We report a pedigree in which six males died of cardiac failure within the first eight months of life. These males were related through healthy females, as with X linked recessive inheritance. There was no consanguinity. None of the affected boys had an anatomical cardiac abnormality. In two affected brothers, histological evidence for endomyocardial fibroelastosis was documented, and in one of...

Familial cardiac conduction disorder of a progressive nature has been reported in adult and paediatric patients. An underlying cardiomyopathy was found in most of these patients, either of hypertrophic, restrictive or dilated variety. Systemic diseases like juvenile rheumatoid arthritis and other collagen disorders and familial dysautonomia infrequently have associated conduction abnormalities....

Up to 10% of all pregnancies in the US are complicated by diabetes. Among them, 0.2 0.5 % individuals had type 1 diabetes (T1DM). Preterm birth, preeclampsia, macrosomia, shoulder dystocia, intrauterine fetal death, growth restriction, cardiac and renal abnormalities, as well uncommon neural disorders including sacral agenesis, heightened risks for affected T1DM. It has been demonstrated that p...

Amyloidosis is caused by deposition of abnormal amyloid fibrils with the feared consequence end stage organ failure. Cardiac amyloidosis (CA) an increasingly recognized antecedent cardiomyopathy. CA classified into transthyretin variants (ATTRwt – wild type and ATTRv genetic variant) a light chain variant (AL); each these can be associated unique electrophysiologic abnormalities. Pacing indicat...

BACKGROUND Kawasaki disease (KD) causes systemic vasculitis and coronary aneurysms. It frequently results in electrocardiographic (ECG) abnormalities of short duration. Cardiac sequelae persist beyond the acute stage in a few patients. There are many areas to be investigated concerning the effects on the vascular system of patients suffering from KD and its sequelae. METHODS The cumulative in...

We report a series of five patients with congenital heart defects in whom a prenatal diagnosis of 22q11 deletion has been made. The accurate cardiac and cytogenetic diagnoses were made between 20 and 23 weeks' gestation in all cases and the cardiac findings were all confirmed postnatally. The cardiac abnormalities included tetralogy of Fallot with absent pulmonary valve, pulmonary atresia with ...

Cardiac involvement is one of the most significant factors in the poor clinical outcome of polymyositis. The case of a 39 year old African American woman with polymyositis, cardiomyopathy, and severe heart failure who had orthotopic heart transplantation is described. Review of the literature reveals that cardiac manifestations of polymyositis are frequent and include conduction system abnormal...

Background Throughout the literature, cardiac MRI (CMR) has become an important diagnostic tool in evaluating congenital cardiac abnormalities. In heterotaxy syndrome, the axis of the body during fetal development fails to rotate correctly resulting in complex cardiovascular abnormalities. We hypothesize that CMR aids in the detection of anatomical and physiological anomalies presented in patie...

Multikinase inhibitors (e.g. Sorafenib), phosphodiesterase-5 inhibitors (e.g. Tadalafil), and endothelin-1 receptor blockers (e.g. Macitentan) exert influential protection in a variety of animal models of cardiomyopathy; however, their effects on thyroxin-induced cardiomyopathy have never been investigated. The goal of the present study was to assess the functional impact of these drugs on thyr...