AIMS Cardiac involvement, including progressive cardiomyopathy, is common in Fabry disease and is a leading cause of premature mortality. We sought to determine if tissue Doppler imaging (TDI) could identify Fabry disease patients at risk for the development of cardiomyopathy and if enzyme replacement therapy (ERT) with agalsidase alfa might slow or prevent the progression of cardiac involvemen...

Objectives: To clarify the value of cycle dependent variation of myocardial integrated backscatter (CV-IB) analysis, which non-invasively measures acoustic properties of the myocardium, for early detection of cardiac involvement in patients with sarcoidosis. Methods: The study population consisted of 22 consecutive patients with biopsy proven sarcoidosis who did not have any abnormal findings o...

BACKGROUND Cardiac assessment was not done routinely in Duchenne (DMD) and Becker muscular dystrophy (BMD) patients in Northern region of England while evidence was gathering on progressive cardiomyopathy in these patients. We wanted to find out the prevalence, progression and clinical features of cardiac involvement in Duchenne and Becker muscular dystrophy. METHODS It is a retrospective rev...

BACKGROUND Cardiac involvement in systemic sclerosis (SSc) is more prevalent than previously thought. In this study, the frequency and severity of cardiovascular involvement were assessed in SSc patients referred to Firouzgar Hospital. METHODS Fifty-eight patients with SSc, selected from the data bank of SSc patients, were reviewed for the frequency and severity of 8 organ involvements in thi...

We report the case of a 17-year-old man with precursor B-lymphoblastic lymphoma involving an intracardiac mass and myocardial infiltration. Intensified chemotherapy followed by autologous peripheral blood stem cell transplantation resulted in long-term complete remission for over 5 years. As the most frequent sites of B-lymphoblastic lymphoma involvement are the skin, soft tissue, bone, and lym...

THOUGH most patients with secondary neoplastic involvement of the heart or pericardium seem to manifest no symptoms or signs of such involvement, a sizable proportion do have actual impairment of cardiac funetion. Refinements in thoracic, and specifically cardiac, surgery, in roentgen therapy, and in the use of isotopic and chemotherapeutic agents have made definite palliation available for met...

Background Sarcoidosis incidence is approximately 1:135,000 Americans. Cardiac involvement triggers multiple conduction disturbances, the most severe being complete heart block and VT/VF. Non-caseating granulomas have been found in the myocardium in up to 50% of the cases of fatal sarcoidosis, and 67% of patients that died from cardiac causes had myocardial involvement. Late gadolinium enhancem...

A clinical case report of late diagnosis (in relation to manifestation) the classic phenotype Fabry disease in patient with cardiac involvement: phenocopy hypertrophy cardiomyopathy, arrhythmias and conduction abnormalities.

Among clinicians, it is common practice to include parents in treatment, and it has been taken for granted that parents' involvement in their children's treatment is beneficial for therapy outcome, although research on this issue is far from clear. A meta-analysis was carried out in order to investigate whether parent involvement potentiates the outcome for children with anxiety disorders when ...

Sarcoidosis is a granulomatous disease of unclear etiology. Cardiac involvement in sarcoidosis is rarely diagnosed clinically. It is not commonly fatal, but when sarcoidosis is fatal, it is most often from cardiac involvement and when sarcoidosis involves the heart, it frequently causes death. We report a case of sudden death of 48 years male, wherein sarcoidosis with cardiac involvement was di...