Pulmonary artery aneurysm associated with a nonstenotic bicuspid pulmonic valve: A role for genetics?

BACKGROUND: Bicuspid pulmonic valves are quite uncommon, being described in only 0.1% of donor's hearts, while pulmonary artery aneurysms (PAAs) are even rarer, having been found in 8 out of 109,571 autopsies. This rarity makes it difficult to characterize the relationship between them. MATERIALS AND METHODS: We describe the case of a 66-year-old female who was found to have a bicuspid pulmonic valve and PAA (5.1 cm) on imaging by her cardiologist. DISCUSSION AND CONCLUSION: This case raises the question of whether the association between bicuspid semilunar valve disease and vascular wall anomalies is more genetic or hemodynamic. Even on the aortic side, despite the robust association between bicuspid aortic valves and thoracic aortic aneurysms, the mechanism still remains unclear. In our patient there was no significant gradient across the bicuspid pulmonic valve, suggesting that hemodynamics are not the primum mobile of this association.

Stentectomy for LAD "full metal jacket" with in-stent restenosis: How to do it.

We report our technique for the surgical revascularization of symptomatic severe in-stent restenosis of a "full metal jacket" (≥60 mm overlapping stents) of the left anterior descending coronary artery without suitable distal targets: on-pump cardioplegic-arrest stent removal (stentectomy) with endarterectomy and skeletonized left internal mammary artery onlay patch reconstruction. We also describe our follow-up protocol, including antiplatelet/anticoagulation and angiography. With proper patient selection, multidisciplinary collaboration, and surgical expertise, this advanced coronary procedure can be beneficial to a growing population of patients otherwise deemed to be untreatable.

A Calculation Tool and Process to Pre-Position Pharmaceuticals for Anthrax Post-Exposure Prophylaxis.

Anthrax, caused by Bacillus anthracis, is considered a severe bioterrorism threat because of its high mortality rate. The Chicago Healthcare System Coalition for Preparedness and Response (CHSCPR) aims to pre-position antibiotic medical countermeasures (MCMs) at healthcare facilities in order to provide on-site anthrax post-exposure prophylaxis. Pharmacists proposed moving toward a new process that involved the development of a standardized calculation methodology for acquiring supply drugs. This was an interventional quality improvement project aimed at optimizing inventory, acquisition, and distribution of antibiotic MCMs for anthrax post-exposure prophylaxis at Chicago hospitals for hospital personnel, associated first responders, and their families. The primary goal of the project was to pre-position a sufficient quantity of pharmaceuticals to allow Chicago hospitals to function as closed points of dispensing (PODs) for 72 hours; a secondary goal was to provide a 96-hour supply of anthrax post-exposure prophylaxis. A total of 35 Chicago hospitals were invited to participate in this intervention study, and 30 hospitals agreed to participate. Based on our calculation tool, we initially identified 6 (20%) hospitals with adequate oral doxycycline and ciprofloxacin inventory to last 72 hours and 3 (10%) hospitals with inventory to last 96 hours as a closed POD for anthrax post-exposure prophylaxis. The necessary quantities of medication needed to establish 72 and 96 hours of anthrax post-exposure prophylaxis were calculated by the CHSCPR and negotiated with a drug wholesaler to obtain product with maximum shelf-life and discounted pricing. Acting as a group purchaser, the CHSCPR organized drop shipment of medication directly to facilities from a wholesaler. This systematically calculated, pre-deployed pharmaceutical cache enhanced availability of antibiotic MCMs for anthrax post-exposure prophylaxis in 30 Chicago hospitals, allowing them to function as closed PODs for 96 hours during an incident.

Management of cardiovascular disease in patients with kidney disease.

The burden of cardiovascular disease is high in patients with chronic kidney disease or end-stage renal disease. The presence of kidney dysfunction affects the cardiovascular system in multiple ways, including accelerated progression of atherosclerosis and valvular disease, the exacerbation of congestive heart failure, and the development of pericardial disease. This comorbidity results not only from the concordance of shared risk factors, but also from other issues specific to this population, such as systemic inflammation and vascular calcification. Furthermore, both the sensitivity and specificity of noninvasive testing modalities, and the efficacy of several pharmacotherapeutic strategies, are diminished in this population. The exclusion of patients with severe kidney disease from many clinical trials of cardiac interventions raises various therapeutic uncertainties, and kidney disease itself is likely to alter the underlying cardiovascular physiology. In this Review, we discuss aspects of the epidemiology, pathophysiology, and diagnosis of cardiovascular disease in patients with kidney disease, and propose specific, evidence-based recommendations for pharmacological and surgical treatment.

Diminished benefits of drug-eluting stents versus bare metal stents in patients with severe renal insufficiency.

BACKGROUND: Since their introduction, the use of drug-eluting stents (DES) has increasingly become standard practice due to their decreased rates of in-stent restenosis and target lesion revascularization (TLR) rates in comparison to bare metal stents (BMS). However, these benefits have not been reproduced in patients with severe renal disease (SRD). This study compared TLR rates in patients with severe renal insufficiency treated with DES vs. BMS. METHODS: Between 2003 and 2006, we collected data on 6,220 consecutive patients receiving either DES or BMS. Both groups were similar in angiographic and clinical variables. TLR rates at 270 days and 1 year were then compared between patients receiving DES or BMS with varying creatinine clearance (CrCl). RESULTS: At 1 year after PCI, TLR rates were significantly lower for DES in patients with CrCl >60 (5 vs. 9.3%; p < 0.0001). However, in patients with CrCl <40 ml/min or on dialysis there was no significant difference in TLR rates for DES vs. BMS. CONCLUSION: While DES showed improved clinical outcomes in patients with normal and mildly impaired renal function, they showed no benefit over BMS in patients with moderate to severe renal insufficiency. Coupled with the possibly increased risk of late stent thrombosis with DES, BMS may be a more appropriate and safer stent in this population.

Elevated homocysteine levels in patients with end-stage renal disease.

PURPOSE: To examine the effect of hemodialysis on plasma homocysteine levels, and the relationship of these values to clinical cardiovascular events in patients with end-stage renal disease (ESRD). METHODS: Adults undergoing chronic hemodialysis were studied at baseline and at six months. Their clinical histories were obtained at the baseline visit, and measurements of plasma homocysteine concentration were made immediately prior to and following routine dialysis. The occurrence of clinical cardiovascular events was assessed over six months. RESULTS: We enrolled 147 patients (85 men and 62 women, age 58 +/- 15 years) who required hemodialysis for 3.4 +/- 3.4 years (mean +/- SD). The median homocysteine level for this population (including both pre- and post-dialysis values) was 17.3 micromoles/L. Mean pre-dialysis plasma homocysteine levels of patients with clinical cardiovascular disease did not differ significantly from those without the disease (22.5 +/- 9.9 vs. 25.4 +/- 24.5 micromoles/L, respectively), nor did post-dialysis levels differ between these populations. During six months follow-up, rates of ischemic events were not related to hyperhomocysteinemia. The difference between mean pre- and post-dialysis homocysteine levels (26.3 +/- 19.7 and 15.6 +/- 11.4 micromoles/L, respectively) and the decline in homocysteine over the course of a single dialysis treatment session (10.3 +/- 10.2 micromoles/L) were highly significant (p<0.0005). CONCLUSIONS: Plasma homocysteine levels were elevated in 82% of 147 patients with ESRD and fell to the normal range in a majority of patients during a single dialysis treatment session. Mean pre-dialysis levels did not change significantly over six months, however, and plasma homocysteine levels did not predict cardiovascular events in this population. There was also a trend towards worse outcomes in patients with lower homocysteine levels, which correlates to findings from recent studies. Further studies are needed to clarify the association between hyperhomocysteinemia and coronary risk in patients with ESRD.