This article summarizes and analyzes the clinical features and gene mutation characteristics of children with noncompaction of the ventricular myocardium (NVM). For the 6 children with NVM (4 boys and 2 girls), the age of onset ranged from 3 months to 12 years. Of the 6 children, 5 had arrhythmia, 3 had cardiac insufficiency, 1 had poor mental state, and 1 had chest distress and sighing. NVM-related gene mutations were detected in 4 children, among whom 2 had
Cardiomyopathies ; Child ; Child, Preschool ; Echocardiography ; Female ; Heart Ventricles/diagnostic imaging* ; Humans ; Infant ; Male ; Mutation ; Myocardium

Amyloidosis/diagnostic imaging* ; Cardiomyopathies/diagnostic imaging* ; Diagnostic Imaging ; Humans ; Prealbumin/genetics*

PURPOSE: As cardiomyopathy is more prevalent and currently the leading cause of death in Duchenne muscular dystrophy (DMD), early detection of myocardial involvement is important. The purpose of this study was to analyze myocardial strain in DMD children, for the possibility of early detection of myocardial dysfunction. MATERIALS AND METHODS: We reviewed medical records of DMD patients who were >10 years of age (15.6±1.6 years, 12.5-18 years), from March 2013 to June 2014. Data of 24 DMD children who underwent echocardiography with three-layer specific myocardial strain were compared with 24 controls (age: 9.3±4.0 years, 5.5-17 years). RESULTS: Epicardial longitudinal strain was lower in DMD (DMD: -9.3±3.8%; control: -12.3±4.3%; p=0.012). Radial strain (DMD: 24.1±11.1%; control: 37.3±25.9%; p=0.027) and strain rate (SR) (DMD: 1.68±0.91; control: 2.42±0.84; p=0.006) on parasternal short axis view were lower in DMD. Circumferential strains in the endocardium (DMD: -17.5±4.7%; control: -24.2±5.3%; p<0.001), myocardium (DMD: -12.7±3.8%; control: -18.0±4.0%; p<0.001), and epicardium (DMD: -8.4±4.0%; control: -12.2±5.0%; p=0.006) were significantly decreased in DMD. Circumferential SRs were lower in the endocardial (DMD: -1.46±0.38; control: -1.78±0.27; p=0.002) and myocardial layers (DMD: 1.02±0.27; control: -1.28±0.22; p=0.001). CONCLUSION: In DMD patients, deteriorations in myocardial circumferential strain might be an indicator for predicting cardiomyopathy.
Adolescent ; Cardiomyopathies/*diagnostic imaging/*etiology ; Case-Control Studies ; Child ; Child, Preschool ; Early Diagnosis ; *Echocardiography ; Female ; Humans ; Male ; Muscular Dystrophy, Duchenne/*complications/*diagnostic imaging ; Predictive Value of Tests

The elastic and functional coupling of heart and vessels makes the stroke work (SW) of the heart optimal. Speckle tracking imaging (STI) can evaluate the myocardial strain and function. We studied ventricular-vascular coupling in 80 diabetic patients with different systolic function using STI. The patients were divided into two groups according to ejection fraction (EF): the diabetes mellitus with normal EF (DMN) group and the diabetes mellitus with abnormal EF (DMA) group. Forty-two volunteers served as control group. The relative wall thickness (RWT), left ventricular mass index (LVMI), stroke volume (SV), SW, rate-pressure product (RPP), systemic vascular resistance index (SVRI), left ventricular end-systolic elastance (Ees), effective arterial elasticity (Ea) and ventricular-vascular coupling index (VVI) were measured and calculated by conventional echocardiography. The longitudinal strain (LS) at basement (LSBA), papillary muscle (LSPM) and cardiac apex (LSAP) was assessed with STI. It was found: (A) compared with control group, in DMN and DMA groups, LSBA, LSPM and LSAP decreased, and they were lower in DMA group. (B) VVI, RPP and SVRI increased, and they were higher in DMN group; Ees decreased, and it was lower in DMA group. (C) LSBA, LSPM, and LSAP had negative correlation with VVI. LSAP, RWT, LVMI and SW were independent predictors for VVI. The area under the receiver operating characteristic (ROC) curves was used for identification of DMA and DMN with LSBA, LSPM, and LSAP, and the area under the ROC of LSAP was the largest. This study supports that myocardial LS could reflect the ventricular-vascular coupling. Different segments had an order to "respond to" the state of the coupling, and the cardiac apex might be the earliest.
Adult ; Aged ; Cardiac-Gated Single-Photon Emission Computer-Assisted Tomography ; Diabetes Mellitus, Type 2 ; diagnostic imaging ; physiopathology ; Diabetic Cardiomyopathies ; diagnostic imaging ; physiopathology ; Female ; Humans ; Male ; Middle Aged ; Radiography ; Stroke Volume ; Vascular Resistance ; Ventricular Function

Adult ; Cardiac Imaging Techniques ; Cardiomyopathies ; diagnostic imaging ; Clinical Protocols ; Diet, Carbohydrate-Restricted ; Diet, High-Fat ; Female ; Fluorodeoxyglucose F18 ; Humans ; Middle Aged ; Multimodal Imaging ; Positron-Emission Tomography ; Radiopharmaceuticals ; Sarcoidosis ; diagnostic imaging ; Tomography, X-Ray Computed

Aspirin ; therapeutic use ; Bisoprolol ; therapeutic use ; Cardiomyopathies ; chemically induced ; etiology ; Cardiotonic Agents ; adverse effects ; Chest Pain ; diagnostic imaging ; Dobutamine ; adverse effects ; Echocardiography, Stress ; adverse effects ; Enalapril ; therapeutic use ; Humans ; Male ; Middle Aged ; Simvastatin ; therapeutic use

Cardiomyopathies ; diagnosis ; Diagnostic Imaging ; Humans ; Ventricular Dysfunction, Left ; diagnosis

OBJECTIVETo evaluate the left ventricular systolic asynchrony in patients with uremic myocardiopathy (UM) using tissue synchronization imaging (TSI).

METHODSUltrasound system with TSI and Q-analyze software were used. Thirty-five patients with UM were enrolled in the study,and thirty normal subjects were included as the control group.

RESULTThe total and mean time to peak velocity (Tc) corrected by the heart rate of all segments in UM group were longer than those in the control group (P<0.05), and the time to peak velocity of most segments in UM group was also longer (P<0.05). Delayed time to peak velocity was found in 175 (175/420) segments in UM group and left ventricular systolic asynchrony was detected in 65.7% (23/35).

CONCLUSIONTSI can detect the ventricular systolic asynchrony in patients with uremic myocardiopathy and provide reliable parameters for clinical management.

Adult ; Cardiomyopathies ; etiology ; physiopathology ; Case-Control Studies ; Echocardiography ; methods ; Female ; Humans ; Male ; Middle Aged ; Systole ; physiology ; Uremia ; complications ; Ventricular Dysfunction, Left ; diagnostic imaging ; physiopathology

OBJECTIVETo analyze the clinical features and outcome of patients with noncompaction of ventricular myocardium (NVM).

METHODSClinical manifestations, electrocardiograms and echocardiographies data were analyzed in 18 patients with NVM. Mean follow-up period was (11 +/- 5) months.

RESULTSThe patients aged from 1.5 to 71 years, 66.7% patients were males, familial history was observed in 2 cases, congestive heart failure was present in 14 cases, thromboembolic event occurred in 1 patient, arrhythmia induced syncopes were diagnosed in 2 patients and 1 patient was asymptomatic. Abnormal electrocardiograms were observed in all patients, including premature ventricular beats (7 cases), heart block (4 cases), and atrial fibrillations (4 cases). Echocardiographies showed that noncompaction of ventricular myocardium localized in the left ventricle in 17 patients, and right ventricle in 1 patient. The extension of noncompaction myocardium was predominantly at the apex (72%). N/C was 2.3 - 3.1. EF was less than 50% in 15 patients. Hypokinetic movements were observed in both noncompacted and compacted segments. During the follow-up, 1 patient with congestive heart failure received heart transplantation. ICD was implanted in one patient due to ventricular tachycardia. One patient suffered from sudden cardiac death.

CONCLUSIONSThe most common clinical presentations of NVM are congestive heart failure, cardiac arrhythmias, and thromboembolism. Echocardiography is considered as the best tool for the diagnosis of NVM. ICD, heart transplantation and anticoagulation therapy could improve the prognosis of patients with NVM in selected cases.

Adolescent ; Adult ; Aged ; Arrhythmias, Cardiac ; diagnosis ; Cardiomyopathies ; diagnosis ; diagnostic imaging ; Child ; Child, Preschool ; Echocardiography ; Female ; Heart Failure ; diagnosis ; Heart Ventricles ; abnormalities ; Humans ; Infant ; Male ; Middle Aged ; Myocardium ; pathology ; Young Adult

OBJECTIVEEarlier studies have confirmed that mesenchymal stem cells (MSCs) can transdifferentiate into myocytes and improve heart function in 2 weeks. But the mechanism is not clear. In this study, the mechanism of improvement of heart function after transplantation of MSCs was examined with suppression subtractive hybridization (SSH).

METHODSMSCs were isolated from thighone and tibia of Wistar rats, purified by adhesion-screening method, and expanded in vitro. Intraperitoneal injection of doxorubicin (at 2.5 mg/kg/time and total doses of 15 mg/kg) established cardiomyopathy models. MSCs were transplanted into cardiomyocytes. The differential genes between tester (rats with cardiomyopathy that were injected with MSCs) and driver (rats with cardiomyopathy that were injected with equivalent volume of culture medium) were screened with suppression subtractive hybridization.

RESULTSAfter 4 weeks of intraperitoneal injection of doxorubicin, left ventricular ejection fraction (LVEF) and left ventricular fractional shortening (LVFS) decreased by 26.48% and 40.61%, respectively (P < 0.01), as compared with those of normal group. Cardiomyopathy model was established successfully. And the heart function of the rats with cardiomyopathy was significantly improved after transplantation. Sixteen gene fragments were detected, and 12 of them were up-regulated in testers. They were rattus norvegicus mitochondrial BN/SsNHsdMCW, rattus norvegicus strain mitochondrial F344 X BN F1, rattus norvegicus mitochondrion H(+)-ATP synthase alphase subunit (Atp5al) mRNA, rattus norvegicus BHE/Cdb tRNA-Lys gene, rat mitochondrial H(+)-ATP synthase alpha subunit mRNA, rattus norvegic (wild-caught animal) complete mitochondrial genome, rattus norvegic clone BB.1.4.1 unknown Glu-Pro dipeptide repeat protein mRNA, Arabidopsis thaliana transgenic line C DNA, rat mitochondrial ATP synthase beta subunit mRNA, rattus norvegic mitochondrial genome, rat cardiac troponin T mRNA and rat mRNA for beta-globin. Four gene fragments were down-regulated in testers. They were rat mRNA for sarcomeric mitochondrial creatine kinase, rat mRNA for ribosomal phosphoprotein P2, rat alpha-crystallin B chain mRNA and rattus norvegicus NADH-ubiquinone oxidoreductase Fe-S protein 7 mRNA.

CONCLUSIONThe expression of the genes relating to mitochondrial synthesizing and contracting proteins synthesizing increased after MSC transplantation. The genes might enhance energy synthesis and promote MSC transdifferentiate into myocytes, and then improve heart function of rats with cardiomyopathy.

Animals ; Antibiotics, Antineoplastic ; toxicity ; Bone Marrow Transplantation ; methods ; Cardiomyopathies ; diagnostic imaging ; genetics ; surgery ; Cell Differentiation ; Cells, Cultured ; Disease Models, Animal ; Down-Regulation ; Doxorubicin ; toxicity ; Female ; Mesenchymal Stem Cell Transplantation ; methods ; Mitochondria ; genetics ; Myocytes, Cardiac ; RNA, Messenger ; Rats ; Rats, Wistar ; Treatment Outcome ; Ultrasonography ; Up-Regulation ; Ventricular Function, Left