in the main pulmonary artery.Rhythm abnormalities are generally picked up by the clinician. The radiologist first needs to rule out a structural anomaly. Then, with the help of M-mode and pulsed Doppler, the rhythm of the heart can be evaluated.The fetal heart rate varies from 120–160 beats per minute (BPM). A rate below 120 BPM is termed as bradycardia and above 200 BPM is termed as tachycardia. When the fetal heart beat is first visualized at close to 5 weeks of gestation, the rate may sometimes be about 90 BPM, but soon the rate picks up. The most common rhythm abnormality seen in practice is premature atrial contraction followed by a compensatory pause. Heart blocks of varying degrees may be seen. Supraventricular tachycardia is one area where fetal therapy has made a mark; maternal digitalization can make this arrhythmia revert back to normal.Size of the heart: the normal cardiothoracic ratio is 1:2Squeeze of the heart: this is most often a visual impression.Two general points should be assessed:Enlargement of the fetal heart and poor contractility are often seen in fetal hypoxia, i.e, in placental insufficiency . ImpaireSome lesions such as minor VSDs may be missed.Progressive defects, such as a bicuspid aortic valve, may not be diagnosed at 18–20 weeks of gestation.Outflow tract anomalies may be missed.Maternal habitus and fetal lie may be limitations.Visualization of details may not be possible before 18 weeks.To conclude, it may be difficult or time consuming to perform a dedicated fetal echocardiogram on all patients. However, it may be worthwhile looking at the four-chamber view, the outflow tracts, and the three-vessel view; this would be sufficient to diagnose 80–85% of cardiac anomalies.


Myotonic dystrophy is a genetic muscular disease that is frequently associated with cardiac arrhythmias. Bradyarrhythmias, such as sinus bradycardia and atrioventricular block, are more common than tachyarrhythmias. Rarely, previously undiagnosed patients with myotonic dystrophy initially present with a tachyarrhythmia. We describe the case of a 14-year-old boy, who was admitted to the hospital with clinical signs and symptoms of decompensated heart failure and severely reduced left ventricular function. Electrocardiography showed common-type atrial flutter with 2 : 1 conduction resulting in a heart rate of 160 bpm. Initiation of medical therapy for heart failure as well as electrical cardioversion led to a marked clinical improvement. Catheter ablation of atrial flutter was performed to prevent future cardiac decompensations and to prevent development of tachymyopathy. Left ventricular function normalized during followup. Genetic analysis confirmed the clinical suspicion of myotonic dystrophy as known in other family members in this case. Pediatric patients suffering from myotonic dystrophy frequently exhibit electrocardiographic abnormalities, most often sinus bradycardia and conduction disturbances , 2. ThesA 14-year-old boy was taken to the outpatient pediatric department after feeling sick following climbing, with vomiting and presyncope. He also complained of recurrent palpitations within the previous two weeks. The initial electrocardiogram showed common type atrial flutter with 2 : 1 conduction resulting in a heart rate of 160 bpm . The preUpon admission to the hospital, echocardiography showed severely reduced left ventricular function with fractional shortening of 14%, but no signs of a congenital heart defect. Clinical examination showed a facies myotonica. Initiation of beta-blocking agents (metoprolol succinate 23.75 mg) was unable to slow the heart rate sufficiently, so that electrical cardioversion was performed resulting in sinus tachycardia with 120 bpm. Further recompensation was achieved by adding diuretics (repetitive i.v. injections of 10 mg furosemide) and angiotensin converting enzyme inhibitors (captopril 3 × 6.25 mg). The PQ interval after cardioversion was within normal range (166 ms).Subsequently, catheter ablation of the arrhythmia was performed. A bidirectional block of the cavo-tricuspid isthmus was achieved without complications. Infra-Hisian conduction time was within normal range 52 ms, . VentricBecause of the family history, as well as an elevated creatin-kinase (1832 U/l), genetic analysis was performed which confirmed the suspicion of myotonic dystrophy of Curschmann-Steinert type 1. During followup, left ventricular function continuously improved  and no recurrence of the arrhythmia could be observed. Magnetic resonance imaging of the heart failed to show signs of pericarditis, myocarditis, or cardiac involvement of myotonic dystrophy, such as myocardial thickening or isolated left ventricular abnormal trabeculation .Arrhythmias, especially in the pediatric population, should prompt for a search for the underlying disease. In most cases, either a congenitally existing primary arrhythmogenic substrate (such as an accessory pathway) or sequelae of congenital heart disease, or its surgical correction can be identified as the cause. Interventional electrophysiological treatment can be performed in these patients with high success rates , 5. The Cardiac involvement is an integral part of Curschmann-Steinert's myotonic dystrophy, the most reported manifestations having various conduction defects , 2, 6. AWhile usually the neurological manifestations lead to the diagnosis of myotonic dystrophy, there are case reports of patients who initially present with symptoms of cardiac involvement . It is noteworthy that in our patient no signs of atrioventricular conduction delay were present in surface ECG, and the electrophysiology study showed normal conduction times.Common-type atrial flutter is a well-defined arrhythmia with a critical zone of slow conduction at the right atrial isthmus between the inferior vena cava and the inferior aspect of the tricuspid annulus. Catheter ablation is the therapy of choice in the adult population, and high success rates of this arrhythmia were also reported in the pediatric population , 5. The clinical course in the presented case with the improvement of cardiac function after rhythm control and the structurally normal heart on echocardiography and magnetic resonance tomography make it very likely that the rhythm disorder itself led to the clinical deterioration. It can be speculated that undetectable structural cardiac abnormalities linked to the underlying myotonic dystrophy (as described above) are the substrate for the rhythm disorder. Curative catheter ablation is an appropriate means of treatment and helps prevent future cardiac decompensations and the development of tachymyopathy . Cardiac arrhythmias in children without detectable structural cardiac abnormalities should be investigated for the presence of myopathy. Close cooperation of pediatric neurologists, pediatric cardiologists, and clinical electrophysiologists is necessary to offer best medical care.


Cardiac Resynchronization Therapy (CRT) leads to hemodynamic and clinical improvement in heart failure patients. The established methods to evaluate myocardial asynchrony analyze longitudinal and radial myocardial function. This study evaluates the new method of circumferential 2D-strain imaging in the prediction of the long-term response to CRT.38 heart failure patients  received CRT and echocardiographic evaluation with a mean follow-up of 9.4 months. 18 (47.4%) of the patients were hemodynamic responders to long-term CRT. In the responder group, the maximum delay in the circumferential 2D-strain in the basal segments decreased . In the non-responder group there was no significant change . This was paralleled by a reduction of the maximum delay in the radial and longitudinal 2D strain in the basal segments. In ROC analysis, the baseline delay of circumferential 2D strain (AUC 0.66 (± 0.14)) does not predict a long-term response to CRT (p = 0.37).There is a significant decrease in the circumferential 2D-strain derived delays after CRT, indicating that resynchronization induces improvement in all three dimensions of myocardial contraction. However, the resulting predictive values of 2D strain delays are not superior to longitudinal and radial 2D-strain or TDI delays. Cardiac resynchronisation therapy (CRT) is an effective therapy for advanced chronic heart failure. Randomized, controlled trials indicate that CRT improves hemodynamics, reverse remodelling, quality of life, hospitalisation and mortality -7. HowevIn this context, it is essential to select patients for CRT carefully and to define predictors. Several studies have focused on prospectively predicting successful CRT by echocardiography. However, only parameters of longitudinal and radial myocardial function were used ,10.There are conflicting data about the best predictor for hemodynamic and clinical improvement. Several echocardiographic (including 3D echocardiography) and clinical predictors were assessed recently -14.Myocardial contraction is a complex three-dimensional motion. Fibre architecture includes longitudinal, radial and circumferential oriented fibres. The majority of fibres have a longitudinal orientation. However, radial and circumferential fibres contribute to myocardial systolic function ,16.Circumferential myocardial function can be analysed by 2D ("speckle tracking") derived echocardiography. Unlike Tissue Doppler derived velocity and strain, 2D-derived analysis  is angleThe aim of this study is to examine the improvement of circumferential myocardial contractility after CRT is analysed.In this monocentric study, we included 38 heart failure patients (NYHA II-IV) with a LVEF < 0.35 and a QRS width > 120 ms. All patients received a CRT-device with automatic defibrillator (ICD) function and were fully documented by 2D and Tissue Doppler echocardiography. Follow-up was at least 6 months. The heart failure medication was unchanged three months prior to implantation of the CRT-ICD to obtain unbiased data regarding cardiac improvement after CRT.Successful resynchronization therapy was defined as a relative reduction of the left-ventricular end-systolic volume (LV-ESV) of more than 15% ,21 and aEchocardiography was performed by Vivid 5 and Vivid 7 . The images were stored digitally and analyzed off-line by EchoPac PC Dimension . For TDI and 2D echocardiography analysis, three beats were stored.The echocardiographic study design was described previously . The LVECircumferential 2D strain was measured in the parasternal short axis at the level of the papillary muscles. The endocardial border of the end-systole was traced manually. The following six segments were analysed: antero-septal, anterior, lateral, posterior, inferior, septal. The maximal delays in opposite segments  were then calculated. The maximum delay was then used for further analyses.The maximal longitudinal delays were obtained from apical and the maximal radial delays were measured in the parasternal short axis views.Written consent was obtained from each patient, and the ethics committee of the Charité University Hospital approved the protocol.2-test. The level of significance was p = 0.05.Statistics were calculated by SPSS . Results are expressed as mean (± standard deviation). Comparisons of parametric variables between the responders and the non-responders were calculated by paired Student's t-test. The comparison of echocardiographic parameters between groups was calculated by unpaired t-test. Dichotomized data were analyzed by the ChiEighteen (47.4%) of the 38 patients were hemodynamic responders to long-term CRT. The baseline characteristics in the responder and non-responder group did not differ significantly. Baseline characteristics see Table The hemodynamics of the responder and non-responder group: see Table See additional file 1) and a healthy control  are attached.The maximum delay in the circumferential, longitudinal and radial 2D-strain decreased significantly in the responder group but not in the non-responder group  curve analysis Figure  was perfIntra- and interobserver reliabilities were calculated by the intraclass correlation coefficient (ICC). There were good correlations for Tissue Doppler measurements  and circumferential (non-Doppler) 2 D strain measurements .The main finding of this study is that circumferential 2D Strain delays decrease significantly in the responders but not in the non-responders to CRT. The analysis of circumferential 2D strain broadens the view of three-dimensional myocardial motion and indicates that circumferential myocardial synchrony is improved by CRT.The majority of myocardial fibres have longitudinal orientation. Early in the development of heart failure, longitudinal function is impaired, with a compensation of radial and circumferential (= short axis) function . The proCircumferential asynchrony might play an important role in the measurement of overall asynchrony. A TAGGED-MRT-study found that asynchrony assessed by longitudinal motion is less sensitive, follows different time courses than circumferential asynchrony, and may manifest CRT benefit during specific cardiac phases depending on pacing mode. These results underscore the limitations of longitudinal analyses of asynchrony .There are technical issues of circumferential 2D Strain of importance. The rather low frame rate (50–100/min) can impair the detection of myocardial contraction peaks. Despite the low temporal resolution of 2D-Strain, significant changes in our study and of previous studies ,27,28 suThis is supported by a recent study by Becker . CircumfIn contrast to our results, Zhang  found anNeither circumferential nor radial and longitudinal 2D strain delays are able to predict CRT response. The 2 D Strain parameters are not superior to Tissue Doppler derived asynchrony. We conclude that no single parameter is able to help in patient selection for CRT.The future role of echocardiography in selection of CRT candidates is not yet resolved. The recently published consensus statement of the American Society of Echocardiography points out that echocardiographic selection of CRT candidates should only be performed in borderline cases with difficult decision making .The results from the CRT patients in our institution have led to the strategy currently used: an integration of seven parameters of inter- and intra-ventricular asynchrony in addition to the established parameters. If more than two parameters are indicative of myocardial asynchrony, a CRT indication supported by echocardiography is seen. We hypothesize that not single parameters of myocardial asynchrony but the integration of multiple echocardiographic parameters of asynchrony will facilitate the selection of CRT candidates.In conclusion, circumferential 2D Strain delays decrease significantly after CRT, indicating an improvement in all three dimensions of myocardial contraction. However, the resulting predictive values of 2D strain delays are not superior to longitudinal and radial 2D-strain or TDI delays.FK and SS have equally contributed to the study. FK, SS, ACB, AG, SE participated in contributions to conception, analysis and interpretation of data, the follow-up echocardiographic examinations and made comments to the manuscript. HB has implanted the CRT systems and has analyzed data. BG has supervised and commented the study. ACB was the supervisor of echo examinations, is head of the echo lab, contributed by revising the manuscript critically.Click here for fileClick here for file


Neuromuscular Diseases are a heterogeneous molecular, clinical and prognosis group. Progress has been achieved in the understanding and classification of these diseases.Cardiac involvement in neuromuscular diseases namely conduction disorders, ventricular dilatation and dilated cardiomyopathy with its impact on prognosis, is often dissociated from the peripheral myopathy. Therefore, close surveillance is mandatory in the affected patients. In this context, preventive therapy (beta-blockers and angiotensin converting enzyme inhibitors) has been recently recommended in the most common Neuromuscular Diseases, Duchenne Muscular Dystrophy and Myotonic Dystrophy.  During last years, progress has been achieved in the understanding and classification of neuromuscular diseases.1) in the neuromuscular diseases namely conduction disorders, ventricular dilatation and dilated cardiomyopathy with its impact on prognosis, is often dissociated from the peripheral myopathy. Therefore, close surveillance is mandatory in affected patients. In this context, preventive therapy has been recently recommended in the most common forms the Duchenne Muscular Dystrophy and the Myotonic Dystrophy.Cardiac involvement . In BMD, prognosis rather depends on the existence of a cardiomyopathy.Both diseases are X-linked recessive disorders due to the Dystrophine gene abnormality with almost absence of Dystrophine in DMD and reduced Dystrophine in BMD -2.DMD is the most common inherited neuromuscular disorder with an incidence of 30/100000 live male births. A cardiomyopathy is always present but is masked by severe muscle weakness. The electrocardiogram (ECG) is often abnormal. Death occurs by the age of 20 years old in DMD versus 40-50 years old in BMD [Angiotensin Converting Enzyme Inhibitors (ACEI) have been shown to be effective in preventing left ventricular (LV) remodeling in ischemic cardiomyopathy and reducing LV hypertrophy and fibrosis which contribute to ventricular arrhythmias and sudden cardiac death (SCD) . RecentlIn the light of these positive results, many authors -6 recommSome studies suggested that corticosteroids might have a beneficial effect in DMD .Males with DMD must be followed-up more closely than female with DMD or those with BMD .The most common form is Myotonic Dystrophy 1(MD1) also known as the Steinert disease with an incidence of 1/8000 births .It is an autosomal dominant disorder due to mutational expansion of a repetitive trinucleotide sequence (CTG) in the 3'-untranslated region of the DMPK (myotonic dystrophy protein kinase gene) on chromosome 19q13.3. SCD is the major risk in affected patients and may occur at a very young age. -9MD1 is the most common form of MD in adults between 20 and 25 years old .The mean age survival is 53 years old. Cardiovascular Events  represents 30% of all cause mortality .Moreover, cardiac involvement may be the first expression of the disease .First degree AV bloc is seen in up to 40% cases. Bundle branch blocs, a long QT, ST-T modifications and axis deviations are other possible findings -11.Because progression of these conduction disorders is unpredictable, in addition to the high prevalence of infra-His defects (conduction impairment below the His bundle), an electrophysiology study (EPS) should always be performed.5).Late potentials (LP), rather than a risk indicator for ventricular arrhythmias, seems to correlate with a delayed activation along the His-Purkinje system -12. LP cAsymptomatic sinus bradycardia has been reported in pediatric series .- Supraventricular tachycardias are very common with up to 25% patients presenting atrial fibrillation or and/or atrial flutter -12.5). 50% of patients who had a pace-maker (PM) for AV bloc, develop VT and are at risk of SCD. Different mechanisms are described for the VT, a particular one is the bundle branch rerentry (BBR) VT since it can be cured by radiofrequency ablation [- Ventricular tachycardias (VT) are frequent, thus any symptomatic patient should undergo an EPS ; EDMD1 is the X-linked form caused by mutations of the STA gene at Xq28 (which encodes the nuclear membrane protein emerin) [ECG is abnormal by age 20 to 30 years old with sinoatrial or auriculo-ventricular conduction disorders, atrial arrhythmias, ventricular arrhythmias (particularly in EDMD2) . CardiomSurvival is 35% at the age of 45 years old with a high risk of SCD -2-11.Family members of the EDMD, especially EDMD2, must be screened after the age of 10 years old, even in the absence of symptoms .It is advised to perform during follow-up, an ECG, a Holter ECG and an Echocardiography once a year. An exercise test may be helpful in children and an EPS will be indicated as for the MD1.Anticoagulation therapy must be started in the setting of atrial arrhythmia or atrial standstill . An ICD Several of these disorders are associated with clinically significant cardiac involvements ,2,11,13.In this autosomal dominant form, conduction disorders progression resembles EDMD2 that can be relevant of a PM or an ICD -13.They are sarcoglycanopathies due to defects on gamma, alpha, beta and delta sarcoglycans.Cardiomyopathy is possible except in alpha-sarcoglycanopathy.Fanin reported cardiomyopathy in 10 patients with LGMD type 2E from 6 different families ; atrial Though guidelines are lacking in these dystrophies, it is advised to repeat ECG/Holter and echocardiography every 2 to 5 years. Empirical therapy by ACEI and /or BB is used in the setting of LV dysfunction .is an alpha-sarcoglycanopathy that associates a dilated cardiomyopathy in 50% cases .have a Becker phenotype except for the cardiac involvement .Described by Landouzy and Dejerine, it is an autosomal dominant disease due to a deletion on the 4q35 chromosome. Arrhythmias are rare in this muscular dystrophy -11.It is a heterogeneous group due to mutations in mitochondrial DNA. Two phenotypes are described: ,11,131. The Kearns-Sayre syndrome oculo-pharyngal myopathy, with chronic progressive external ophtalmoplegia. Infra-His conduction disorders are progressive [gressive . Therefogressive -15.2. The Melas syndrome and Leber disease ,may associate a supraventricular tachycardia due to an accessory pathway [ pathway -11.Advances in the understanding of the incidence, the type, the pathophysiology and molecular biology of the various peripheral myopathies and their cardiac complications had allowed recommendations for the cardiological follow-up of the patients especially in the commonest diseases . Little is known regarding the management of cardiac complications in children with the same diseases; prospective studies in this age group of patients are therefore mandatory. This may lead to early screening in affected families and adjustment of guidelines for device implantation in adults for this young population.