Noncompaction Of Left Ventricular Myocardium Research Articles

Prognostic prediction of left ventricular myocardial noncompaction using machine learning and cardiac magnetic resonance radiomics.

Although there are many studies on the prognostic factors of left ventricular myocardial noncompaction (LVNC), the determinants are varied and not entirely consistent. This study aimed to build predictive models using radiomics features and machine learning to predict major adverse cardiovascular events (MACEs) in patients with LVNC. In total, 96 patients with LVNC were included and randomly divided into training and test cohorts. A total of 105 cine cardiac magnetic resonance (CMR)-derived radiomics features and 35 clinical characteristics were extracted. Five different oversampling algorithms were compared for selection of the optimal imbalanced processing. Feature importance was assessed with extreme gradient boosting (XGBoost). We compared the performance of 5 machine learning classification methods with different sample:feature ratios to determine the optimal hybrid classification strategy. Subsequently, radiomics, clinical, and combined radiomics-clinical models were developed and compared. The machine learning pipeline included an adaptive synthetic (ADASYN) algorithm for imbalanced processing, XGBoost feature selection with a sample:feature ratio of 10, and support vector machine (SVM) modeling. The areas under the receiver operating characteristic curves (AUCs) of the radiomics model, clinical model, and combined model in the validation cohort were 0.87 (sensitivity 83.33%, specificity 64.29%), 0.65 (sensitivity 16.67%, specificity 78.57%), and 0.92 (specificity 33.33%, sensitivity 100.00%), respectively. The radiomics model performed similarly to the clinical and combined models (P=0.124 and P=0.621, respectively). The performance of the combined model was significantly better than that of the clinical model (P=0.003). The machine learning-based cine CMR radiomics model performed well at predicting MACEs in patients with LVNC. Adding radiomics features offered incremental prognostic value over clinical factors alone.

Value of cardiac magnetic resonance feature tracking technology in the differential diagnosis of isolated left ventricular noncompaction and dilated cardiomyopathy.

This study explored the value of myocardial strain in the differential diagnosis of isolated left ventricular myocardial noncompaction (ILVNC) and dilated cardiomyopathy (DCM) using cardiac magnetic resonance (CMR) feature tracking technology. This retrospective analysis was performed on consecutive patients (25 with ILVNC, 30 with DCM, and 30 healthy controls) presenting to Shanxi Cardiovascular Hospital. All ILVNC patients met echocardiographic and CMR criteria for ventricular non-compaction. All patients with DCM met the 2016 American Heart Association and 2018 Chinese Medical Association Cardiovascular Branch diagnostic criteria. cvi42 software (Circle Cardiovascular Imaging) was used to measure radial, circumferential, and longitudinal strain (LS) globally and in segments of the left ventricle. Analysis of variance was used to compare strains among groups and among different segments within the same group. Receiver operating characteristic (ROC) curves were used to evaluate the diagnostic efficacy of different parameters in ILVNC and DCM. Basal circumferential strain was lower in the DCM than in the ILVNC group (P=0.05). Both median and apical LS were lower in the ILVNC than in DCM group (P=0.02 and P=0.01, respectively). ROC curves showed that apical LS was the most effective in distinguishing ILVNC from DCM [area under the curve (AUC) =0.883; P<0.001; 95% CI: 0.850-0.977]. Comparing strains among different segments within the same group revealed that in DCM, the circumferential and LS of the apex were higher than those of the basal segment, which is consistent with the pattern in healthy controls; however, has no such regular pattern was seen in ILVNC. Myocardial strain parameters are of considerable value in the differential diagnosis of ILVNC and DCM. Differences in patterns between ILVNC and DCM can be sensitively identified, providing more comprehensive information for early clinical diagnosis.

Prognostic Value of Left Atrial Reservoir Strain in Left Ventricular Myocardial Noncompaction: A 3.0 T Cardiac Magnetic Resonance Feature Tracking Study.

The relationship of left atrial (LA) strain to high-risk heart failure (HF) events in patients with left ventricular myocardial noncompaction (LVNC) remains to be thoroughly investigated. To evaluate the LA performance in patients with LVNC, and to investigate the prognostic value of LA phasic strain on high-risk HF events, and its influencing factors. Retrospective. A total of 95 LVNC patients (74 with LA enlargement [LAE] and 21 without LAE) and 50 healthy controls. A 3.0 T, balanced steady-state free-precession cine imaging. LA longitudinal strains were measured by cardiac MRI feature tracking technique. LA volume index (LAVI) and LA ejection fraction (LAEF) were calculated. Their intraobserver and interobserver reproducibility were evaluated. The primary outcome was high-risk HF events, a composite of first HF hospitalization, hospitalization for worsening HF and death from HF. Student's t/Mann-Whitney U, one-way analysis of variance/Kruskal-Wallis, Chi-squared, receiver operating characteristic, Kaplan-Meier, log-rank, Cox regression, Pearson and Spearman correlation and linear regression analyses were performed. The significance threshold was set at P < 0 .05. LAEF and LA longitudinal strains decreased in LVNC patients irrespective of the presence of LAE. During a median follow-up of 32.17months, high-risk HF occurred in 13 (13.68%) patients. Patients with increased LAVI, decreased LAEF and decreased LA longitudinal strain had significantly higher risks of high-risk HF events. In patients with LVNC, LA reservoir strain (εs) was independently associated with high-risk HF (hazard ratio=23.208 [95% CI: 2.993-179.967]). LV global longitudinal strain (LV GLS) (β=-1.783 [95% CI: -2.493 to -1.073]) was significantly and independently associated with εs. Intraobserver and interobserver reproducibility was excellent for LAVI, LAEF, and LA strain. In patients with LVNC, εs was an independent predictor for high-risk HF events. LV GLS was an independent determinant of εs in LVNC. 4 TECHNICAL EFFICACY: Stage 4.

P4654Clinical and electrocardiographic features of restrictive cardiomyopathy in children

Abstract Introduction Restrictive cardiomyopathy (RCM) is a rare myocardial disease with an impaired diastolic function and poor prognosis. The mean survival duration after a diagnosis of RCM is reported to be around 2 years in children and most need heart transplantations. Purpose This study aimed to determine the 12-lead electrocardiogram (ECG) diagnostic criteria of RCM based on the initial diagnostic electrocardiogram. Methods ECGs in pediatric cardiomyopathy patients were collected from 15 institutes in Japan between 1979 and 2013. We compared the ECG findings, especially of the P wave, in RCM patients between the cardiomyopathy group and healthy children group separately for each gender and the age. The ECGs in the healthy group were obtained from school heart screening in Japan of first-graders, and seventh-graders. Statistical significance was determined as p&lt;0.001. Results Among 376 registered cardiomyopathy patients, 63 had hypertrophic cardiomyopathy (HCM) (36%), 91 (24%) dilated cardiomyopathy (DCM), 106 (28%) a left ventricular myocardial noncompaction (LVNCs), 25 (7%) restrictive cardiomyopathy (RCM), 14 (4%) arrhythmogenic right ventricular cardiomyopathy (ARVC), and 5 (1%) other cardiomyopathies. Of the 25 RCM patients (9.9±3.4 years old, F:M=11:14), 36% were discovered during school heart screening. The first onset was an abnormal ECG in 9, symptoms of heart failure in 6, respiratory tract infections in 3, syncope in 1, and 6 with other. Of those patients, 2 (8%) had a family history of RCM, 24 (92%) no family history. A genetic diagnosis was performed in 5 of the 25 cases, and 3 had genetic abnormalities related to RCM. The mean follow-up period was 65±95 months (mean±standard deviation). During follow up, 19 patients (76%) survived, 6 (24%) died, 7 (28%) had heart transplantations, and 3 (12%) were waiting for heart transplantations with a left ventricular assist device. The P wave was bimodal in lead I or biphasic in lead V1 in 15 patients (93%), and 13 (81%) patients had both variations. We evaluated the duration and amplitude of the first and second component of the P wave as P1 and P2. The number of control and RCM patients (control/RCM), duration of P1+P2, and sum total absolute value of the amplitude of P1+P2 in lead V1 were 8350/5, 90±9/116±10ms, and 72±28/528±278μV in first grade boys, 8423/3, 91±10/120±22ms, and 66±28/326±229μV in first grade girls, 8943/1, 97±1/100ms, and 71±31/328μV in seventh grade boys, and 9183/5, 98±11/112±10ms, and 55±27/315±56μV in seventh grade girls. Although the number of patients in the RCM group was small, sum total absolute value of the amplitude of P1+P2 in lead V1 showed a significant difference in any group. Conclusion The ECG in children with RCM exhibits P wave abnormalities in almost all patients. In particular, not the P wave interval but P wave shape in I and V1 and the sum total absolute value of the amplitude of P1+P2 in lead V1 were observed differences.

The results of surgical treatment of a patient with isolated left ventricular apical hypoplasia and left ventricular noncompaction

There is a presentation of the results of a two-year follow-up after surgical treatment of a patient with chronic heart failure and a permanent form of atrial fibrillation, in which isolated apex hypoplasia and non-compaction of left ventricular myocardium were revealed.

Novel α-Actin Gene Mutation p.(Ala21Val) Causing Familial Hypertrophic Cardiomyopathy, Myocardial Noncompaction, and Transmural Crypts. Clinical-Pathologic Correlation.

BackgroundMutations of α‐actin gene (ACTC1) have been phenotypically related to various cardiac anomalies, including hypertrophic cardiomyopathy and dilated cardiomyopathy and left ventricular (LV) myocardial noncompaction. A novel ACTC mutation is reported as cosegregating for familial hypertrophic cardiomyopathy and LV myocardial noncompaction with transmural crypts.Methods and ResultsIn an Italian family of 7 subjects, 4 aged 10 (II‐1), 14 (II‐2), 43 (I‐4) and 46 years (I‐5), presenting abnormal ECG changes, dyspnea and palpitation (II‐2, I‐4, and I‐5), and recurrent cerebral ischemic attack (I‐5), underwent 2‐dimensional echo, cardiac magnetic resonance, Holter monitoring, and next‐generation sequencing gene analysis. Patients II‐2 and I‐5 with ventricular tachycardia underwent a cardiac invasive study, including coronary with LV angiography and endomyocardial biopsy. In all the affected members, ECG showed right bundle branch block and left anterior hemiblock with age‐related prolongation of QRS duration. Two‐dimensional echo and cardiac magnetic resonance documented LV myocardial noncompaction in all and in I‐4, I‐5, and II‐2 a progressive LV hypertrophy up to 22‐mm maximal wall thickness. Coronary arteries were normal. LV angiography showed transmural crypts progressing to spongeous myocardial transformation with LV dilatation and dysfunction in the oldest subject. At histology and electron microscopy detachment of myocardiocytes were associated with cell and myofibrillar disarray and degradation of intercalated discs causing disanchorage of myofilaments to cell membrane. Next‐generation sequencing showed in affected members an unreported p.(Ala21Val) mutation of ACTC.ConclusionsNovel p.(Ala21Val) mutation of ACTC1 causes myofibrillar and intercalated disc alteration leading to familial hypertrophic cardiomyopathy and LV myocardial noncompaction with transmural crypts.

Heart transplantation for the treatment of isolated left ventricular myocardial noncompaction. First case in Mexico

Heart transplantation for the treatment of isolated left ventricular myocardial noncompaction. First case in Mexico

Progress in Molecular Genetic Study of Mitochondrial Cardiomyopathy.

Mitochondria plays a key role in providing ATP for the energy-consuming cardiac tissues. Mitochondrial cardiomyopathy is a myocardial condition characterized by abnormal heart structure and/or function secondary to genetic defects involving the mitochondrial respiratory chain. The typical cardiac manifestations of mitochondrial cardiomyopathy include hypertrophic and dilated cardiomyopathy,while left ventricular myocardial noncompaction is less common. Recent research has suggested that most mitochondrial diseases result from mitochondrial DNA mutation,which can be found in genes that encode ancillary proteins needed for genetic transcription (tRNA),in genes that encode subunits of the electron transport chain complexes,or in genes that control the activities of the mitochondria called D-loop zone. However,the exact physiological mechanisms remain unclear. This review summarizes the recent advances in the molecular mechanism of mitochondrial cardiomyopathy.

Non-compaction of left ventricular myocardium in sub-Saharan African adults

BackgroundNon-compaction of the left ventricle (NCLV) is an unclassified cardiomyopathy due to intrauterine arrest of compaction of the loose interwoven meshwork. Only a few studies involving sub-Saharan Africans insmall numbers...

Advances in Diagnosis and Management of Mitochondrial Cardiomyopathy.

Mitochondrial cardiomyopathy (MCM) is a series of myocardial conditions characterized by abnormal heart-muscle structure,function,or both,secondary to genetic defects involving the mitochondrial respiratory chain,in the absence of concomitant coronary artery disease,hypertension,valvular disease,or congenital heart disease. MCM patients typically have hypertrophic or dilated cardiomyopathy. Arrhythmias and left ventricular myocardial noncompaction are less common,and heart failure may occur as the first symptom in some patients. Since MCM patients often have symptoms of multiple organ involvement,the symptoms are not specific and the diagnosis can be difficult. Thus,awareness of this disease must be increased in clinical settings. Treatments for MCM are mostly supportive and nonspecific. In this review,we summarize new advances in the diagnosis and management of MCM,with an improve the clinical management of this disease.

A case of noncompaction of left ventricular myocardium detected by Ventricular fibrillation

A case of noncompaction of left ventricular myocardium detected by Ventricular fibrillation

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 noncompaction 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 LVejection 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 transthoracic echocardiography in NCVM diagnosis. Therefore, this technique should be clinically performed to diagnose suspected NCVM.

Cardiomyopathy Phenotypes and Outcomes for Children With Left Ventricular Myocardial Noncompaction: Results From the Pediatric Cardiomyopathy Registry

BackgroundLeft ventricular noncompaction (LVNC) is a distinct form of cardiomyopathy characterized by hypertrabeculation of the left ventricle. The LVNC phenotype may occur in isolation or with other cardiomyopathy phenotypes. Prognosis is incompletely characterized in children. Methods and ResultsAccording to diagnoses from the National Heart, Lung, and Blood Institute–funded Pediatric Cardiomyopathy Registry from 1990 to 2008, 155 of 3,219 children (4.8%) had LVNC. Each LVNC patient was also classified as having an associated echocardiographically diagnosed cardiomyopathy phenotype: dilated (DCM), hypertrophic (HCM), restrictive (RCM), isolated, or indeterminate. The time to death or transplantation differed among the phenotypic groups (P = .035). Time to listing for cardiac transplantation significantly differed by phenotype (P < .001), as did time to transplantation (P = .015). The hazard ratio for death/transplantation (with isolated LVNC as the reference group) was 4.26 (95% confidence interval [CI] 0.78–23.3) for HCM, 6.35 (95% CI 1.52–26.6) for DCM, and 5.66 (95% CI 1.04–30.9) for the indeterminate phenotype. Most events occurred in the 1st year after diagnosis. ConclusionsLVNC is present in at least 5% of children with cardiomyopathy. The specific LVNC-associated cardiomyopathy phenotype predicts the risk of death or transplantation and should inform clinical management.

A case of noncompaction of left ventricular myocardium detected by cardiopulmonary arrest

A case of noncompaction of left ventricular myocardium detected by cardiopulmonary arrest

HEART's Original [症例] エプスタイン奇形に左室緻密化障害を合併した1成人例

HEART's Original [症例] エプスタイン奇形に左室緻密化障害を合併した1成人例

Hypertrophic Cardiomyopathy due to Mitochondrial Disease: Prenatal Diagnosis, Management, and Outcome

A case of prenatally diagnosed fetal hypertrophic cardiomyopathy is reported. The mother was referred to our department at 37 weeks' gestation because of suspected congenital heart disease. Prenatal echocardiography showed biventricular hypertrophy and pericardial effusion, without additional abnormalities. Postnatal echocardiography confirmed prenatal diagnosis. Neonatal EKG showed biventricular hypertrophy and Wolff-Parkinson-White syndrome. Skeletal muscle biopsy was consistent with mitochondrial oxidative phosphorylation defect involving a combined defect of respiratory complexes I and IV. Echocardiographic followup during the first year of life showed progressive regression of hypertrophy and evolution to left ventricular myocardial noncompaction.

Miocardiopatía no compactada en paciente con infección VIH

Nowadays, HIV-infected patients have higher rates of cardiovascular diseases than non-infected patients because these patients now live longer and have to deal with the complications of ageing. Noncompaction left-ventricular cardiomyopathy is a rare disorder characterized by the persistence of embryonic pattern of myoarchitecture with arrest in endomyocardial morphogenesis and hypertrabeculation with deep intertrabecular recesses. This condition has been described more often in infants and children and very rarely in adults. The disorder has been clinically associated with depressed ventricular function, heart failure and significant risk of life-threatening arrhythmia. The echocardiographic pattern is diagnostic and prognosis is similar to that of idiopathic dilated cardiomyopathy, with a mortality rate of 80% at 6 years. Isolated noncompaction of left ventricular myocardium should be included in the differential diagnosis of young patients with heart failure, especially in those with familial background of sudden death.

Isolated non- compaction of left ventricular myocardium . A 3D perspective”

Abstract Isolated non-compaction of left ventricular myocardium is an uncommon anomaly . It is characterized by prominent trabeculations and recesses communicating with the left ventricular cavity on 2D echocardiography . We studied a case of non compacted left ventricle by 3D echocardiography . The left ventricle appeared to be filled with tissue with ‘spongy’ appearance . Cropping the apex of the left ventricle showed a ‘sievelike’ pattern. These findings were in addition to the characteristic trabeculations and recesses seen on 2D echocardiography .

Cardiac MRI and Transthoracic Echocardiography of Left Ventricular Myocardial Noncompaction in A Patient with Congestive Heart Failure: A Case Report

We report a case of a 38-year-old male presenting with new-onset dyspnea, that was diagnosed as left ventricular noncompaction by transthoracic echocardiographic and cardiac MR. The tests revealed left ventricular systolic dysfunction with prominent trabeculations associated with deep intertrabecular recesses and an enddiastolic noncompacted to compacted ratio of 2.5 in the whole apical wall and mid-ventricular anterolateral and inferolateral walls. Delayed gadolinium contrast-enhanced MRI revealed subepicardial mid-wall hyperenhancement of the midventricular anteroseptal and inferoseptal walls, which suggested myocardial fibrosis. We review the pathophysiology, clinical characteristics, and diagnostic approach of the left ventricular noncompaction associated with congestive heart failure.

Isolated Noncompaction of Left Ventricular Myocardium With an Unusual Presentation of Angina Pectoris

A 45-year-old man presented to the hospital with typical chest pain compatible with myocardial infarction. An electrocardiogram showed left bundle branch block. The patient underwent urgent coronary angiography, which revealed no significant coronary artery disease. Echocardiography showed noncompaction of the left ventricular myocardium. This unusual case of angina occurring in a patient with isolated noncompaction of the left ventricle is discussed with a review of the literature.