Infant ; Humans ; Atrioventricular Block/therapy* ; Subclavian Vein ; Pacemaker, Artificial ; Endocardium

Little is known regarding cardiac involvement (CI) by neuromuscular disorders (NMDs). The purpose of this review is to summarise and discuss the major findings concerning the types, frequency, and severity of cardiac disorders in NMDs as well as their diagnosis, treatment, and overall outcome. CI in NMDs is characterized by pathologic involvement of the myocardium or cardiac conduction system. Less commonly, additional critical anatomic structures, such as the valves, coronary arteries, endocardium, pericardium, and even the aortic root may be involved. Involvement of the myocardium manifests most frequently as hypertrophic or dilated cardiomyopathy and less frequently as restrictive cardiomyopathy, non-compaction, arrhythmogenic right-ventricular dysplasia, or Takotsubo-syndrome. Cardiac conduction defects and supraventricular and ventricular arrhythmias are common cardiac manifestations of NMDs. Arrhythmias may evolve into life-threatening ventricular tachycardias, asystole, or even sudden cardiac death. CI is common and carries great prognostic significance on the outcome of dystrophinopathies, laminopathies, desminopathies, nemaline myopathy, myotonias, metabolic myopathies, Danon disease, and Barth-syndrome. The diagnosis and treatment of CI in NMDs follows established guidelines for the management of cardiac disease, but cardiotoxic medications should be avoided. CI in NMDs is relatively common and requires complete work-up following the establishment of a neurological diagnosis. Appropriate cardiac treatment significantly improves the overall long-term outcome of NMDs.
Arrhythmias, Cardiac ; Cardiomyopathies ; Cardiomyopathy, Dilated ; Cardiomyopathy, Restrictive ; Coronary Vessels ; Death, Sudden, Cardiac ; Diagnosis ; Endocardium ; Glycogen Storage Disease Type IIb ; Heart Arrest ; Heart Diseases* ; Heart* ; Muscular Diseases ; Myocardium ; Myopathies, Nemaline ; Myotonia ; Neuromuscular Diseases ; Pericardium ; Tachycardia, Ventricular

OBJECTIVE: To understand microstructural changes after myocardial infarction (MI), we evaluated myocardial fibers of rhesus monkeys during acute or chronic MI, and identified the differences of myocardial fibers between acute and chronic MI. MATERIALS AND METHODS: Six fixed hearts of rhesus monkeys with left anterior descending coronary artery ligation for 1 hour or 84 days were scanned by diffusion tensor magnetic resonance imaging (MRI) to measure apparent diffusion coefficient (ADC), fractional anisotropy (FA) and helix angle (HA). RESULTS: Comparing with acute MI monkeys (FA: 0.59 ± 0.02; ADC: 5.0 ± 0.6 × 10(-4) mm2/s; HA: 94.5 ± 4.4°), chronic MI monkeys showed remarkably decreased FA value (0.26 ± 0.03), increased ADC value (7.8 ± 0.8 × 10(-4) mm2/s), decreased HA transmural range (49.5 ± 4.6°) and serious defects on endocardium in infarcted regions. The HA in infarcted regions shifted to more components of negative left-handed helix in chronic MI monkeys (-38.3 ± 5.0°-11.2 ± 4.3°) than in acute MI monkeys (-41.4 ± 5.1°-53.1 ± 3.7°), but the HA in remote regions shifted to more components of positive right-handed helix in chronic MI monkeys (-43.8 ± 2.7°-66.5 ± 4.9°) than in acute MI monkeys (-59.5 ± 3.4°-64.9 ± 4.3°). CONCLUSION: Diffusion tensor MRI method helps to quantify differences of mechanical microstructure and water diffusion of myocardial fibers between acute and chronic MI monkey's models.
Anisotropy ; Coronary Vessels ; Diffusion* ; Endocardium ; Haplorhini ; Heart ; Ligation ; Macaca mulatta* ; Magnetic Resonance Imaging* ; Methods ; Myocardial Infarction* ; Water

The purpose of this study was to determine if contrast media would enhance visualization of the endocardium for assessment of left ventricle (LV) function. In addition, differences between pre- and post-contrast evaluation for the cardiac output measurements including the modified Simpson's method and automated contour tracking (ACT) method were examined. Ten clinically healthy adult beagle dogs (three males and seven females) between 2~3 years old and weighing 6.6~10.8 kg were used. Echocardiographic examinations were performed to compare pre- and postcontrast LV endocardium visualization using a segmental scoring method. Two different methods for measuring cardiac output were also compared. LV visualization was significantly enhanced in post-contrast echocardiography (p < 0.01). Significant differences between pre- and post-contrast measurements for the modified Simpson's method (p < 0.05) were also observed. No significant difference was found for the ACT method. Contrast echocardiography provides better LV chamber opacification and significantly improves wall segment visualization. Furthermore, contrast echocardiography for measuring cardiac output is helpful for the modified Simpson's method.
Adult ; Animals ; Cardiac Output* ; Contrast Media* ; Dogs ; Echocardiography* ; Endocardium ; Heart Ventricles ; Humans ; Male ; Research Design ; Ultrasonography*

Aged ; Coronary Angiography ; Endocardium ; pathology ; Heart Aneurysm ; diagnosis ; diagnostic imaging ; Humans ; Male ; Myocardial Infarction ; complications

Abnormal enhanced transmural dispersion of repolarization (TDR) plays an important role in the maintaining of the severe ventricular arrhythmias such as torsades de pointes (TDP) which can be induced in long-QT (LQT) syndrome. Taking advantage of an in vitro rabbit model of LQT2, we detected the effects of KN-93, a CaM-dependent kinase (CaMK) II inhibitor on repolarization heterogeneity of ventricular myocardium. Using the monophasic action potential recording technique, the action potentials of epicardium and endocardium were recorded in rabbit cardiac wedge infused with hypokalemic, hypomagnesaemic Tyrode's solution. At a basic length (BCL) of 2000 ms, LQT2 model was successfully mimicked with the perfusion of 0.5 μmol/L E-4031, QT intervals and the interval from the peak of T wave to the end of T wave (Tp-e) were prolonged, and Tp-e/QT increased. Besides, TDR was increased and the occurrence rate of arrhythmias like EAD, R-on-T extrasystole, and TDP increased under the above condition. Pretreatment with KN-93 (0.5 μmol/L) could inhibit EAD, R-on-T extrasystole, and TDP induced by E-4031 without affecting QT interval, Tp-e, and Tp-e/QT. This study demonstrated KN-93, a CaMKII inhibitor, can inhibit EADs which are the triggers of TDP, resulting in the suppression of TDP induced by LQT2 without affecting TDR.
Action Potentials ; drug effects ; Animals ; Anti-Arrhythmia Agents ; pharmacology ; Arrhythmias, Cardiac ; etiology ; physiopathology ; prevention & control ; Benzylamines ; pharmacology ; Calcium-Calmodulin-Dependent Protein Kinase Type 2 ; antagonists & inhibitors ; metabolism ; Electrocardiography ; Electrophysiologic Techniques, Cardiac ; Endocardium ; drug effects ; physiopathology ; Heart ; drug effects ; physiopathology ; In Vitro Techniques ; Long QT Syndrome ; complications ; Pericardium ; drug effects ; physiopathology ; Piperidines ; pharmacology ; Protein Kinase Inhibitors ; pharmacology ; Pyridines ; pharmacology ; Rabbits ; Sulfonamides ; pharmacology ; Torsades de Pointes ; etiology ; physiopathology ; prevention & control

Endomyocardial biopsy (EMB) is one of the reliable methods for the diagnosis of various cardiac diseases. However, EMB can cause various complications. The purpose of this study is to evaluate the complication of transfemoral EMB with both fluoroscopic and two-dimensional (2-D) echocardiographic guidance. A total of 228 patients (148 men; 46.0+/-14.6 yr-old) who underwent EMB at Kyungpook National University Hospital from January 2002 to June 2012 were included. EMB was performed via the right femoral approach with the guidance of both echocardiography and fluoroscopy. Overall, EMB-related complications occurred in 21 patients (9.2%) including one case (0.4%) with cardiac tamponade requiring emergent pericardiocentesis, four cases (1.8%) with small pericardial effusion without pericardiocentesis, two cases (0.9%) with hemodynamically unstable ventricular tachycardia (VT), one case (0.4%) with nonsustained VT, one case (0.4%) with tricuspid regurgitation, twelve cases (5.3%) with right bundle branch block. There was no occurrence of either EMB-related death or cardiac surgery. Left ventricular ejection fraction was significantly lower (32.0+/-18.7% vs 42.0+/-19.1%, P=0.023) and left ventricular end-diastolic dimension was larger (60.0+/-10.0 mm vs 54.2+/-10.2 mm, P=0.013) in patients with EMB related complications than in those without. It is concluded that transfemoral EMB with fluoroscopic and 2-D echocardiographic guidance is a safe procedure with low complication rate.
Adult ; Biopsy/*adverse effects ; Cardiac Tamponade/etiology ; Echocardiography/*adverse effects ; Endocardium/*ultrasonography ; Female ; Fluoroscopy/*adverse effects ; Heart Diseases/*pathology ; Heart Ventricles/metabolism ; Humans ; Male ; Middle Aged ; Pericardial Effusion/etiology ; Tachycardia, Ventricular/etiology ; Ventricular Function

The heart is frequently the site of metastasis of various malignant tumors. Lung cancer is one of the most common primary tumors of cardiac metastasis and usually involves the pericardium or epicardium by direct invasion and/or lymphatic spread. However, metastasis of lung cancer involving the left ventricular endocardium is extremely rare. We report a patient with adenocarcinoma of lung, metastasized to the left ventricular myocardium and endocardium, diagnosed by echocardiography and 18-fludeoxyglucose positron emission tomography-computed tomography.
Adenocarcinoma ; Echocardiography ; Electrons ; Endocardium ; Heart ; Heart Ventricles ; Humans ; Lung ; Lung Neoplasms ; Myocardium ; Neoplasm Metastasis ; Pericardium

Loeffler's endocarditis involves progressive eosinophilic infiltration of the endocardium, which leads to apical thrombotic obliteration of the ventricle and endomyocardial fibrosis, that may finally represent a characteristic feature of restrictive cardiomyopathy. This paper presents a case of a 44-year-old male with symptoms of dyspnea and peripheral hypereosinophilia, who was diagnosed with early stage Loeffler's endocarditis via multicardiac imaging modalities.
Cardiomyopathy, Restrictive ; Dyspnea ; Echocardiography ; Endocardium ; Endomyocardial Fibrosis ; Eosinophils ; Humans ; Hypereosinophilic Syndrome ; Male ; Thrombosis

Loeffler's endocarditis is generally caused by hypereosinophilic syndrome. It is a restrictive cardiomyopathy characterized with eosinophilia and eosionophilic penetration leading to the fibrous thickening of endocardium of both ventricles, apical obliteration and heart failure. We report a case of a 23-year-old male with Loeffler's endocarditis caused by Churg-Strauss syndrome. The echocardiogram showed that biventricular failure with large thrombus in left ventricle. His symptoms and typical echocardiographic findings markedly improved within 2 months after treatment for Churg-Strauss syndrome.
Cardiomyopathy, Restrictive ; Churg-Strauss Syndrome ; Endocardium ; Eosinophilia ; Heart Failure ; Heart Ventricles ; Humans ; Hypereosinophilic Syndrome ; Male ; Thrombosis ; Young Adult