Lamin A/C gene mutations can be associated with myocardial diseases, usually characterized by dilated cardiomyopathy and/or arrhythmic disorders. Phenotypic penetrance is age-related but expression is extremely heterogeneous, so that muscular and arrhythmic disease can be present in combination in the same patient, or one phenotypic manifestation can appear earlier than the other or even not be...

Sarcoidosis is a systemic disease of unknown aetiology characterised by the formation of noncaseating epitheloid cell granulomas, which can occur in virtually any organ. Cardiac involvement is of critical importance, due to the poor prognosis if this organ manifestation is left undiagnosed and untreated. Early initiation of therapy seems to be associated with a good prognosis; therefore, the di...

Echocardiography was performed in 16 children undergoing bone marrow transplantation (BMT) for mucopolysaccharidoses. Cardiac involvement before BMT was detected in seven (44%). One year after BMT (11 patients/five with cardiac involvement), left ventricular restriction resolved in 2/3 patients and hypertrophy in one. In the remainder, at mean follow up of 2.5 years, no progression of preexisti...

Parent involvement (PI) in school is associated with more positive academic performance and social competence in children. However, there are inadequacies in current measures of PI and a need for a better understanding of predictors of PI. In this study, measures were obtained from a normative sample of 387 children in kindergarten and first grade from high-risk neighborhoods in 4 different sit...

OBJECTIVE Churg-Strauss syndrome (CSS) is a rare form of systemic vasculitis. Previous studies showing cardiac involvement in CSS patients were limited in the number of patients and were often based solely on clinical manifestations. The aim of the present study was to determine in detail the incidence of cardiac involvement in a large population of ambulatory CSS patients. METHODS Thirty-two...

thrombotic thrombocytopenic purpura (ttp) is a rare syndrome affecting multiple organs. there is no sufficient evidence regarding the clinical cardiac manifestations of ttp. nonetheless, pathologic cardiac involvement is quite frequent in acute ttp, which is predominantly manifested as myocardial necrosis due to coronary arteriolar microthrombosis. the present case report describes a 43-year-ol...

cardiac hydatid cyst (chc) is a rare disease that was endemic in some regions especially in sheep-raising areas. the most commonly accepted theory for the routes of heart involvement in hydatid cyst was infestation by the hexacanthus embryo through coronary arteries. here, we describe a case with the primitive cardiac hydatid cyst located around the pulmonary valve (pv) and main pulmonary arter...

Background Autopsy studies report a cardiac involvement in sarcoidosis as high as 20-60%. Clinically a much lower detection rate of approximately 5% has been reported. Since cardiac events are the second most frequent cause of death, a high rate of undetected cases of cardiac involvement can be presumed, potentially leading to a fatal event as the fist manifestation. Previous studies have shown...