Fate of infants with hypoplastic left heart syndrome listed for cardiac transplantation: a multicenter study.

BACKGROUND: Infants with hypoplastic left heart syndrome (HLHS) commonly undergo cardiac transplantation as primary management. METHODS: We examined outcomes of primary transplantation for unpalliated HLHS. We analyzed data from the 20 institutions of the Pediatric Heart Transplant Study Group, from January 1, 1993, through December 31, 1998, using actuarial and parametric survival analysis and competing outcomes analysis. RESULTS: During the 6 years studied, 1,234 patients were listed for cardiac transplantation; 262 patients (21.2%) had unpalliated HLHS. The number (and percentage) of patients with HLHS decreased from 58 (27% of patients listed) in 1993 to 30 (14%) in 1998. Overall, 25% of infants with HLHS died while waiting; primary cause of death was cardiac failure (50%). Of the remaining patients awaiting transplantation, 23 (9%) underwent Norwood/Fontan-type surgeries as interim palliation: 52% died. Ultimately, 175 patients underwent cardiac transplantation (67%); 50% received organs by 2 months after listing. Post-transplant actuarial survival was 72% at 5 years, with 76% of deaths (35/46) occurring within 3 months; early mortality was caused primarily by graft failure within the first 30 days after transplantation (in 54%). Among 1-month survivors, survival at 1 and at 5 years was 92% and 85%, respectively. Of the 262 patients listed with unpalliated HLHS, overall survival, taking into account mortality after listing and after transplantation, was 68% at 3 months and 54% at 5 years. CONCLUSIONS: Cardiac transplantation offers good intermediate survival for infants with unpalliated HLHS.

Carvedilol as therapy in pediatric heart failure: an initial multicenter experience.

OBJECTIVE: The objective was to determine the dosing, efficacy, and side effects of the nonselective beta-blocker carvedilol for the management of heart failure in children. STUDY DESIGN: Carvedilol use in addition to standard medical therapy for pediatric heart failure was reviewed at 6 centers. RESULTS: Children with dilated cardiomyopathy (80%) and congenital heart disease (20%), age 3 months to 19 years (n = 46), were treated with carvedilol. The average initial dose was 0.08 mg/kg, uptitrated over a mean of 11.3 weeks to an average maintenance dose of 0.46 mg/kg. After 3 months on carvedilol, there were improvements in modified New York Heart Association class in 67% of patients (P =.0005, chi2 analysis) and improvement in mean shortening fraction from 16.2% to 19.0% (P =.005, paired t test). Side effects, mainly dizziness, hypotension, and headache, occurred in 54% of patients but were well tolerated. Adverse outcomes (death, cardiac transplantation, and ventricular-assist device placement) occurred in 30% of patients. CONCLUSIONS: Carvedilol as an adjunct to standard therapy for pediatric heart failure improves symptoms and left ventricular function. Side effects are common but well tolerated. Further prospective study is required to determine the effect of carvedilol on survival and to clearly define its role in pediatric heart failure therapy.

Screening people for illicit substances: a survey of current portal technology.

The need to implement more effective, cost efficient, non-intrusive ways of screening people for concealed drugs, explosives and weapons is a major security initiative for the U.S. and elsewhere. A new generation of portals suitable for security checkpoints is under development. These will be able to find contraband with high probabilities of detection, and low false-alarm rates. Some portals image the body using either low dose X-rays or millimeter wave interrogation, and analyze the radiation back-scattered from the body, providing images of concealed objects. Other portals operate essentially as anomaly detectors, finding objects on the human body. A series of portals is also under development that can harvest and analyze vapors or particles of the contraband substances. Some of the issues surrounding portal development include perceived safety issues, privacy issues, and operational issues such as ease of operation, non-invasiveness, and ease of interpretation. It is believed that portal technology will mature, and the cost of portals will fall sufficiently to make widespread deployment of portals at security checkpoints a reality within the next decade.

Relative binding constants of arsenical-antidote adducts determined by NMR spectroscopy.

Proton nuclear magnetic resonance spectroscopy was used to determine relative binding constants for several arsenical-antidote adducts. It was found that BAL (2,3-dimercaptopropanol) and DMPS (2,3-dimercaptopropanesulfonic acid) had a higher affinity than DMSA (2,3-dimercaptosuccinic acid) for the two organic arsenicals studied.

Enzyme-amplified receptor assay screening test for chlorpromazine, trifluoperazine, and phencyclidine.

A new receptor based assay is described for the determination of classes of drugs which have high affinities for the acetylcholine receptor. The method is based upon the inhibition of the enzyme activity of an enzyme-drug conjugate by the binding to the receptor protein, and competition between free drugs and the enzyme-drug conjugate for a limited number of receptor sites. The activity of the enzyme marker system, glucose-6-phosphate dehydrogenase covalently conjugated to desipramine, is monitored by colorimetric detection of the rate of NADH formation at 340 nM. The procedure proposed is designed to provide a simple drug screen which can be done in a minimally equipped laboratory while achieving the required sensitivity. The technique is illustrated for three acetylcholine channel binding compounds: the hallucinogen phencyclidine (PCP) and the antipsychotic agents chlorpromazine and trifluoperazine. This procedure yields calibration curves with detection limits at nanomolar levels of drug, with binding responses dependent on the amounts of receptor and enzyme-labeled drug used. Aspecific binding responses of unlabeled enzyme to drug or receptor to compounds with low affinity for the receptor are shown to have minimal effect on the assay.

Reaction of trans-2-chlorovinylarsine oxide with polydeoxynucleotides.

Trans-2-chlorovinylarsine oxide (in DCl/acetone-d6) was added to various polydeoxynucleotides. The arsenical did react with poly[dG].poly[dC], releasing guanine, and resulting in a partial apurinic duplex.