Left ventricular noncompaction associated with hypertrophic cardiomyopathy and Wolff-Parkinson-White syndrome

We report a 35-year-old female patient with hypertrophic cardiomyopathy, left ventricular noncompaction, and Wolff-Parkinson-White EKG pattern. Several other family members present the same clinical condition. We speculate that this phenotype is related to the genotypes PRKAG2 and LAMP2 represented by mutations of the genes encoding AMP-activated protein kinase (PRKAG2) and lysosome associated membrane protein 2 (LAMP2).

Effect of trabeculated myocardial mass on left ventricle global and regional functions in noncompaction cardiomyopathy

BACKGROUND Left ventricular(LV)noncompaction cardiomyopathy is a rare cardiomyopathic subtype that has been recognized in recent years and is being diagnosed at an increased rate.There is no consensus regarding the diagnosis of the disease,and increased trabeculation rates that meet the existing diagnostic criteria may even be present in healthy asymptomatic people.This indicates that differentiating criteria for diagnosis are needed.AIM To examine the increase in myocardial trabeculation and the change in left ventricular global and regional functions.METHODS This retrospective study included 65 patients(28 females,37 males)diagnosed with LV noncompaction cardiomyopathy who underwent cardiac magnetic resonance imaging between January 2011 and August 2016 and had a noncompacted/compacted myocardial thickness ratio of over 2.3 in more than one segment in the left ventricle.The distribution and ratios of trabeculations in apical,midventricular,and basal regions were examined in short-axis images obtained from cardiac magnetic resonance.In addition,by using short-axis cine images,regional ejection fraction(EF)and global EF were calculated using the Simpson method in the left ventricle at apical,basal,and midventricular levels.RESULTS While the number of trabeculated segments were similar at the apical(3.2±1.0)and midventricular levels,a statistically significant level of involvement was not observed at the basal level(0.4±0.9)(P>0.05).The highest noncompacted/compacted(trabeculation)ratio was observed at the apical level(3.9±1.4),while this ratio was higher at the anterior(59%-89.4%)and lateral(62%-84.8%)segments(P>0.05).Global EF was positively correlated with apical,midventricular,and basal regional EF(P<0.05).However,there was no significant correlation between regional EF and the number of trabeculated segments or trabeculation ratio in all three regions;nor was there a significant correlation between regional EF and the number of trabeculated segments or trabeculation ratio in the entire LV(P>0.05).CONCLUSION No global or regional relationship was observed between LV dysfunction and trabeculation rate or the number of trabeculated segments.This limits the usefulness of change in LV functions in the differentiation between normal and pathological trabeculation.

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.

Incremental value of contrast echocardiography in the diagnosis of left ventricular noncompaction

Contrast echocardiography with left ventricular opacification （LVO） improves the definition of endocardium in two-dimensional echocardiography （2DE）. This study was aimed to determine whether LVO offered added diagnostic value in noncompaetion of left ventricular myocardium （NCVM）. A total of 85 patients （40± 20 years, 54 males） with suspected NCVM were subjected to transthoracic 2DE and LVO, and 40 healthy volunteers were examined with 2DE and assigned as control subjects. The location of NCVM, the thickness ratio of noncompacted to compacted myocardium （NCR）, and the cavity size and ejection fraction of LV were quantified. Results revealed that NCVM was mainly located in the LV medium （53.2%）, apical （46.2%） segments, and lateral wall （39.8%）. The NCR obtained through LVO was greater than that detected through 2DE （4.2 ±1.3 vs. 3.3 ±1.2, P 〈 0.001）, and higher inter-correlations and less intra- and inter-observer variabilities were determined in the former than in the latter. The NCVM detection rates were also increased from 63.5% via 2DE to 83.5% via LVO and 89.4% via 2DE combined with LVO （2DE ＋ LVO） （P = 0.0004）. The LV cavity size was greater and the LV ejection fraction （LVEF） was lower in the NCVM patients than in the control group （P 〈 0.01）. In the NCVM group, the LV cavity size was higher and the LVEF was lower in LVO than in 2DE （P 〈 0.01）. In conclusion, contrast echocardiography contributes significant sensitivity and reproducibility to routine transthoraeic echoeardiography in NCVM diagnosis. Therefore, this technique should be clinically performed to diagnose suspected NCVM.

Noncompaction cardiomyopathy associated with hypogenetic lung

Noncompaction of the ventricular myocardium is a rare form of congenital cardiomyopathy with a high incidence of cardiovascular complications. It frequently manifests in an isolated form, namely isolated noncompaction of the ventricular myocardium. Not only does noncompaction of the ventricular myocardium associate with other congenital cardiac malformations, but also with other neuromuscular disorders. The purpose of this study was to explore the correlation of hypogenetic lung with myocardial noncompaction in terms of the clinical course of one of our patients.

Genetic heterogeneity of noncompaction

To the Editor： With interest we read the article by Wu et all on a 53-year male with agenesia of the right lung and left-ventricular hypertrabeculation / noncompaction （LVHT）. The paper raises the following concerns.

A male with chronic thromboembolic pulmonary hypertension caused by isolated noncompaction of ventricular myocardium

Noncompaction of the ventricular myocardium （NVM） is a rare disease of endomyocardialmorphogenesis characterized by numerous, prominent trabeculations and deep intertrabecular recesses. It is commonly associated with congenital heart disease. Isolated noncompaction of the ventricular myocardium （INVM） is thought to be caused by arrest of normal embryogenesis of the endocardium and myocardimn. A significant amount of data on INVM are available for the left ventricle but right ventricular involvement is rare.

An eIF3a gene mutation dysregulates myocardium growth with left ventricular noncompaction via the p-ERK1/2 pathway

Left ventricular noncompaction(LVNC)is a heterogeneous disorder with undlear genetic causes and an unknown mechanism.elF3a,an important member of the Eukaryotic translation initiation factor 3(elF3)family,is involved in multiple biological processes,indluding cell prolif eration and migration during myocardial development,suggesting it could play a role in LVNC development.To investigate the association between a novel variant(C.1145 A->G)in elF3a and LVNC,and explore potential mechanisms that could lead to the development of LVNC.A novel elF3a variant,C.1145 A->G,was identified by whole-exome sequencing in a familial pedigree with LVNC.Adenovirus vectors containing wild-type elF 3a and the mutated version were constructed and co-infected into H9C2 cells.Cell proliferation,apoptosis,cell migration,and differentiation,as well as phosphorylation of ERK1/2 were stud-ied and were measured by proliferation assays,flow cytometry,real-time PCR and Westem blot,respectively.The elF3a mutation inhibited the proliferation of H9C2 cells,induced apoptosis,promoted cell migration,and inhibited the dif ferentiation of human induced plurip-otent stem cell-derived cardiomyocytes(hiPSC-CMs).The effect of the elF3a mutation may be attributed to a decrease in expression of p-ERK1/2.A novel elF3a gene mutation disrupted the p-ERK1/2 pathway and caused decreased myocardium proliferation,differentiation,acceler-ated migration.This finding may provide some insight into the mechanism involved in LVNC development.

Isolated biventricular noncompaction： a case report

Myocardial noncompaction （MN） is defined as an .unclassified congenital cardiomyopathy which is characterized by the presence of prominent ventricular trabeculations and deep intertrabecular recesses. It is assumed to occur as an arrest in normal endomyocardial morphogenesis and is mostly associated with other congenital cardiac malformations The left ventricle is usually involved; but, both ventricules can be affected.

Existence of Solutions of a Convolution Integral Equation

In this study, we prove the of existence of solutions of a convolution Volterra integral equation in the space of the Lebesgue integrable function on the set of positive real numbers and with the standard norm defined on it. An operator P was assigned to the convolution integral operator which was later expressed in terms of the superposition operator and the nonlinear operator. Given a ball Br belonging to the space L it was established that the operator P maps the ball into itself. The Hausdorff measure of noncompactness was then applied by first proving that given a set M∈ B r the set is bounded, closed, convex and nondecreasing. Finally, the Darbo fixed point theorem was applied on the measure obtained from the set E belonging to M. From this application, it was observed that the conditions for the Darbo fixed point theorem was satisfied. This indicated the presence of at least a fixed point for the integral equation which thereby implying the existence of solutions for the integral equation.

Research progress on noncompaction of ventricular myocardium

Background Noncompaction of ventricular myocardium （NVM） is a rare type of primary cardiomyopathy. The disease is caused by the disorder in the densification of the myocardium in the early stage of the embryo process. The morphological characteristics are projecting trabeculation in the ventricle and the deep trabecular space interlinked with ventricular chamber. In recent years, many studies have found that the left ventricular growth associated genetic mutation is closely related to the occurrence of NVM. The most clinical manifestations such as heart failure, thromboembolism and arrhythmia are specific. Echocardiography is the most commonly used tech- nique for the diagnosis of NVM. Cardiac computed tomography （CT） scan, cardiac magnetic resonance imaging and left ventricular angiography are other important techniques for its diagnosis. The NVM patients have a long course of disease, poor prognosis and a high rate of misdiagnosis. This article reviews the research progress in the aspects of epidemiological characteristics, genetic characteristics, clinical manifestations, pathophysiology, diagnosis, treatment and so on, in order to provide the basis for the diagnosis and treatment of NVM.

Comparison of systolic and diastolic criteria for isolated LV noncompaction in CMR

ABSTRACT Objectives This study used cardiac magnetic resonance （CMR） to compare standard criteria for left ventricular non- compaction （LVNC）. Background LVNC as a distinct cardiomyopathy is supported by a growing number of publications. Echocardiographic and CMR criteria have been established to diagnosis LVNC but have led to concerns of diagnostic accura- cy.

SOME CONVERGENCE PROBLEMS REGARDING THE FRACTIONAL SCHRODINGER PROPAGATOR ON NONCOMPACT MANIFOLDS

Let L be the Laplace-Beltrami operator.On an n-dimensional(n≥2),complete,noncompact Riemannian manifold M,we prove that if 0<α<1,s>α/2 and f∈Hs(M),then the fractional Schr?dinger propagator e(it|L|α/2)(f)(x)→f(x)a.e.as t→0.In addition,for when M is a Lie group,the rate of the convergence is also studied.These results are a non-trivial extension of results on Euclidean spaces and compact manifolds.

Unusual Cardiomyopathies:Some May Be More Usual Than Previously Thought and Simply Underdiagnosed

Heart failure is the most common cause of hospitalization in the United States.Just as the prevalence of heart failure has increased,the number of diseases identifi ed that result in the heart failure syndrome has escalated.Certain cardiomyopathies that have previously been regarded as very rare are being recognized with increasing frequency,because of improved imaging techniques and an increased understanding of the pathophysiologic mechanisms that result in these diseases.Improved echocardiographic techniques and methods such as spectral Doppler and 3D image rendering,along with the use of advanced diagnostic tools such as cardiac CT angiography and cardiac magnetic resonance imaging are now common.These advanced imaging methods have led to an increased appreciation of the frequency of diseases such as isolated left ventricular noncompaction and cardiac amyloidosis.Left ventricular noncompaction,once thought to occur in roughly one in one million patients,may actually be seen in fewer than one in 1000 patients.Cardiac amyloidosis,in the senile form,may exist in 80% of 80-year-old patients,although the incidence of clinical symptoms is less.As the genetic alterations that contribute to these diseases are further elucidated,improved diagnosis and a better understanding of the prognosis of these uncommon cardiomyopathies will follow.

EXISTENCE OF SOLUTION AND APPROXIMATE CONTROLLABILITY OF A SECOND-ORDER NEUTRAL STOCHASTIC DIFFERENTIAL EQUATION WITH STATE DEPENDENT DELAY

This paper has two sections which deals with a second order stochastic neutral partial differential equation with state dependent delay. In the first section the existence and uniqueness of mild solution is obtained by use of measure of non-compactness. In the second section the conditions for approximate controllability are investigated for the distributed second order neutral stochastic differential system with respect to the approximate controllability of the corresponding linear system in a Hilbert space. Our method is an extension of co-author N. Sukavanam’s novel approach in [22]. Thereby, we remove the need to assume the invertibility of a controllability operator used by authors in [5], which fails to exist in infinite dimensional spaces if the associated semigroup is compact. Our approach also removes the need to check the invertibility of the controllability Gramian operator and associated limit condition used by the authors in [20], which are practically difficult to verify and apply. An example is provided to illustrate the presented theory.

ON MONOTONE ITERATIVE METHOD FOR INITIAL VALUE PROBLEMS OF NONLINEAR SECOND-ORDER INTEGRO-DIFFERENTIAL EQUATIONS IN BANACH SPACES

Using the monotone iterative method and Monch Fixed point theorem, the existence of solutions and coupled minimal and maximal quasisolutions of initial value problems for mixed monotone second-order integro-differential equations in Banach spaces are studied. Some existence theorems of solutions and coupled minimal and maximal quasisolutions are obtained.

THE EXISTENCE OF SOLUTIONS OF NONLINEARFREDHOLM INTEGRAL EQUATIONS IN BANACH SPACES

This paper improves Tychonov ford point theorem and discusses the existence of solutions of nonlinear Fredholm integral equations on [0,+∞] in Banach spaces with Frechet space theory.

CHARACTERIZATIONS OF COMPACT OPERATORS ON SOME EULER SPACES OF DIFFERENCE SEQUENCES OF ORDER m

In the past, several authors studied spaces of m-th order difference sequences, among them, H.Polat and F.Basar （[17]） defined the Euler spaces of m-th order difference sequences e r 0 （△ （ m ） ）, e r c （△ （ m ） ） and e r ∞ （△ （ m ） ） and characterized some classes of matrix transformations on them. In our paper, we add a new supplementary aspect to their research by characterizing classes of compact operators on those spaces. For that purpose, the spaces are treated as the matrix domains of a triangle in the classical sequence spaces c 0 , c and ∞ . The main tool for our characterizations is the Hausdorff measure of noncompactness.

STATIONARY DISTRIBUTIONS OF INFINITE PARTICLE SYSTEMS WITH NONCOMPACT STATE SPACE.

This paper deals with the preblem of existence and uniqueness of the stationary distributions (abbr., s. d.'s) for the processes constructed in [4] .The main results are stated in § 1. For the reader's convenience we first restate the existence theorems (Theorem 1 and 2) of the processes given in [4]. Then two existence theorems (Theorem 3 and 4) and a uniqueness theorem (Theorem 5) for the s. d.'s of the processes are presented. The last result (Theorem 6), as an application of the previous ones, is about the Schlgl model which comes from nonequilibrium statisticali physics. The details of the proofs of Theorem 3—6 are given in § 2—4.

NONCOMPACT INFINITE OPTIMIZATION AND EQUILIBRIA OF CONSTRAINED GAMES IN GENERALIZED CONVEX SPACES

By applying a new existence theorem of quasi-equilibrium problems due to the author, some existence theorems of solutions for noncompact infinite optimization problems and noncompact constrained game problems are proved in generalized convex spaces without linear structure. These theorems improve and generalize a number of important results in recent literature.