a 76-yr-old man with unipolar ventricular pacemaker, previously implanted in the right ventricular apex, presented with syncopal attack and twitching of the right pectoralis muscle. he finally was diagnosed as having twiddler's syndrome. the patient had a pulse rate of 36/min and cannon waves in the neck veins. chest radiography showed a dislodged pacing lead completely wrapped around the pulse...

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an autosomal dominant genetic form of cardiomyopathy (CM), which primarily affects the right ventricle (RV) and results in life threatening ventricular arrhythmias and sudden cardiac death (SCD). Diagnosis is difficult due to the broad spectrum of phenoltypic variations, especially in the early stage. Clinical suspicion shoul...

Noncompaction of the ventricular myocardium is a recently recognized genetic cardiomyopathy. The left ventricle is the most affected site, but right ventricular involvement has been reported in some cases. Diagnosis is made with 2-dimensional echocardiography or cardiac magnetic resonance imaging. The major clinical manifestations are heart failure, arrhythmias and embolic events. A 20-year old...

OBJECTIVES This study sought to examine the changes in ventricular function of hypoplastic left heart syndrome (HLHS) between the first 2 stages of surgical palliation. BACKGROUND The mortality risk between first and second stages of surgical palliation in HLHS remains high. Right ventricular (RV) dysfunction predicts mortality. Postulated mechanisms include a maladaptive contraction pattern,...

introduction: cardiovascular abnormalities have been reported in liver cirrhosis (lc). in these patients, cardiac symptoms and physical signs occur as the liver functions worsen. cirrhosis is associated with hyper dynamic circulation and beta-adrenergic system changes responsible for the cardiovascular abnormalities. the purpose of the present study was to explore the echocardiographic findings...

UNLABELLED The utility of (99m)Tc-tetrofosmin myocardial SPECT for assessment of pulmonary hypertension and right ventricular thickness has not been well studied. We hypothesized that a ratio of right ventricular activity to left ventricular activity (RV/LV uptake ratio) from SPECT myocardial perfusion images could identify the presence of increased right ventricular wall thickness and elevated...

Background ARVC is a genetically determined cardiomyopathy which typically manifests clinically during the second to fourth decade of life. The diagnosis is made using a scoring system of signs and symptoms, known as the revised task force criteria (rTFC). MRI has recently been shown to be of little added value in the diagnosis of ARVC in adults. Its role in the pediatric age group is unclear. ...

Beta-blockers are used to treat acquired heart failure in adults, though their role in early muscular dystrophy cardiomyopathy is unclear. We treated 2 different dystrophic mouse models which have an associated cardiomyopathy (mdx: model for Duchenne Muscular Dystrophy, and Sgcd-/-: model for limb girdle muscular dystrophy type 2F) and wild type controls (C57 Bl10) with the beta blocker metopro...

A 20-year-old Japanese woman with systemic sclerosis was evaluated for the progressive aggravation of chest pain, palpitations and dyspnea. Cardiac magnetic resonance imaging revealed diffuse wall thinning, segmental dyskinesis and late gadolinium enhancement of the right ventricular (RV) myocardium. Cardiac catheterization demonstrated no pulmonary hypertension. 24-hour electrocardiography mon...

Purpose Right ventricular (RV) function is the most important determinant of survival in patients with pulmonary hypertension (PH), thus monitoring of RV function is critical. However, RV regional function assessment is challenging using current MR tagging techniques. Thus, the purpose was to evaluate regional longitudinal ventricular deformation (ELL) acquired by free breathing single heart-be...