Contemporary epidemiology of infective endocarditis in patients with congenital heart disease: A UK prospective study.

OBJECTIVES: Infective endocarditis is a life-threatening complication of congenital heart disease (CHD), but there are few studies concerning the contemporary risk profile, preceding invasive procedures and outcomes in this patient population. The aim of this study was to investigate the epidemiology of infective endocarditis (IE) in patients with CHD. METHODS: Cases of IE in children and adults with CHD were prospectively recorded as part of the UK National Institute for Cardiovascular Outcomes Research (NICOR) National Congenital Heart Disease Audit. Patients were entered into the database between April 2008 and March 2016. RESULTS: Eight hundred episodes of IE were recorded in 736 patients with CHD. Sixty-five patients (9%) were infants (aged <1 year), 235 (32%) were children (aged 1-15 years), and 436 (59%) were adults (aged >15 years). The most common diagnoses were Tetralogy of Fallot (n = 150, 22.8%), ventricular septal defect (n = 129, 19.6%) and bicuspid aortic valve (n = 70, 10.7%). Dental procedures preceded 67 of 635 episodes (11%) of IE, and non-dental invasive procedures preceded 177 of 644 episodes (27.4%). The most common causative organisms were streptococci, accounting for 40% of cases. Overall in-hospital mortality was 6.7%. On multivariable analysis, adverse factors associated with in-hospital mortality were staphylococcal infection and presence of an underlying atrioventricular septal defect. CONCLUSIONS: Infective endocarditis in patients with CHD is an ongoing clinical challenge. In contemporary practice in tertiary congenital centers, 1 of 15 patients do not survive to hospital discharge. Streptococci remain the most common causative organism, and antecedent dental or medical procedures were undertaken in a significant minority in the 3 months before diagnosis. The presence of an atrioventricular septal defect or staphylococcal infection is associated with significantly increased risk of early mortality.

Why quad bike safety is a wicked problem: an exploratory study of attitudes, perceptions, and occupational use of quad bikes in northern Queensland, Australia.

Quad bike safety represents the quintessential wicked problem. To address this wicked problem, this research explores the use of quad bikes in the northwestern region of outback Queensland, Australia, concentrating efforts on the pastoral industry. Focus groups and interviews were conducted with pastoralists, farmers, retailers, repairers, healthcare professionals, and regulators. The discussions revealed the diverse activities currently undertaken in the region's pastoral enterprises while riding a quad bike, and that attitudes toward the purchase and use of quad bikes vary and may be influenced by retailers. Perceptions of risk and safety in the use of quad bikes in occupational settings also varied. The findings from this study provide insight into the decisions of local pastoralists and agriculturalists to use quad bikes in their workplaces, and attitudes toward safety and injury prevention relating to quad bike use in these industries in northwestern outback Queensland. This study is the first step toward understanding quad bike use in this region, with the goal of reducing injury and death, and will be used in policy and legislation development regarding the use and safety of quad bikes in Australia.

Predicting 22q11.2 deletion syndrome: a novel method using the routine full blood count.

BACKGROUND: 22q11.2 deletion syndrome is common affecting nearly 1 in 3000, including many with DiGeorge Syndrome and 5% of individuals with congenital heart disease. Diagnosis is important because affected patients have impaired immune function and may suffer high mortality rates if given non-irradiated blood products from graft versus host disease. Symptomatic hypocalcaemia may also occur. Our objective was to determine whether mean platelet volume (MPV), available from the routine full blood count, may be a useful and rapid indicator of 22q11.2 deletion. METHOD: A retrospective case control cohort study analysing MPV and 22q11.2 deletion status was performed in a paediatric population (n = 166) undergoing cardiac surgery between 1999 and 2005. RESULTS: Twenty children were 22q11.2 positive. The median MPV was significantly larger for the 22q11.2 positive patient group compared to the non-22q11.2 patients (10.9fL versus 8.6fL, p<0.001). The area under the curve of the receiver operating characteristics (ROC) curve of MPV was large enough (0.85) to enable the accurate prediction of 22q11.2 deletion using MPV. CONCLUSIONS: MPV is a useful screening test, involving no extra laboratory work, cost or patient discomfort. MPV>10fL is a positive predictor of the presence of 22q11.2 deletion in children with congenital heart disease (specificity 89.7%). This finding should aid rapid decision-making for ordering irradiated blood products to prevent potentially fatal transfusion-associated graft versus host disease. It will alert clinicians to monitor serum calcium levels closely to prevent hypocalcaemic seizures.

Hearing screening program impact on noise reduction strategies.

The objective of this study was to determine the impact of the New South Wales Rural Hearing Conservation Program on the implementation of personal hearing protection (PHP) and noise management strategies among farmers who had participated in this program in New South Wales, Australia. A follow-up survey of a random sample of people screened through the New South Wales Rural Hearing Conservation Program was linked to their baseline data. The use of PHP at baseline was compared to use at follow-up in four specific scenarios: use with non-cabbed tractors, with chainsaws, with firearms, and in workshops. For non-cabbed tractors, the net gain in PHP use was 13.3%; the net gain was 20.8% for chainsaws, 6.7% for firearms, and 21.3% for workshops. Older farmers and those with a family history of hearing loss were less likely to maintain or improve PHP use. Those with severe hearing loss, males, and participants reporting hearing problems in situations where background noise was present were more likely to maintain or improve PHP use. Forty-one percent of farmers had initiated other strategies to reduce noise exposure beyond the use of PHP, which included engineering, maintenance, and noise avoidance solutions. The early (hopefully) identification of hearing deficit in farmers and farm workers can help promote behavior change and help reinforce a farm culture that supports hearing conservation. The continuation and expansion of hearing screening programs such as these should be encouraged as basic public health strategy in farming communities.

A paediatric telecardiology service for district hospitals in south-east England: an observational study.

OBJECTIVES: To compare caseloads of new patients assessed by paediatric cardiologists face-to-face or during teleconferences, and assess NHS costs for the alternative referral arrangements. DESIGN: Prospective cohort study over 15 months. SETTING: Four district hospitals in south-east England and a London paediatric cardiology centre. PATIENTS: Babies and children. INTERVENTION: A telecardiology service introduced alongside outreach clinics. MEASUREMENTS: Clinical outcomes and mean NHS costs per patient. RESULTS: 266 new patients were studied: 75 had teleconsultations (19 of 42 newborns and 56 of 224 infants and children). Teleconsultation patients generally were younger (49% being under 1 year compared with 32% seen personally (p = 0.025)) and their symptoms were not as severe. A cardiac intervention was undertaken immediately or planned for five telemedicine patients (7%) and 30 conventional patients (16%). However, similar proportions of patients were discharged after being assessed (32% telemedicine and 39% conventional). During scheduled teleconferences the mean duration of time per patient in sessions involving real-time echocardiography was 14.4 min, and 8.5 min in sessions where pre-recorded videos were transmitted. Mean cost comparisons for telemedicine and face-to-face patients over 14-day and 6-month follow-up showed the telecardiology service to be cost-neutral for the three hospitals with infrequently-held outreach clinics (1519 UK pounds vs 1724 UK pounds respectively after 14 days). CONCLUSION: Paediatric cardiology centres with small cadres of specialists are under pressure to cope with ever-expanding caseloads of new patients with suspected anomalies. Innovative use of telecardiology alongside conventional outreach services should suitably, and economically, enhance access to these specialists.

Factors affecting farm noise during common agricultural activities.

Hearing injury due to exposure to excessive noise during common farming activities is a significant problem for farmers. The aim of this study was to investigate factors that affect the level of risk to hearing caused by common farming activities. Noise levels on farms were measured across a range of activities and producer groups, and situational factors that effect noise levels were also investigated. Older tractors were found to be 6 dB louder than newer tractors. Cabs reduced noise to the operator by 16 dB, which was halved to 8 dB if a door was open. Radios added between 3 and 5 dB to the noise in the cab. These variables significantly affect the noise level at the ear of operators and others in the workplace, and affect the subsequent exposure limits that are considered safe. Situational factors need to be considered in assessing the level of risk to farmers' hearing and in choosing noise management strategies on the farm. This information has been incorporated into material about hearing and discussions with farmers who participated in field day hearing screening programs in Australia.

Saddle arterial embolus in a patient with Down syndrome.

We report acute thromboembolic events in a 14-year-old boy with Down syndrome and repaired atrioventricular septal defect. He presented with sudden onset of bilateral lower limb ischemia. Transesophageal echocardiography detected a thrombus in the right atrium. An arterial saddle embolus was removed following bilateral iliac embolectomy. Despite anticoagulation, he presented again with sudden bilateral lower limb ischemia and respiratory distress. Multiple pulmonary emboli and a thrombus in the right atrium were noted on imaging studies. An arterial embolus was removed from the abdominal aorta at the bifurcation. To our knowledge, this is the first report of a child or adolescent with a repaired congenital heart lesion and arterial embolism requiring embolectomy. This association and possible etiological factors are discussed.

Farm noise emissions during common agricultural activities.

Noise injury in agriculture is a significant yet often unrecognized problem. Many farmers, farm workers, and family members are exposed to noise levels above recommended levels and have greater hearing loss than their non-farming contemporaries. The aim of this study was to gather up-to-date information on farm noise levels and to enhance the quality of information available to assist farmers in reducing noise exposure and meeting Occupational Health and Safety (OHS) regulations regarding noise management. Farm visits were conducted on 48 agricultural establishments that produce a range of commodities. Noise levels were measured at the ears of operators and bystanders involved in typical activities on farms. The average and peak noise levels were measured for 56 types of machinery or sites of farming activity, totaling 298 separate items and activities. Common noise hazards identified included firearms, tractors without cabs, workshop tools, small motors (e.g., chainsaws, augers, pumps), manual handling of pigs, shearing sheds, older cabbed tractors, and heavy machinery such as harvesters, bulldozers, and cotton module presses. We found that use of firearms without hearing protection presents a pressing hearing health priority. However, farming activities involving machinery used for prolonged periods also present significant risks to farmers' hearing health. Noise management strategies on the farm are essential in order to prevent noise injury among farmers.

Congenital heart disease and neuroblastoma: just coincidence?

The clinical history of a neonate with simple transposition of the great arteries in whom a metastatic neuroblastoma was diagnosed incidentally at autopsy is described, and the literature containing all 66 previously reported cases of neuroblastoma associated with congenital cardiac malformations is reviewed. One third of the described cases were classified as in situ neuroblastoma; neural crest derived cardiac lesions were present in 31%. Several possible aetiological mechanisms are discussed, and we conclude that the association of neuroblastoma with congenital cardiac malformations is multifactorial in origin. The described case represents the first reported example in which catecholamine release may have contributed to the fatal outcome of definitive congenital cardiac surgery.

Octreotide to treat postoperative chylothorax after cardiac operations in children.

Chylothorax after pediatric cardiac operations is associated with significant morbidity and increased hospitalization. An octreotide (a synthetic somatostatin analogue) infusion (1 to 4 microg/kg per hour) with medium-chain triglyceride diet or parenteral nutrition was used in 4 pediatric cardiac surgical patients after chylothorax was diagnosed. Resolution followed within 5 days in all without recurrence, while on a normal diet.

Farm-related fatalities involving children in Australia, 1989-92.

OBJECTIVE: To describe the types of and circumstances surrounding unintentional farm-related fatal injuries involving children aged less than 15 years in Australia. METHODS: Information concerning 115 deaths were obtained from inspection of coronial files for the period 1989-92. RESULTS: Children less than 15 years made up 20% of all unintentional farm-related fatalities in Australia, with children less than 5 years representing 63% of all child fatalities. The majority of children were fatally injured while bystanders to farm work and equipment used on the farm (including dams), with drowning the most common mechanism of the fatal incident for children aged both 5 years or less and 5-9 years. Vehicle accidents were common for children aged 10-14 years. CONCLUSIONS: Children are exposed to various hazards in the farming environment and as such are at risk of being injured. IMPLICATIONS: This study has highlighted a number of particular hazards for children on farms, with drowning, transport and tractor-related injuries of particular concern. A national strategy for child safety on farms has been developed by Farmsafe Australia aimed at providing a nationally co-ordinated plan for improving child safety on farms.

Agricultural work-related fatalities in Australia, 1989-1992.

Farm-related fatalities in Australia were studied as part of a larger study of all work-related traumatic fatalities from 1989-1992. Information concerning 373 unintentional work-related fatalities was obtained from inspection of coronial files and described. The fatality rate per 100,000 workers was four times higher for agricultural industry workers (20.6) compared to the all-industry rate during the same time frame (5.5). Males comprised 95% of all agricultural work-related deaths. Agents such as farm vehicles, mobile farm machinery (mainly tractors), and farm structures (mainly dams) were among the most common involved in the fatal incident. Being hit by moving objects, vehicle accidents, and rollovers of mobile machinery (mainly tractors) were among the most common mechanisms of fatal injury on farms. Transport for work purposes, working with animals, working with crops, and maintenance were the most common activities being undertaken. The information obtained from this study is being used to develop health and safety risk profiles for agricultural industries, and these profiles are being used in turn to develop guidance material for farmers, on-farm checklists, and other tools to help farmers manage their occupational health and safety risk.

Non-work-related farm fatalities in Australia, 1989-1992.

This study aims to examine farm fatalities, between 1989-1992 in Australia, of individuals not working at the time of the incident. Non-work-related farm fatalities in Australia were studied as part of a larger study of all work-related traumatic fatalities from 1989-1992. Information on 214 unintentional non-work-related farm fatalities was obtained from inspection of coronial files. The information was examined according to type of bystander (bystander to work or bystander to farm equipment) and other farm deaths. Agents such as dams, tractors, utilities, and cars were among the most common causes of death for bystanders involved in fatal incidents. Fire and smoke, creeks or rivers, and cars were the most common agents for other farm deaths. Drowning, vehicle accidents, and being hit by moving objects were among the most common mechanisms of fatal injury for non-work-related farm deaths. The information gained from this study has been used to develop the Child Safety on Farms Strategy for Farmsafe Australia and Farmsafe Australia's Farm Machinery Safety Strategy.

Severe airflow limitation after the unifocalization procedure: clinical and morphological correlates.

BACKGROUND: While unifocalization techniques have improved the treatment options in patients with pulmonary atresia, ventricular septal defect (PA-VSD), and major aortopulmonary collaterals (MAPCAs), severe airflow limitation contributes to significant early postoperative morbidity and mortality. Although this has been attributed to bronchospasm, characteristically it is refractory to bronchodilators, suggesting that other mechanisms may play a role. METHODS AND RESULTS: The clinical course and preoperative angiograms of patients who underwent unifocalization were reviewed. Patients who developed airflow limitation early after surgery underwent fiberoptic bronchoscopy. In addition, the anatomy of the MAPCAs was examined in 14 heart-lung blocks from patients with PA-VSD. Twenty-two procedures were performed in 16 children. Three developed marked airflow limitation early after surgery, necessitating prolonged high-pressure ventilation. Bronchoscopy demonstrated tracheobronchial epithelial necrosis in 2 and signs of tracheobronchial ischemia in the third. Two were successfully extubated after 15 and 16 days, but the third died after 57 days of ventilatory support. Review of the preoperative angiograms demonstrated an extensive peribronchial arterial supply arising from a MAPCA in 1 of the patients who developed severe airway necrosis after unifocalization. This was also obvious in a second patient, but the MAPCA was not included in the unifocalization. In 7 autopsy specimens, MAPCAs contributed to a peribronchial or peritracheal vascular network. Dissection of the distribution of these branches in 2 specimens revealed extensive intrapulmonary peribronchial anastomoses. CONCLUSIONS: Airflow limitation early after unifocalization is related to airway ischemia resulting from interruption of the tracheobronchial blood supply during mobilization of MAPCAs.

Heterotopic cardiac transplantation and Batista operation.

We report the case of an infant who was first seen with dilated cardiomyopathy at the age of 3 1/2 months and 2 months later, successfully underwent heterotopic cardiac transplantation in combination with partial left ventriculectomy. The benefits of combining both procedures in such a young infant particularly with regard to containment of early graft dysfunction, reduction of respiratory problems, and weight mismatch are discussed.

The real fate of pulmonary arteries after bidirectional superior cavopulmonary anastomosis: is there a need for concern?

Controversy remains about the growth of the pulmonary arteries following a bidirectional superior cavopulmonary anastomosis in children with complex cyanotic congenital cardiac malformations. This is partially due to the morphological heterogeneity of the patients, and partially due to methodological differences in series published so far. It is further complicated by the variable use, in different centres, of additional sources of pulmonary blood flow. We believe that the fate of these arteries preoperatively is significantly influenced by the amount of pulmonary blood flow and the initial size of the arteries. Separate assessment of the pulmonary arterial development postoperatively is recommended for those who, initially, had relatively small as opposed to larger than normal pulmonary arteries. Measurement of the diameters of both pulmonary arteries just prior to their first point of branching, together with the use of Z-score evaluation rather than the Nakata index, is discussed. It remains to be established whether, over time, the bidirectional cavopulmonary anastomosis is effective in developing adequately the pulmonary arteries in preparation for an ultimate total cavopulmonary connection, or even as isolated long-term palliation. A prospective, multi-institutional study involving sequential non-invasive assessment of pulmonary arterial development (using, for example, magnetic resonance imaging) is required to solve the outstanding problems.