Cardiac assessment of patients with Becker's muscular dystrophy.

Becker's muscular dystrophy (BMD) is one of the most common muscular dystrophy syndromes. The heart is always affected by myocardial fibrosis with early involvement of the right side. Traditionally, ECG, echocardiography and stress testing have been used to evaluate these patients. Cardiac MRI, offering superior assessment of the right side and visualization of the myocardial fibrosis could become the preferred imaging modality for evaluating patients with BMD.

Apical aneurysm in a patient with apical hypertrophic cardiomyopathy.

Apical hypertrophic cardiomyopathy (AHC) is a variant of hypertrophic cardiomyopathy (HCM) in which the hypertrophy predominantly involves the left ventricular apex. The typical features of AHC include giant negative T waves in the precordial ECG leads, a spade-like configuration of the left ventricle at end-systole, the absence of an outflow tract pressure gradient, and mild symptoms. We present a patient with AHC, evidence of prior myocardial infarction with aneurysm of the apical region, and myocardial ischemia on radionuclide scanning despite angiographically normal coronary arteries. The characteristic electrocardiographic, echocardiographic, hemodynamic and angiographic findings as well as prognosis and treatment options are discussed.

Intermittent outpatient ultrafiltration for the treatment of severe refractory congestive heart failure.

BACKGROUND: Patients with severe congestive heart failure (CHF) become refractory to conventional medical therapy, leading to recurrent rehospitalizations. We examined the impact of intermittent outpatient ultrafiltration (UF), using either peritoneal dialysis or hemofiltration, on long-term clinical outcomes in patients with refractory CHF. METHODS AND RESULTS: We analyzed clinical and hemodynamic data in 19 consecutive patients with refractory CHF who received intermittent outpatient UF for at least 1 year between July 1998 and November 2002. The mean left ventricular ejection fraction of all 19 patients was 30.2 +/- 19.0%. All patients (100.0%) were New York Heart Association (NYHA) class IV. Only 5 patients (26.3%) received peritoneal dialysis; the remaining 14 (73.7%) received hemofiltration. There were 6 patients with a normal left ventricular ejection fraction (45%). After UF was started, the number of patients that were considered inotrope-dependent was reduced from 86.4% to 36.8% (P < .005). Compared with the year before UF was initiated, the number of CHF hospitalizations during follow-up was reduced from 2.6 to 0.3 (P < .005), and the NYHA class was improved from 4 to 3.1 (P < .005). Among all patients, 2 deaths were related to complications of UF, and cumulative 1-year survival was 63.2%. CONCLUSION: Our study suggests that UF is a safe, feasible therapy, but it needs further evaluation in carefully designed, prospective, randomized clinical trials. UF has the potential for offering another important therapeutic option for patients with severe and refractory CHF.

Stroke in patient with an intervalvular fibrosa pseudoaneurysm and aortic pseudoaneurysm.

We describe a case of an intervalvular fibrosa pseudoaneurysm associated with a cerebrovascular accident. This case in unusual as the likely source of embolic stroke was thrombus from within the pseudoaneurysm. Transesophageal echocardiography also demonstrated a communication between the intervalvular fibrosa and the proximal aorta.