Holmes heart in the adult: transesophageal echocardiography findings and long-term natural survival

We describe a 59-year-old patient with Holmes heart (single ventricle with normally related great arteries). To our knowledge, this is the oldest patient in the literature with this rare congenital heart disease and the oldest one with single ventricle and unrestricted pulmonary blood flow who has developed Eisenmenger's physiology. A transesophageal echocardiographic examination showed a single large ventricle that was a morphologic left ventricle with a normally appearing aorta originating from this chamber and a normally related pulmonary artery arising from a hypoplastic right infundibulum located anteriorly-superiorly and to the left of the left ventricle. The cardiac catheterization confirmed the echocardiographic findings and revealed a pulmonary artery pressure of 160/60 mmHg. There were no systolic gradients across the pulmonary and aortic outflow tracts. The estimated pulmonary vascular resistance was 2720 dynes-sec-cm-5. Oximetry showed oxygen saturation in the left ventricle of 84% and femoral artery saturation of 77.7%. Our report further demonstrates that pulmonary stenosis is not the ultimate determinant of prolonged natural survival in patients with single ventricle. Since this patient is too old for cardiopulmonary transplantation, we plan to follow him conservatively with medical therapy

Use of Non-Pathogenic Biological Agents as Biological Warfare Simulants for the Development of a Stand-Off Detection System.

Development of new technologies for Biological Warfare Agents (BWA) stand-off detection implies several safeties, logistic and economic drawbacks that involve production of different highly virulent bacteria and viruses, their isolation and characterization under adequate bio-containment and sample preparation for each agent to evaluate the testing method. In order to overcome these difficulties most of the research activities and tests reported so far, are performed using simulants: Biological Agents (BA) which are phylogenetically or structurally related to BWA. The use of the simulants (BWA-S) show, however, some limitations: they can share some of the properties of the biological warfare agents but have different antigens, proteome and genome. In this work, different BWA-S was evaluated for the application in the development and training of stand-off detection systems. This study is the basis for the use of simulants in the development of an Ultraviolet Laser Induced Fluorescence (UV-LIF) based detection systems

Ebola Virus Disease 2013-2014 Outbreak in West Africa: An Analysis of the Epidemic Spread and Response

The Ebola virus epidemic burst in West Africa in late 2013, started in Guinea, reached in a few months an alarming diffusion, actually involving several countries (Liberia, Sierra Leone, Nigeria, Senegal, and Mali). Guinea and Liberia, the first nations affected by the outbreak, have put in place measures to contain the spread, supported by international organizations; then they were followed by the other nations affected. In the present EVD outbreak, the geographical spread of the virus has followed a new route: the achievement of large urban areas at an early stage of the epidemic has led to an unprecedented diffusion, featuring the largest outbreak of EVD of all time. This has caused significant concerns all over the world: the potential reaching of far countries from endemic areas, mainly through fast transports, induced several countries to issue information documents and health supervision for individuals going to or coming from the areas at risk. In this paper the geographical spread of the epidemic was analyzed, assessing the sequential appearance of cases by geographic area, considering the increase in cases and mortality according to affected nations. The measures implemented by each government and international organizations to contain the outbreak, and their effectiveness, were also evaluated

Prevalence of the Congenital Long-QT Syndrome

Background-The prevalence of genetic arrhythmogenic diseases is unknown. For the long-QT syndrome (LQTS), figures ranging from 1: 20 000 to 1: 5000 were published, but none was based on actual data. Our objective was to define the prevalence of LQTS. Methods and Results-In 18 maternity hospitals, an ECG was performed in 44 596 infants 15 to 25 days old ( 43 080 whites). In infants with a corrected QT interval (QTc) >450 ms, the ECG was repeated within 1 to 2 weeks. Genetic analysis, by screening 7 LQTS genes, was performed in 28 of 31 (90%) and in 14 of 28 infants (50%) with, respectively, a QTc >470 ms or between 461 and 470 ms. A QTc of 451 to 460, 461 to 470, and >470 ms was observed in 177 (0.41%), 28 (0.06%), and 31 infants (0.07%). Among genotyped infants, disease-causing mutations were found in 12 of 28 (43%) with a QTc >470 ms and in 4 of 14 (29%) with a QTc of 461 to 470 ms. One genotype-negative infant ( QTc 482 ms) was diagnosed as affected by LQTS on clinical grounds. Among family members of genotype-positive infants, 51% were found to carry disease-causing mutations. In total, 17 of 43 080 white infants were affected by LQTS, demonstrating a prevalence of at least 1:2534 apparently healthy live births (95% confidence interval, 1:1583 to 1:4350). Conclusions-This study provides the first data-based estimate of the prevalence of LQTS among whites. On the basis of the nongenotyped infants with QTc between 451 and 470 ms, we advance the hypothesis that this prevalence might be close to 1:2000. ECG-guided molecular screening can identify most infants affected by LQTS and unmask affected relatives, thus allowing effective preventive measures