Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is characterized by progressive fatty and fibrous replacement. A female in her 70s, suddenly found in cardiopulmonary arrest. The heart weighed 452 g and yellow discoloration was observed. Histological examination revealed the replacement of the right ventricular myocardium by adipose tissue and fibrosis. The cause of death was ...

We have observed in 11 infants, aged 2 years or less, a distinct clinicopathologic lesion responsible for tachyarrhythmias that were fatal in 96% (25/26) of previously reported cases. Nine of the 11 patients, who underwent electrophysiologic mapping and surgical excision of the lesion, have survived, with follow-up periods ranging from 1 month to 6 years. The morphologic findings in these 11 pa...

Metabolic cardiomyopathies include amino acid, lipid and mitochondrial disorders, as well as storage diseases. A number of metabolic disorders are associated with both myopathy and cardiomyopathy. These include the glycogen storage diseases, ie, acid maltase deficiency (infantile, childhood, and adult onset), McArdle disease, and debrancher and brancher deficiencies. Disorders of lipid metaboli...

Neonatal lupus is a rare congenital syndrome resulting from placental passage of maternal antibodies against SSA/Ro and/or SSB/La to the fetus. It generally benign condition, cardiac involvement determining prognosis. NNL rarely manifested by isolated cardiomyopathy. The subsequent evolution towards systemic and unpredictable. We report case daughter non-consanguineous marriage with healthy par...

Introduction: Differentiating between ischemic cardiomyopathy (ICM) and idiopathic dilated cardiomyopathy (IDCM) is important as coronary revascularization can improve prognosis in the ischemic subgroup. Due to inherent problems of coronary angiography in patients with depressed ejection fraction (EF) introducing a noninvasive tool to diagnose those who will benefit from angiography seems...

Primary amyloidosis is a systemic disorder characterized by the clonal production and tissue deposition of immunoglobulin light chain (LC) proteins. Congestive heart failure remains the greatest cause of death in primary amyloidosis, due to the development of a rapidly progressive amyloid cardiomyopathy. Amyloid cardiomyopathy is largely unresponsive to current heart failure therapies, and is a...