OBJECTIVES The authors sought to describe characteristics, treatments, and in-hospital outcomes of hospitalized heart failure (HF) patients stratified by etiology. BACKGROUND Whether characteristics and outcomes of HF patients differ by cardiomyopathy etiology is unknown. METHODS The authors analyzed data on 156,013 hospitalized HF patients from 319 U.S. hospitals participating in Get With ...

To test the hypothesis that actin dysfunction leads to heart failure, patients with hereditary idiopathic dilated cardiomyopathy (IDC) were examined for mutations in the cardiac actin gene (ACTC). Missense mutations in ACTC that cosegregate with IDC were identified in two unrelated families. Both mutations affect universally conserved amino acids in domains of actin that attach to Z bands and i...

Because of an incomplete right bundle branch block, a severe right ventricular dilatation with no left ventricular cardiomyopathy was found in a 44-year-old man. Magnetic resonance and transesophageal echocardiography confirmed the finding and these tests also failed to find any potential cause. A pulmonary hemodynamic study and a coronary angiography were strictly normal. Lastly pulmonary func...

RESULTS Sudden arrhythmic death syndrome (SADS) was the most prevalent cause of death (n 1⁄4 149 [42%]). Myocardial disease was detected in 40% of cases, including idiopathic left ventricular hypertrophy (LVH) and/or fibrosis (n 1⁄4 59, 16%); arrhythmogenic right ventricular cardiomyopathy (ARVC) (13%); and hypertrophic cardiomyopathy (HCM) (6%). Coronary artery anomalies occurred in 5% of case...

AIMS The role of a novel non-contact mapping system (ESI 3000, Endocardial Solutions) to guide radiofrequency catheter ablation of untolerated ventricular tachycardia was investigated in 17 patients; 11 with prior myocardial infarction, three with arrhythmogenic right ventricular dysplasia, and three with idiopathic dilated cardiomyopathy. METHODS Twenty-seven monomorphic ventricular tachycar...

Peripartum cardiomyopathy is idiopathic heart failure occurring in the absence of any determinable heart disease during the last month of pregnancy or the first 5 months postpartum. The incidence varies worldwide but is high in developing nations; the cause of the disease might be a combination of environmental and genetic factors. Diagnostic echocardiographic criteria include left ventricular ...

Peripartum cardiomyopathy (PPCM) is an idiopathic cardiomyopathy presenting with heart failure (HF) secondary to left ventricular systolic dysfunction towards the end of pregnancy or in the months following delivery, where no other cause of HF is found. The symptoms and signs of this rare disorder mimic those of the physiological changes of pregnancy or other cardiovascular diseases. Consequent...

BACKGROUND Fabry disease is a treatable lysosomal storage disorder, which is often misdiagnosed or belatedly diagnosed. METHODS AND RESULTS To determine the disease incidence in the Taiwan Chinese population, a Fabry disease newborn screening study was initiated. A total of 110 027 newborns were screened by assaying the alpha-galactosidase A (alpha-Gal A) activity using dry blood spots. Low p...

BACKGROUND Contemporary therapeutic options have led to substantial improvement in survival of patients with heart failure. However, limited evidence is available specifically on idiopathic dilated cardiomyopathy. We thus examined changes in prognosis of a large idiopathic dilated cardiomyopathy cohort systematically followed during the past 30 years. METHODS AND RESULTS From 1977 to 2011, 60...

We describe a case of a patient with idiopathic dilated cardiomyopathy and cardiac conduction abnormalities who presented a strong family history of sudden cardiac death. Genetic screening of lamin A/C gene revealed in proband the presence of a novel missense mutation (R189W), near the most prevalent lamin A/C mutation (R190W), suggesting a "hot spot" region at exon 3.