Cardiomyopathies ; etiology ; Humans ; Polymyositis ; complications

Cardiomyopathies ; diagnosis ; etiology ; Child ; Humans

Cardiomyopathies ; diagnosis ; etiology ; therapy ; Humans

Most patients with liver cirrhosis have hyperdynamic circulatory alterations with increased cardiac output, and decreased systemic vascular resistance and arterial pressure. But, in spite of the increased resting cardiac output, ventricular contractile response to stressful stimuli is attenuated in cirrhotic patients which is termed as cirrhotic cardiomyopathy. The prevalence of cirrhotic cardiomyopathy remains unknown at present. Clinical features include structural, histological, electrophysiological, systolic and diastolic dysfunction. Multiple factors are considered as responsible, including impaired beta-adrenergic receptor signal transduction, abnormal membrane biophysical characteristics, and increased activity of cardiodepressant systems mediated by cGMP. Generally, cirrhotic cardiomyopathy with overt severe heart failure is rare. However, major stresses on the cardiovascular system such as liver transplantation, infections and insertion of transjugular intrahepatic portosystemic shunts (TIPS) can unmask the presence of cirrhotic cardiomyopathy and thereby convert latent to overt heart failure. Cirrhotic cardiomyopathy may also contribute to the pathogenesis of hepatorenal syndrome and circulatory failure in liver cirrhosis. Because of the marked paucity of treatment studies, current recommendations for management are empirical, nonspecific measures. Further studies for pathogenesis and new therapeutic strategies in this area are required.
Cardiomyopathies/*diagnosis/*etiology/therapy ; Humans ; Liver Cirrhosis/*complications ; Prognosis

Acute Disease ; Aged ; Cardiomyopathies ; diagnosis ; etiology ; pathology ; China ; Female ; Humans

Cardiomyopathies ; classification ; diagnosis ; etiology ; Cardiomyopathy, Dilated ; diagnosis ; etiology ; Cardiomyopathy, Hypertrophic ; diagnosis ; etiology ; Child ; Humans ; Pediatrics ; Suggestion

This study determined the levels of serum soluble intercellular adhesion molecule-1 (sICAM-1) and soluble vascular cell adhesion molecular-1 (sVCAM-1) in patients with different types of Keshan disease (KD), examined the relationship between Coxsackie B virus-specific IgM antibody (CBV-IgM) and sICAM-1 or sVCAM-1 in KD patients, and investigated the role of these adhesion molecules in the pathogenesis of KD and their clinical implications. The levels of serum sICAM-1, sVCAM-1 and CBV-IgM were measured by using enzyme-linked immunosorbent assay in 22 patients with chronic Keshan disease (CKD), 27 with latent Keshan disease (LKD) and 28 healthy controls. The subjects in different groups were adjusted for sex and age. Echocardiography was adopted to determine left ventricular ejection fraction (LVEF) in 22 patients with CKD. The results showed that CKD patients had significantly higher levels of sICAM-1 and sVCAM-1 than LKD patients and healthy controls (P<0.01 for all). And there was significant difference in the levels of the 2 adhesion molecules between LKD patients and healthy controls (P<0.05). A negative correlation was found between LVEF and sICAM-1 or sVCAM-1 in CKD patients. The percentage of CBV-specific IgM positive individuals in KD patients was significantly higher than that of healthy controls. In CVB-specific IgM positive patients, the levels of serum sICAM-1 and sVCAM-1 were significantly greater than those in CBV-specific IgM negative counterpart. It was concluded that the increase in the levels of sICAM-1 and sVCAM-1 suggests the progression of inflammation in KD. sICAM-1 and sVCAM-1 can promote the development of myocardial pathology and lead to poor myocardial function. The increased serum sICAM-1 and sVCAM-1 in KD patients may be related to CBV infection.
Cardiomyopathies/*blood ; Cardiomyopathies/etiology ; Cardiomyopathies/*virology ; Coxsackievirus Infections/*complications ; Enterovirus B, Human ; Intercellular Adhesion Molecule-1/*blood ; Selenium/blood ; Vascular Cell Adhesion Molecule-1/blood ; Young Adult

Emery-Dreifuss muscular dystrophy (EDMD) and limb-girdle muscular dystrophy type 1B (LGMD1B) are characterized by cardiac dysrhythmias, late-onset cardiomyopathy, slowly progressive skeletal myopathy and contractures of the neck, elbows and ankles. The causative mutation is either in the emerin gene (X-linked recessive EDMD) or lamin A/C gene (autosomal dominant EDMD2 or LGMD1B). We report three cases of EDMD, EDMD2 and LGMD1B. A 14-yr-old boy showed limitation of cervical flexion and contractures of both elbows and ankles. Sinus arrest with junctional escape beats was noted. He was diagnosed as X-linked recessive EDMD (MIM 310300). A 28-yr-old female showed severe wasting and weakness of humeroperoneal muscles. Marked limitation of cervical flexion and contractures of both elbows and ankles were noted. Varying degrees of AV block were noted. She was diagnosed as autosomal dominant EDMD2 (MIM 181350). A 41-yr-old female had contractures of both ankles and limb-girdle type muscular dystrophy. ECG revealed atrial tachycardia with high grade AV block. She was diagnosed as autosomal dominant LGMD1B (MIM 159001). Cardiac dysrhythmias in EDMD and LGMD1B include AV block, bradycardia, atrial tachycardia, atrial fibrillation, and atrial standstill, causing sudden death necessitating pacemaker implantation. Cardiologists should know about these unusual genetic diseases with conduction defects, especially in young adults.
Adolescent ; Adult ; Arrhythmia/*etiology ; Cardiomyopathies/*etiology ; Female ; Humans ; Male ; Muscle, Skeletal/*pathology ; Muscular Dystrophies, ; Muscular Dystrophy,

Ventricular premature complexes (VPCs) are known to be one of the most benign cardiac arrhythmias when they occur in structurally normal hearts. We experienced a 32-year old man who presented with dyspnea, palpitations and very frequent VPCs (31% of the total heart beats). Echocardiography revealed a dilated left ventricle (LV 66 mm at end-diastole and 57 mm at end-systole) and a decreased ejection fraction (34%). Very frequent VPCs had been detected 10 years previously and he underwent a failed radiofrequency catheter ablation (RFCA) procedure at that time. The patient had been treated with heart failure medications including betablockers, ACE inhibitors and spironolactone for the two most recent years. Six months after we eliminated these VPCs with a second RFCA procedure, the heart returned to normal function and size. Long standing and very frequent VPCs could be the cause of left ventricular dysfunction in a subset of patients who suffer with dilated cardiomyopathy, and RFCA should be the choice of therapy for these patients.
Ventricular Premature Complexes/*complications ; Tachycardia, Ventricular/*etiology/therapy ; Male ; Humans ; *Catheter Ablation ; Cardiomyopathies/*etiology/therapy ; Adult

Cardiomyopathies ; classification ; diagnosis ; etiology ; Cardiomyopathy, Dilated ; diagnosis ; etiology ; Child ; Child, Preschool ; China ; Humans