Successful heart transplantation from a donor with Ullrich congenital muscular dystrophy.

Heart transplantation is the most effective therapy for children with end-stage heart disease; however, its use is limited by the number of donor organs available. This shortage may be further compounded by concerns about organ quality, leading to refusal of potential donor organ offers. We report on the successful transplantation and 5-year follow-up of a heart from a donor with Ullrich congenital muscular dystrophy (UCMD). The candidate was critically ill at the time of the transplant and the donor organ was declined repeatedly on the match run list due to concerns about organ quality, despite having normal cardiac function by echocardiography on minimal inotropic support. We believe the diagnosis of "muscular dystrophy" in the donor combined with a lack of understanding about the specifics of the diagnosis of UCMD enabled our candidate to receive a primary offer for this organ. We are unaware of any previous reports of the use of a heart from a donor with UCMD for orthotopic heart transplantation in adults or children.

Effect of creatine monohydrate on cardiac function in a rat model of endotoxemia.

BACKGROUND: Reports have attributed cardiac failure during acute models of endotoxemia to a lack of high-energy phosphates. This study was undertaken to investigate whether creatine (Cr) administered during perfusion could enhance myocardial protection and improve recovery of cardiac function in a rat model of endotoxemia. METHODS: Acute endotoxemia was induced in rats by a bolus injection of Escherichia coli endotoxin (LPS: 4 mg/kg, ip) while control rats were injected with an equal volume of 0.9% normal saline. To assess the adequacy of energy metabolism, freeze-clamped hearts were obtained from animals to study the concentrations of endogenous ATP, phosphocreatine (PCr), inorganic phosphate (P(i)), and intracellular pH by (31)P-cryomagnetic resonance spectroscopy. In a separate experiment, isolated hearts were perfused via a Langendorff column with Krebs-Henseleit buffer containing different concentrations of creatine monohydrate (1, 3, or 10 mM). Cardiac performance was evaluated via a paced (300 bpm) isovolumetric balloon preparation. Measurements of cardiac function including left ventricular developed pressure (LVDP), the maximum rates of ventricular pressure rise (LV +dP/dt) and fall (LV -dP/dt), and coronary flow were made for both LPS and saline-treated animals. RESULTS: High-energy phosphate ratios of PCr/ATP and PCr/P(i) in hearts declined significantly at 4 h after endotoxin treatment. As anticipated, LVDP and LV +dP/dt(max) at a given preload and heart rate were significantly (P < 0.05) lower at 4 h when measured at the same time point. The functional recovery of these parameters was not improved by the addition of creatine monohydrate to the perfusion buffer. Creatine produced a significant (P < 0.05) negative inotropic effect in hearts from saline-treated animals. The LVDP was reduced by 30% at the lowest concentration and by 50% at the highest concentration of creatine monohydrate. Furthermore, creatine significantly (P < 0.05) reduced LV -dP/dt(max) in both saline and LPS-treated rats. These data demonstrate that exogenous creatine does not contribute to myocardial preservation in endotoxemia. CONCLUSIONS: Energy stores in the rat heart decline early in endotoxemia accompanied by reduced myocardial performance, suggesting that the ability of the heart to perform mechanical work is impaired. Cardiac dysfunction in an acute model of endotoxemia was not improved with exogenous creatine during perfusion. Creatine's effects were primarily lusitropic by delaying the onset of myocardial relaxation in all hearts. The deleterious effects of exogenous creatine monohydrate in normal hearts should be examined in future experimental studies.

Comparison of nonbronchoscopic techniques with bronchoscopic brushing in the diagnosis of ventilator-associated pneumonia.

OBJECTIVE: To determine the diagnostic accuracy of quantitative cultures obtained via nonbronchoscopic protected specimen brushing (PSB) and nonbronchoscopic bronchoalveolar lavage (BAL) compared with quantitative cultures obtained by bronchoscopic PSB in surgical patients suspected of ventilator-associated pneumonia. DESIGN: Prospective, crossover controlled study of 15 ventilated surgical intensive care unit patients in a university teaching hospital. METHODS: Fifteen consecutive ventilated patients suspected of ventilator-associated pneumonia on the basis of leukocytosis, purulent sputum, and appearance of chest roentgenogram were enrolled. All patients underwent nonbronchoscopic PSB and BAL followed by bronchoscopic PSB. The duration of each procedure was noted. Culture results were considered positive only if greater than 10(4) colony forming units (CFU) per milliliter were present. MAIN RESULTS: Perfect concordance was noted between bronchoscopic PSB and nonbronchoscopic BAL (kappa = 1.0). The concordance between bronchoscopic PSB and nonbronchoscopic PSB was 93% ((kappa = 0.86). The nonbronchoscopic procedures were performed in significantly less time than the bronchoscopic procedure. CONCLUSION: Nonbronchoscopic PSB and BAL provide similar microbiologic data to bronchoscopic PSB in the diagnosis of ventilator-associated pneumonia while shortening procedure time significantly.

Technique for management of an axilloaxillary by-pass graft complicating a median sternotomy.

A technique is presented for intraoperative management of an axilloaxillary by-pass graft when performing a median sternotomy for myocardial revascularization. This method allows continual perfusion of both upper extremities during the operation thereby preventing the potential complications of upper extremity or cerebral ischemia.