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

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

Adult ; Cardiomyopathies ; diagnostic imaging ; surgery ; Echinococcosis ; diagnostic imaging ; surgery ; Echinococcosis, Hepatic ; diagnostic imaging ; surgery ; Female ; Humans ; Mediastinum ; parasitology ; Radiography ; Thoracotomy ; methods

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

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

OBJECTIVETo apply echocardiography in diagnosis of isolated noncompaction of ventricular myocardium (INVM) in adults.

METHODSSix patients with INVM underwent echocardiographic examination using HP5500 ultrasound system with the frequency of 2 approximately equals 4 MHz, and the observation was focused on ventricularmyocardium and endocardium at one-third of the apex.

RESULTSAll 6 patients showed typical echocardiographic images characterized by an altered structure of the ventricular myocardium with extremely thickened, hypokinetic segments consisting of two layers: a thin, compacted epicardium and an extremely thickened endocardium, resulting in an extremely thickened ventricular wall.

CONCLUSIONEchocardiography should be the first choice to be applied in diagnosis of INVM, which is a distinct entity of specific cardiomyopathy in adults.

Adult ; Cardiomyopathies ; congenital ; diagnostic imaging ; pathology ; Echocardiography, Doppler, Color ; Female ; Heart Ventricles ; diagnostic imaging ; pathology ; Humans ; Male ; Middle Aged ; Myocardium ; pathology

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

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

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

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