Pediatric acute heart failure caused by endocardial fibroelastosis mimicking dilated cardiomyopathy:A case report

BACKGROUND Endocardial fibroelastosis(EFE)is a diffuse endocardial collagen and elastin hyperplasia disease of unknown etiology,which may be accompanied by myocardial degenerative changes leading to acute or chronic heart failure.However,acute heart failure(AHF)without obvious associated triggers is rare.Prior to the report of endomyocardial biopsy,the diagnosis and treatment of EFE are highly susceptible to being confounded with other primary cardiomyopathies.Here,we report a case of pediatric AHF caused by EFE mimicking dilated cardiomyopathy(DCM),with the aim of providing a valuable reference for clinicians to early identify and diagnose EFE-induced AHF.CASE SUMMARY A 13-mo-old female child was admitted to hospital with retching.Chest X-ray demonstrated enhanced texture in both lungs and an enlarged heart shadow.Color doppler echocardiography showed an enlarged left heart with ventricular wall hypokinesis and decreased left heart function.Abdominal color ultrasonography revealed a markedly enlarged liver.Pending the result of the endomyocardial biopsy report,the child was treated with a variety of resuscitative measures including nasal cannula for oxygen,intramuscular sedation with chlorpromazine and promethazine,cedilanid for cardiac contractility enhancement,and diuretic treatment with furosemide.Subsequently,the child’s endomyocardial biopsy report result was confirmed as EFE.After the above early interventions,the child’s condition gradually stabilized and improved.One week later,the child was discharged.During a 9-mo follow-up period,the child took intermittent low-dose oral digoxin with no signs of recurrence or exacerbation of the heart failure.CONCLUSION Our report suggests that EFE-induced pediatric AHF may present in children over 1 year of age without any apparent precipitants,and that the associated clinical presentations are grossly similar to that of pediatric DCM.Nonetheless,it is still possible to be diagnosed effectively on the basis of the comprehensive analysis of auxiliary inspection findings before the result of the endomyocardial biopsy is reported.

Caution in the use of sedation and endomyocardial biopsy for the management of pediatric acute heart failure caused by endocardial fibroelastosis

Endocardial fibroelastosis(EFE)is commonly considered to be an inflammatory reactive lesion of hyperplasia and deposition of tissue fibers and collagen in the endocardium and/or subendocardium,which is strongly associated with endocardial sclerosis,ventricular remodeling and acute and chronic heart failure,and is one of the important causes for pediatric heart transplantation.Early diagnosis and treatment are the key factors in determining the prognosis of the children.In this paper,we would like to highlight the potential unintended consequences of the use of sedation and biopsy for pediatric acute heart failure caused by EFE and the comprehensive considerations prior to clinical diagnosis.

Incremental value of three-dimensional and contrast echocardiography in the evaluation of endocardial fibroelastosis and multiple cardiovascular thrombi: A case report

BACKGROUND Endocardial fibroelastosis(EFE)is a rare heart disease characterized by thickening of the endocardium caused by massive proliferation of collagenous and elastic tissue,usually leading to impaired cardiac function.Multimodality cardiovascular imaging for the evaluation of EFE with thrombi is even rarer.CASE SUMMARY We report a rare case of EFE associated with multiple cardiovascular thrombi.Three-dimensional(3D)and contrast echocardiography(CE)were used to assess ventricular thrombi.Anticoagulant therapy was administered to eliminate the thrombi.The peripheral contrast-enhanced thrombi with the highest risk were dissolved with anticoagulant therapy at the time of reexamination,which was consistent with the presumption of fresh loose thrombi.CONCLUSION This new echocardiography technique has a great advantage in the diagnosis and treatment of EFE.On the basis of conventional echocardiography,3D echocardiography is used to display the position,shape,and narrow base of the thrombus.CE does not only help to confirm the diagnosis of thrombus,but also determines its risk.

Influence of TRPV1 and LGR5 on downstream proliferation and invasion-related gene expression in endocardial cancer lesions

Objective:To investigate the influence of TRPV1 and LGR5 on downstream proliferation and invasion-related gene expression in endocardial cancer lesions.Methods:A total of 40 patients with endometrial cancer undergoing surgical treatment in our hospital between June 2013 and June 2017 were selected, TRPV1 and LGR5 gene expression in endometrial cancer tissue and adjacent normal tissue were detected, and their median was referred to divide the patients into high TRPV1 group and low TRPV1 group as well as high LGR5 group and low LGR5 group, 20 cases in each group. The differences in proliferation and invasion gene expression in endometrial cancer tissues with different TRPV1 and LGR5 expression were compared. Results:TRPV1 and LGR5 gene expression in endometrial cancer tissues were significantly higher than those in adjacent tissues. Proliferation genes HMGB1, SRPX2 and Wip-1 mRNA expression in lesion tissues of high TRPV1 group and high LGR5 group were higher than those of low TRPV1 group and low LGR5 group respectively whereas ITLN-1 mRNA expression were lower than those of low TRPV1 group and low LGR5 group respectively;invasion genes MTA1, hnRNP H and Snail mRNA expression in lesion tissues of high TRPV1 group and high LGR5 group were higher than those of low TRPV1 group and low LGR5 group respectively whereas Dickkopf and DKK1 mRNA expression were lower than those of low TRPV1 group and low LGR5 group respectively.Conclusions:TRPV1 and LGR5 expression abnormally increase in endometrial cancer, which are directly correlated with the expression of downstream proliferation and invasion genes, and can affect the progress of the disease together.

Characteristics of the Nonselective Cation Current (NSCCs) in Rabbit Left Ventricular Epicardial, Midmyocardial and Endocardial Myocytes

Objectives Recent studies have described regional differences in the electrophysiology and pharmacology of ventric- ular myocardium in canine, feline, rat, guinea pig, and human hearts. This has been shown to be due to a smaller IKs and a lager sodium-calcium exchange current （INa-Ca） and late INa in M region （ deep subepicardial to midmyocardial）. Studies from our laboratory have found a new repolarization current-nonselective cation current （NSCCs） existing in rabbit fight ventricular myocytes. Methods We examined the characteristics of NSCCs in epicardial, M region, and endocardial cells isolated from the rabbit left ventricle with standard microelectrode and whole-cell patch-clamp tech- niques. The permeability to Na^＋ , K^＋ , Li^＋ , Cs^＋ but not to Cl^- indicating that it was a nonselective cation current. Gd^＋3 （0. 1 mmol/1） and La^3＋ （0. 1 retool/1 ） can block the current markedly. Results Further characterization of NSCCs was significantly smaller in M cells than in epicardial and endocardial cells. NSCCs current density was significantly smaller in M cells than in epicardial and endocardial cells. With repolarization to - 80 mV, INa current density was （ -0. 44 ±0. 05） PA/PF in endocardial cells, （ -0. 12 ±0. 05） PA/PF in M cells and （ - 0. 28 ±0. 07） PA/PF in epicardial cells ; and with repolarization to ＋ 30 mV, INa, current density was （ 1.09 ± 0. 29） PA/PF in endocardial cells, （0. 38±0. 09） PA/PF in M cells and （0. 91 ± 0. 32） PA/PF in epicardial cells. Conclusions Transmural dispersion of repolarization was due to the heterogeneity of NSCCs in rabbit left ventricle epicardial, endocardial myocytes and M cells. These findings may advance our understanding of the ionic basis for our understanding of factors contributing to the development of cardiac arrhythmias.

Fetal Endocardial Fibroelastosis with Coarctation of Aortic Arch: A Case Report

This study describes a 34-year-old pregnant woman who underwent prenatal echocardiography at 24 weeks of gestation and found fetal heart abnormalities. She underwent fetal echocardiography in our hospital. Echocardiography showed complex malformation of fetal heart, thickening and calcification of left ventricular myocardium and tendon, weakening or even flattening of left ventricular wall movement, very severe mitral stenosis, severe mitral regurgitation, aortic stenosis, narrowing of the aortic arch, countercurrent of the ductal arch to aortic arch, small diameter of the oval foramen and significantly increased flow velocity. The patient decided to induce labor after expert consultation, but no autopsy was performed due to his family’s refusal. Color Doppler ultrasound can find the positive signs of endocardial elastofibroplasia earlier, and accurately diagnose patients with neonatal elastofibroplasia. It provides accurate diagnostic information for the clinic and can be used as the first choice. This report has obtained the informed consent of its parents.

Biventricular repair for endocardial cushion defects with double outlet right ventricle

Objective-Double outlet right ventricle,which often associated with total anomalous pulmonary venous connection and complete endocardial cushion defects,has been considered a risk factor for biventricular repair procedure. To reviewed cases treated by biventricular repair for endocardial cushion defects with double outlet right ventricle. Methods From July to November of 2009,6

Spatial distribution and network morphology of epicardial,endocardial,interstitial,and Purkinje cell-associated elastin fibers in porcine left ventricle

Cardiac extracellular matrices(ECM)play crucial functional roles in cardiac biomechanics.Previous studies have mainly focused on collagen,the major structural ECM in heart wall.The role of elastin in cardiac mechanics,however,is poorly understood.In this study,we investigated the spatial distribution and microstructural morphologies of cardiac elastin in porcine left ventricles.We demonstrated that the epicardial elastin network had location-and depth-dependency,and the overall epicardial elastin fiber mapping showed certain correlation with the helical heart muscle fiber architecture.When compared to the epicardial layer,the endocardial layer was thicker and has a higher elastin-collagen ratio and a denser elastin fiber network;moreover,the endocardial elastin fibers were finer and more wavy than the epicardial elastin fibers,all suggesting various interface mechanics.The myocardial interstitial elastin fibers co-exist with the perimysial collagen to bind the cardiomyocyte bundles;some of the interstitial elastin fibers showed a locally aligned,hinge-like structure to connect the adjacent cardiomyocyte bundles.This collagen-elastin combination reflects an optimal design in which the collagen provides mechanical strength and elastin fibers facilitate recoiling during systole.Moreover,cardiac elastin fibers,along with collagen network,closely associated with the Purkinje cells,indicating that this ECM association could be essential in organizing cardiac Purkinje cells into“fibrous”and“branching”morphologies and serving as a protective feature when Purkinje fibers experience large deformations in vivo.In short,our observations provide a structural basis for future in-depth biomechanical investigations and biomimicking of this long-overlooked cardiac ECM component.

Left ventricular endocardial pacing predicts the reduction of left ventricular outflow tract pressure gradient immediately after percutaneous transseptal myocardial ablation in patients with hypertrophic obstructive cardiomyopathy refractory to medicatio

Background Hypertrophic obstructive cardiomyopathy （HOCM） carries an increased risk for sudden cardiac death. No data regarding the percutaneous transseptal myocardial ablation （PTSMA） and epicardial left ventricular pacing （LVP） were reported. Methods Seven patients with recurrent symptoms and increased resting left ventricular outflow tract pressure gradient （LVOTG） after PTSMA and another 14 patients with HOCM without history of PTSMA were studied. Both resting and dobutamine stress echocardiography, PTSMA and LVP were routinely performed. Results In patients without previous PTSMA procedure, mild reduction of resting LVOTG was detected at 5 minutes after left ventricular pacing, and this reduction became significant at 10 minutes. All patients were divided into successful and unsuccessful groups according to their response to LVP. In contrary to patients in unsuccessful group, resting and R-S2 stimuli-induced LVOTG during PTSMA procedure were decreased dramatically （（9±5） mmHg vs （58±12） mmHg, （12±2） mmHg vs （113±27） mmHg, P〈0.001）. Analysis of Logistic regression demonstrated that only LVOTG level dudng left ventdcular pacing was an independent factor predicting the reduction of LVOTG immediately after PTSMA （odds ratio （OR）, 0.59; 95% CI 2.67 to 5.82; P=0.0002）. Conclusion Left ventricular endocardial temporary pacing plays a critical role in predicting acute effect on the reduction of LVOTG immediately after PTSMA procedure.

Tropical Coronary Artery Disease and Arrhythmogenic Potentials—The Changing Pattern towards Endomyocardial Fibrosis—An Analysis

Aim: To analyse the increasing burden of coronary artery disease (CAD) in tropical and subtropical belts of the Equator since it remains blurred and carries a grim prognosis. Introduction: Endomyocardial fibrosis [EMF] is a tropical febrile disorder, confined to peculiar and limited geographical areas. Plaque buildup in endocardium and coronary arteries, causing ischemic injury and arrhythmic episodes, is a vanishing mystery in its pathogenesis and emphasizing alternative routes for understanding and treatment of this enigmatic disease. Case Report: 15 cases in various age groups were reported with potential complications of coronary artery disease and arrhythmias, associated with endocardial lesions, the characteristic feature of endomyocardial fibrosis. Conclusion: The narrowing of coronary arteries as a result of thickening of the walls, spasm, inflammation, plaques and its rupture produce ischemic episodes which can occur slowly or suddenly in a devastating pattern with arrhythmogenic potentials. The important steps to prevent and decrease the risk of CAD is to reduce the chance of getting this disorder by epidemiological measures with an advice of blood thinning medications such as small daily dose aspirin, antibiotics in susceptible individuals and revascularization in established myocardial infarction.

Arrhythmogenic right ventricular cardiomyopathy characterized by recurrent syncope during exercise:A case report

BACKGROUND Arrhythmogenic right ventricular(RV)cardiomyopathy is a rare and currently underrecognized cardiomyopathy characterized by the replacement of RV myocardium by fibrofatty tissue.It may be asymptomatic or symptomatic(palpitations or syncope)and may induce sudden cardiac death,especially during exercise.To prevent adverse events such as sudden cardiac death and heart failure,early diagnosis and treatment of arrhythmogenic RV cardiomyopathy(ARVC)are crucial.We report a patient with ARVC characterized by recurrent syncope during exercise who was successfully treated with combined endocardial and epicardial catheter ablation.CASE SUMMARY A 43-year-old man was referred for an episode of syncope during exercise.Previously,the patient experienced two episodes of syncope without a firm etiological diagnosis.An electrocardiogram obtained at admission indicated ventricular tachycardia originating from the inferior wall of the right ventricle.The ventricular tachycardia was terminated with intravenous propafenone.A repeat electrocardiogram showed a regular sinus rhythm with negative T waves and a delayed S-wave upstroke from leads V1 to V4.Cardiac magnetic resonance imaging showed RV free wall thinning,regional RV akinesia,RV dilatation and fibrofatty infiltration(RV ejection fraction of 38%).An electrophysiological study showed multiple inducible ventricular tachycardia as of a focal mechanism from the right ventricle.Endocardial and epicardial voltage mapping demonstrated scar tissue in the anterior wall,free wall and posterior wall of the right ventricle.Late potentials were also recorded.The patient was diagnosed with ARVC and treated with combined endocardial and epicardial catheter ablation with a very satisfactory follow-up result.CONCLUSION Clinicians should be aware of ARVC,and further workup,including imaging with multiple modalities,should be pursued.The combination of epicardial and endocardial catheter ablation can lead to a good outcome.

A Case of Pediatric Heart Failure Caused by Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery: Case Report and Literature Review

A female patient aged 3 months and 10 days was admitted to the cardiology department because of symptoms of heart failure.According to the echocardiography results,the patient received a diagnosis of primary endocardial fi broelastosis and was treated withγ-globulin,prednisone,digoxin,and diuretics.Coronary computed tomographic angiography and coronary angiography were performed as there was no improvement after 2 months of treatment.Finally,the patient received a diagnosis of anomalous origin of the left coronary artery from the pulmonary artery(ALCAPA).ALCAPA is a rare congenital heart defect that can cause severe heart failure during infancy,and is easily misdiagnosed clinically.In this report,we show the process of misdiagnosis of the case and consult the relevant literature,hoping to improve the understanding and early diagnosis of ALCAPA.

Experience of a Single Center in the Diagnosis and Classification of Cases of Left Ventricular Noncompaction

Objectives: To analyse the clinical profile of consecutive cases of Left Ventricular Non Compaction (LVNC) with particular interest in non-compacted segments valuation. Methods: There were 18,000 patients seen from 2007 to 2010, with a complete evaluation including family history and personal cardiac history, clinical examination and electrocardiography. Diagnosis was based on three published definitions. Results: The diagnosis of LVNC was placed in 1.4% of cases. Clinical and echo-cardiographic data for the 250 cases of LVNC are presented. Trabecular meshwork was observed predominantly at the apex (91.6%), in the lateral and inferior wall (40.4% and 38.0% respectively), and less frequently in the posterior and anterior wall (21.6% and 9.2% respectively). Conclusions: This study suggests that LVNC is a form of cardiomyopathy with higher prevalence and relatively better prognosis than previously reported.

Surgical Experience of a Partial Atrioventricular Septal Defect in an Elderly Patient: A Case Report

The following paper describes patch closure (bovine pericardial sheet) of an ostium primum atrial septal defect and mitral valve repair (sutured mitral valve cleft + autologous pericardial annuloplasty + Alfieri’s method) in a 50-year-old man. He had been perfectly well until he was brought to the emergency room because of acute heart failure. Chest radiography revealed right-side heart enlargement. Electrocardiography indicated atrial flutter. Echocardiography revealed a large ostium primum atrial septal defect and moderate mitral regurgitation. The pulmonary-to-systemic blood flow ratio was 3.24. First, cardiologists performed catheter ablation of the cavotricuspid isthmus for atrial flutter. We performed patch closure of an ostium primum atrial septal defect and mitral valve repair after the patient’s heart failure was under control. The patient was discharged 13 days postoperatively in a satisfactory condition without any critical complications.

Accessory Leaflet of Tricuspid Valve Masquerading as Infective Endocarditis Preoperatively and Causing RVOT Obstruction Postoperatively

Accessory tricuspid valve tissue is a rare congenital anomaly. We report a case where preoperative echocardiogram was suggestive of infective vegetations over the tricuspid valve but intra-operatively it was found to be accessory tricuspid valve leaflets which were causing right ventricular outflow tract obstruction.

Validation of a Marker of Atrial Contraction in the SonR Signal

Introduction: The main component of the endocardial acceleration signal (SonR) is today used for cardiac resynchronization therapy (CRT) optimization. This prospective, single center pilot study focuses on another signal component, SonR4 that may provide further information on the atrial activity. Methods and Results: SonR signal and ECG tracings were recorded simultaneously during a CRT-D optimization procedure in 15 patients (12 men, 68 ± 9.5 years, ischemic heart disease 53%) indicated for CRT. Correlation between SonR4 signal, recorded using SonR and atrial contraction, identified by Echo Doppler was evaluated by Pearson and Student’s t tests under different Atrio-Ventricular (AV) delay programming. From 15 consecutive screened patients, 9 had concomitant analyzable SonR4 and ECG recordings and were included in the study population. The presence of the SonR4 component was systematically correlated to the presence of the A wave. A significant correlation was observed between SonR4 and A wave timings (r = 0.75, p = 0.02) according to different AV delays, with a high reproducibility in SonR4 assessment. Conclusion: A strong correlation between SonR4 and atrial contraction timings was observed, further suggesting that SonR4 is a marker of the atrial contraction. Additional assessments in larger populations are required to confirm these results and build further applications.

“Burnt-Out” Endomyocardial Fibrosis—An Overview

Tropical endomyocardial fibrosis (EMF) is a public health problem affecting the children, young adults and elderly individuals in an epidemic fashion in the coastal districts of south India. Due to lack of resources for research in these endemic areas, its etiology remains elusive and hypotheses ranging from infections and allergic causes to malnutrition and toxins have not been tested rigorously. The disease is characterized by endocardial fibrosis and the right ventricle is the cardiac chamber most frequently affected. Patients may present clinically with heart failure and an associated AV (atrioventricular) valve regurgitation is common. Several features of the advanced disease called as “burnt-out” stage of endomyocardial fibrosis (EMF) are not fully understood. Background of these case studies described the clinical presentation, echocardiographic features and management of this late stage of the disease.

Cardiac cell type-specific responses to injury and contributions to heart regeneration

Heart disease is the leading cause of mortality worldwide.Due to the limited proliferation rate of mature cardiomyocytes,adult mammalian hearts are unable to regenerate damaged cardiac muscle following injury.Instead,injured area is replaced by fibrotic scar tissue,which may lead to irreversible cardiac remodeling and organ failure.In contrast,adult zebrafish and neonatal mammalian possess the capacity for heart regeneration and have been widely used as experimental models.Recent studies have shown that multiple types of cells within the heart can respond to injury with the activation of distinct signaling pathways.Determining the specific contributions of each cell type is essential for our understanding of the regeneration network organization throughout the heart.In this review,we provide an overview of the distinct functions and coordinated cell behaviors of several major cell types including cardiomyocytes,endocardial cells,epicardial cells,fibroblasts,and immune cells.The topic focuses on their specific responses and cellular plasticity after injury,and potential therapeutic applications.