Lambl's Excrescence and Management of Recurrent Cerebrovascular Accident (CVA).

Lambl's excrescence is a rare valvular finding of uncertain pathologic significance. This case describes a previously healthy 42-year-old woman experiencing a sudden onset of word-finding difficulty. MRI of the brain demonstrated acute and chronic infarcts, prompting echocardiography, which revealed Lambl's excrescence of the aortic valve.

Clinical implications of defective B-type natriuretic peptide.

Our understanding of the natriuretic peptide system continues to evolve rapidly. B-type natriuretic peptide (BNP), originally thought to be a simple volume-regulating hormone that is produced in response to cardiac stretch, has been shown to also play important roles in modulating bronchodilation, endothelial function, and cardiac remodeling. Recent data demonstrate that elevated levels of BNP in patients with heart failure do not represent a simple ratcheting up of normal production in response to increased stimulus. Instead, we now know that chronic stimulation of BNP synthesis induces a reversion to fetal gene expression, resulting in production of high molecular weight forms of BNP that are functionally deficient. Standard point-of-care BNP assays are immunoassays that will detect any molecule containing the target epitopes. Consequently, these assays cannot distinguish between defective, high molecular weight forms of BNP and normal, physiologically active BNP. In 2 separate evaluations, mass spectroscopy detected little, if any, normal BNP in patients with heart failure, despite the appearance of high circulating levels of immunoreactive BNP (iBNP) using commercial assays. Therefore, these commercial assays should be considered to be only an indication of myocardial stress. They do not measure physiologic BNP activity. This accounts for the "BNP paradox," namely, that administration of exogenous recombinant human BNP (rhBNP, nesiritide) has substantial clinical and hemodynamic impact in the presence of high levels of circulating iBNP using commercial assays. In addition to its short-term hemodynamic impact, rhBNP may have other important effects in this setting, and further investigation is warranted.

Peri-infarct pacing with CRT in the early postinfarct phase to attenuate long-term remodeling.

Deleterious left ventricular remodeling routinely occurs after myocardial infarction (MI) and novel strategies to attenuate this phenomenon may prove valuable. Here, we describe the potential role of left ventricular (LV) peri-infarct pacing (delivered via biventricular pacemakers) to reduce post-MI remodeling. Regional wall stress in the infarcted area is predictive of remodeling and therefore represents a potential therapeutic target. Using an intrinsic property of pacing to reduce stress and work at the pacing site, there are animal data to suggest that long-term peri-infarct pacing can have a salutary effect on cardiac structure after MI. This concept was tested in a pilot human study, suggesting attenuation of ventricular dilation in post-MI patients treated with LV pacing compared with control. To further characterize this concept, a 110-patient, multicenter, randomized Prevention of Myocardial Enlargement and Dilatation post-Myocardial Infarction Study (MENDMI) has completed enrollment. Inclusion criteria included anterior MI, QRS < 120 ms, ejection fraction 2,000, and wall motion abnormalities in at least five of 16 segments. MENDMI will help to determine whether chronic application of peri-infarct pacing provides structural and clinical benefits and will help in the design of further investigations to modify postinfarction ventricular remodeling.

Comparison of ultrafiltration, nesiritide, and usual care in acute decompensated heart failure.

To study the short-term effects of initial treatment strategies for acute decompensated heart failure, 25 patients treated with ultrafiltration (UF) were retrospectively compared with 25 patients treated with usual care (UC) and 25 patients treated with UC plus adjunctive nesiritide infusion (UN), matched for age, sex, ejection fraction, etiology, and serum creatinine. The median length of hospitalization was 6 days for UF, 4 days for UC, and 6 days for UN. All-cause 30-day readmissions tended to be fewer in the UF (16%) compared with the UN (24%) or UC (24%) groups. All groups lost weight, although to a greater extent in the UF group (15.8 lb vs 6.3 lb in UC and 4.7 lb in UN). Concomitantly, UF manifested the greatest increase in serum urea nitrogen, creatinine, and the number of patients with creatinine increases of >0.5 mg/dL (44% vs 24% in UC and 20% in UN). In acute decompensated heart failure, UF appears to be more effective for volume removal and possibly prevention of all-cause hospital readmission to 30 days than UC or UN. These findings, as well as the effects on renal function and length of stay, need to be further evaluated in a prospective randomized study.

Pulmonary hypertension in neurofibromatosis.

Two cases of severe pulmonary arterial hypertension in patients with neurofibromatosis are reported. The published research is reviewed. In conclusion, it is suggested that the association between these conditions be recognized in the classification of pulmonary hypertension.

Feasibility of biventricular pacing in patients with recent myocardial infarction: impact on ventricular remodeling.

To test the hypothesis that biventricular pacing after a myocardial infarction with reduced ejection fraction can attenuate left ventricular (LV) remodeling, the authors studied 18 patients (myocardial infarction within 30-45 days, ejection fraction

Early revascularization and ACC/AHA guideline-compliant medical management improve left ventricular function and short-term prognosis in patients presenting with acute myocardial infarction and severe left ventricular dysfunction.

BACKGROUND: Myocardial infarction (MI) complicated by severe left ventricular (LV) dysfunction is associated with significant morbidity and mortality. The natural history of this population with contemporary revascularization and guideline-based medical therapies is poorly defined. We sought to determine the impact of contemporary treatment strategies on LV function and prognosis in patients with MI and severe LV dysfunction. METHODS: Consecutive MI patients were prospectively followed as part of an ongoing internal database. The current report comprises 75 patients with first MI and severe LV systolic dysfunction (EF less than or equal to 3%). Initial demographic and clinical data were collected during hospitalization and at 1-, 3- and 6-month follow up. RESULTS: Patients were 71% male, 36% diabetic and 51% had prior coronary disease with a mean (+/- SD) age of 65 +/- 14 years. The average hospital stay was 5.7 days for ST-elevation (CPK range 424 to 5,250) and 2.4 days for non-ST-elevation MI (CPK range 175 to 705). Revascularization in-hospital was performed in 87% of patients (62 percutaneous, 3 surgical). At hospital discharge, treatment included beta-blockers (84%), ACE-inhibitors (73%), statins (81%), aspirin (88%) and clopidogrel (84%). Mean (+/- SD) LVEF was 25.7 +/- 5.9% in hospital, 36.6 +/- 11.8% by 1 to 3 months (p < 0.01), and 37.6 +/- 9.3% at 6 months (p < 0.01). By 1 to 3 months, 63% had improved LVEF, 24% were unchanged and 14% were worse. One patient died in the hospital and 3 died by 6-month follow up (mortality 5.3%). CONCLUSION: A strategy of early revascularization combined with guideline-based medical management favorably impacts LV function and short-term prognosis in MI patients with severe LV systolic dysfunction. With contemporary treatment strategies, the majority (> 60%) of patients demonstrate improvement in LVEF and mortality is low (5.3%).

Safety and tolerability of serial home infusions of nesiritide for advanced heart failure.

Although the therapeutic strategy of serial 6-hour intravenous infusions of nesiritide for advanced chronic heart failure (HF) is currently under investigation, frequent hospital or clinic visits may present logistical challenges for many patients with HF. Therefore, a pilot study was conducted to evaluate the safety and tolerability of serial nesiritide infusions administered at home in this patient population. Twenty patients with advanced HF were enrolled into a protocol for the administration of 12 weekly intravenous infusions of nesiritide (2 microg/kg bolus, 0.01 microg/kg/min for 6 hours) at home. Blood samples and weights were obtained weekly, and clinical assessments were made at 4 and 12 weeks. During the 12-week study period, there were 3 deaths unrelated to nesiritide therapy. Six patients required >or=1 hospitalization for HF. Two of 204 infusions resulted in symptomatic hypotension, and there were no adverse renal events. Mean body weight decreased at week 4 but returned to baseline by week 12. Antibodies to nesiritide were not detected in any patient. In conclusion, serial home infusions of nesiritide in patients with advanced HF appear to be safe and well tolerated, but the efficacy of this therapeutic strategy requires definition in a larger, randomized trial.