A binational registry of adults with pulmonary arterial hypertension complicating congenital heart disease.

BACKGROUND: The management of children with congenital heart disease (CHD) has improved over recent decades and several patients surviving with CHD into adulthood are increasing. In developed countries, there are now as many adults as there are children living with CHD. Pulmonary arterial hypertension (PAH) occurs in ∼ 5% of patients with CHD. AIM: We aimed to understand the characteristics and outcomes of this emerging population. METHODS: We collected data retrospectively and prospectively from 12 contributing centres across Australia and New Zealand (2010-2013). Patients were included if they had been diagnosed with PAH and CHD and had been seen once in an adult centre after 1 January 2000. RESULTS: Of 360 patients with CHD-PAH, 60% were female and 90% were New York Heart Association functional class II or III at the time of adult diagnosis of PAH. Mean age at diagnosis of PAH in adulthood was 31.2 ± 14 years, and on average, patients were diagnosed with PAH 6 years after symptom onset. All-cause mortality was 12% at 5 years, 21% at 10 years and 31% at 15 years. One hundred and six patients (30%) experienced 247 hospitalisations during 2936 patient years of follow up. Eighty-nine per cent of patients were prescribed PAH specific therapy (mean exposure of 4.0 years). CONCLUSIONS: Adults with PAH and CHD often have this diagnosis made after significant delay, and have substantial medium-term morbidity and mortality. This suggests a need for children transitioning to adult care with CHD to be closely monitored for this complication.

Pulmonary hypertension and breathlessness: is it a combination we can ignore?

On 5 May 2013 it was World Pulmonary Hypertension (PHT) Day marking three decades on from the first reported deaths in an epidemic because of toxic rapeseed (canola) oil. This epidemic provided the impetus to the first World Health Organization to set up a world symposia. World leaders of PHT met for the fifth time in Nice, France in February 2013. Although we wait the official proceedings, this meeting provides us opportunity to reflect on the current situation in Australia and New Zealand, and examine the implications for our two countries. PHT remains difficult to identify, delays in patient diagnosis persist, and breathlessness remains dominant in the diagnosis of all causes of PHT. This review examines some of the recent changes in diagnosis, our understanding of the emerging expanding epidemiology data and the patient's journeys through the healthcare system. We also review the current treatment options on monotherapy and in poly-pharmacy or combination therapy, along with the strategic management implications of the lack of funded combination therapy associated with prognosis.

Congenital heart disease-associated pulmonary arterial hypertension: preliminary results from a novel registry.

BACKGROUND/AIMS: Pulmonary arterial hypertension (PAH) frequently accompanies childhood congenital heart disease (CHD) and may persist into adult life. The advent of specific PAH therapies for PAH prompted formation of a national Australian and New Zealand registry in 2010 to document the incidence, demographics, presentation and outcomes for these patients. METHODS: This multicentre, prospective, web-based registry enrols patients with CHD-associated PAH being followed in a tertiary centre. The inclusion criteria stipulated patient age ≥16 years, a measured mean pulmonary arterial pressure >25 mmHg at rest or echocardiographical evidence of PAH or a diagnosis of Eisenmenger syndrome, and followed since 1 January 2000. A single observer collected standardised data during a series of site visits. RESULTS: Of the first 50 patients enrolled, 30 (60%) were female. The mean age (standard deviation (SD)) at the time of PAH diagnosis or confirmation in an adult centre was 27.23 (10.07) years, and 32 (64%) patients are currently aged >30 years. Fourteen (28%) patients were in World Health Organization Functional Class II and 36 (72%) in Class III at the time of diagnosis. Forty-seven of 50 (94%) had congenital systemic-pulmonary shunts, and 36 (72%) never underwent intervention. Thirteen (26%) had Down syndrome. Confirmation of PAH by recent cardiac catheterisation was available in 30 (60%) subjects. During follow up, a total of 32 (64%) patients received a PAH-specific therapy. CONCLUSIONS: CHD associated with PAH in adult life has resulted in a new population with unique needs. This registry will allow documentation of clinical course and long-term outcomes for these patients.

The Bosentan Patient Registry: long-term survival in pulmonary arterial hypertension.

BACKGROUND/AIMS: The Bosentan Patient Registry (BPR) was a prospective, multicentre, Australian registry funded by Actelion Pharmaceuticals. The primary aim of the registry was to collect survival data in patients with pulmonary arterial hypertension (PAH) treated with bosentan. METHODS: The BPR was initiated in 15 specialized PAH centres. All patients on or starting bosentan were invited to enrol. Treating physicians notified the registry if patients discontinued bosentan, because of either a change in therapy, transplantation, intervention or death. Survival data were validated against the Australian Institute of Health and Welfare National Death Index. RESULTS: Between 2004 and 2007, a total of 528 patients (mean age 59 ± 17 years) were enrolled representing 69% of patients either previously taking or initiated on bosentan during that time. The BPR population was generally older with more advanced functional deficit than patients enrolled in randomized, placebo-controlled trials. Aetiology was idiopathic (iPAH) in 58% and connective tissue disease related (scleroderma (SSc)-PAH) in 42%. For iPAH patients, World Health Organisation functional classes II, III and IV at enrolment was 8.2%, 66.4% and 20.5%, and for the SSc-PAH cohort, 3.2%, 75.8% and 17.9% respectively. The observed annual mortality was 11.8% in patients with iPAH and 16.6% in patients SSc-PAH. CONCLUSION: This large Australian registry provides 'real life' information on the characteristics and management of PAH in clinical practice. Treatment with bosentan improved survival outcomes in both iPAH and SSc-PAH compared with historical controls. Age, disease severity and aetiology were critical factors in determining clinical outcomes.

Survival after the initiation of combination therapy in patients with pulmonary arterial hypertension: an Australian collaborative report.

BACKGROUND: Several cellular pathways are implicated in the pathogenesis of pulmonary arterial hypertension (PAH) and attempts to arrest disease progression with a single drug would not be expected to succeed in the medium term. In clinical practice, combination therapy is often used in patients deteriorating on monotherapy, despite the absence of firm evidence from randomized controlled controls. METHODS: From January 2005 to August 2009, 112 patients with World Health Organisation Functional Class (FC) II-IV PAH deteriorating on monotherapy received non-parenteral combination therapy at six Australian PAH expert hospitals. Combination therapy included bosentan, sitaxentan, ambrisentan, iloprost and sildenafil. Data were prospectively collected for survival status, 6-min walk distance, FC and echocardiographic parameters at the start of monotherapy through to commencement of combination therapy and at 6-monthly intervals thereafter. RESULTS: After varying periods of monotherapy (18.7±13.4onths), survival estimates on combination therapy were 88%, 71% and 61% for the additional 1, 2 and 3years respectively. Survival on dual therapy in patients with idiopathic PAH/familial PAH was 93% at 1year and 79% at 2years, and for scleroderma-related PAH, 72% at 1 year and 48% at year 2 after initiation of combination therapy. In survivors, dual therapy reversed the deterioration in FC, from 3.1±0.6 on monotherapy to 2.2±0.6 at 12months. Similarly, dual therapy improved 6-min walk distance from 316±119m to 406±129m at 12months, and sequential echocardiography demonstrated a fall in pulmonary artery systolic pressure and improved right ventricular function. CONCLUSIONS: Dual non-parenteral therapy appears safe and effective and should be considered for PAH patients who are deteriorating on monotherapy to improve long-term outcomes.

Prevalence of pulmonary arterial hypertension in an Australian scleroderma population: screening allows for earlier diagnosis.

BACKGROUND: We sought to determine the prevalence of pulmonary complications and especially pulmonary arterial hypertension (PAH) in an Australian scleroderma population. METHODS: Between July 2005 and June 2007, physicians in Western Australia were asked to refer patients with scleroderma specifically for pulmonary hypertension screening. All patients were assessed for PAH and other respiratory conditions using echocardiography, lung function testing and clinical assessments. Right heart catheterization was carried out in patients with evidence of increased right ventricular systolic pressure. RESULTS: Of the 184 patients analysed, 44 had possible PAH on echocardiography. Right heart catheterization confirmed the diagnosis in 24 (13%). Diffuse interstitial lung disease was found in 32 patients representing a point prevalence of 17.4%. The severity of PAH at diagnosis varied according to whether the patients were referred for screening (group A) or for diagnostic (group B) purposes. The 6-min-walk test distance and median pulmonary vascular resistance were significantly worse in group B versus group A (324 vs 402 m; P= 0.02 and 884 dynes/s per cm(-5) vs 486 dynes/s per cm(-5); P < 0.01, respectively). CONCLUSION: Screening may result in earlier diagnosis of PAH with, in general more mild disease. This is important, given that early treatment for PAH while patients are less symptomatic is associated with improved exercise tolerance and pulmonary haemodynamics: indices indicative of disease progression and clinical worsening.

The perceived effectiveness of total quality management as a tool for quality improvement in emergency medicine.

OBJECTIVES: To describe the perceived effectiveness of using the Total Quality Management (TQM) approach to quality improvement in both academic and nonacademic EDs, and to discuss some important barriers to effectiveness of TQM programs. METHOD: A mail survey of 100 EDs was conducted with telephone follow-up. Hospitals were randomly selected from three subgroups: university teaching hospitals, nonuniversity teaching hospitals, and private nonteaching hospitals. ED physician directors or nonphysician administrators with knowledge of departmental quality improvement initiatives were surveyed. RESULTS: The overall response rate was 60%. Of the respondents, 54 (90.0%) used TQM techniques as part of their quality improvement initiatives. TQM techniques were used more frequently and for a longer duration in academic programs. ED staff participation in TQM projects was relatively low; less than 25% in the majority (79.6%) of all EDs. TQM initiatives were ranked least effective in university settings, of which 11 of 13 (84.6%) rated their TQM programs as ineffective or having no effect. More mature programs (>5 years old) had a significantly higher ranking for effectiveness than those programs less than 2 years old. CONCLUSIONS: Total Quality Management is being utilized in a large number of EDs. TQM initiative is perceived as having little or no positive effect. This is particularly the case in academic EDs.

Use of emergency departments by elder patients: a five-year follow-up study.

OBJECTIVE: To compare the use of emergency medical care by elders in the United States in 1995 with that previously described for 1990. METHODS: A computerized billing database of 88 EDs in 21 states was retrospectively reviewed for 1995, comparing elder and nonelder patients, estimating national use of emergency medical services by elders, and comparing the 1995 data with previously published results for 1990. RESULTS: From 1990 to 1995, the number of ED visits in the United States increased from 92 million to 100 million. The number of visits made by patients aged 65 years or older increased from 13,639,400 (15%) to 15,666,300 (15.7%), but this increase did not reach statistical significance (p = 0.17). The admission rate for elder ED patients increased from 32% to 46% over the five-year interval (p<0.01). This represents more than 7 million hospital admissions for elder patients in 1995. The rate of intensive care unit (ICU) admission for elders decreased from 7% to 6% over the five-year interval (p = 0.56), compared with 1.3% for nonelder patients for both years. Thirty percent of elder ED patients arrived by ambulance in 1990, compared with 33% in 1995 (p = 0.02). Based on 1995 data, elders comprised 39% of patients arriving by ambulance [odds ratio (OR) 4.75, 95% confidence interval (CI) = 4.71 to 4.79], 43% of all admissions (OR 6.59, 95% CI = 6.54 to 6.64), and 47% of ICU admissions (OR 5.00, 95% CI = 4.91 to 5.09). The comparable ORs in 1990 were 4.4, 5.6, and 5.5, respectively. CONCLUSIONS: From 1990 to 1995, the overall number of ED visits increased. The rate of increase was somewhat greater for elder patients. The use of ambulance services also disproportionately grew among elder patients, as did the rate of hospital admission. The overall rate of ICU admission was stable, but actually fell modestly for elder patients. Of these changes, only the increase in the rate of hospital admission for elders reached statistical significance.

Resident service hours in emergency medicine. SAEM Education Committee.

OBJECTIVE: The number of hours worked by residents in all specialties has become a controversial issue. Residents often are expected to competently conduct patient care activities and to take educational advantage of clinical experiences in spite of frequent fatigue and sleep deprivation. This survey of residency directors was designed to assess the scheduled clinical time for emergency medicine (EM) residents. METHODS: A 13-question survey dealing with time commitments of EM residents was sent to the residency directors of all accredited EM residency programs in the United States in the fall of 1991. Residency directors were asked to indicate the number of shifts, hours, and days off per week; and the number of night shifts and weekend days off per month for each postgraduate year of residency training (PGY1-PGY4). Directors also were asked whether shifts were scheduled randomly or predictably with progression from days to nights with time off after nights. RESULTS: Seventy of 71 (98.6% response rate) residency directors responded. Residents were scheduled for an average of 49.1 hours per week. Scheduled hours decreased from an average of 51.9 at the PGY1 level to an average of 44.5 at the PGY4 level. A similar progression with year of training was noted for scheduled night shifts/month, days off/week, and weekend days off/month. A PGY1 trainee averaged 7.0 night shifts/month, 1.9 days off/week, and 3.0 weekend days off/month; while a PGY4 trainee averaged 5.3, 2.4, and 3.2, respectively. Only 40% of the directors reported predictable scheduling progressing from days to nights. CONCLUSION: Emergency medicine resident schedules, as reported by residency directors, fall well within current specialty-specific requirements and compare favorably with the reported numbers for other specialties. However, because large ranges in scheduling parameters were reported, the data may be of value to residency directors, residents, and prospective residents. Most programs did not report a predictable schedule progression of shifts.

Influence of food enrichment on cage side preference.

A preference test paradigm was used to assess the value of two enrichment techniques for rhesus macaques (Macaca mulatta): 1) a Kong toy stuffed with food treats and 2) a fleece board covered with particulate food. The duration of time spent in the enriched cage side was compared with that spent in the unenriched cage side. Additionally, the number of cage side changes made during an observation interval and the duration and frequency of occurrence of select behaviors were recorded. Half the subjects altered their cage side preference during the experimental condition, and a fifth animal reversed side preference in the postexperimental phase. Subjects spent a mean time of 14% of a session engaged with the foraging devices. The occurrence of several behaviors, including self-directed and locomotor activities, varied significantly with the experimental condition. These results were compared with data from a previous preference study of nonnutritive enrichments, and a hypothesis regarding the relative value of different types of enrichment was developed.

Role of emergency medicine residency programs in determining emergency medicine career choice among medical students.

STUDY OBJECTIVE: To characterize the role of emergency medicine residency programs in determining emergency medicine career choice among medical students. DESIGN: Observational, cross-sectional, descriptive study. Information on student career choice was obtained through a targeted query of the National Resident Matching Program data base, simultaneously stratified by specialty and school, and adjusted for class size. PARTICIPANTS: All accredited emergency medicine residency programs and four-year allopathic medical schools. RESULTS: Fifty-two schools (42%) had a closely affiliated emergency medicine residency program, ie, one based primarily at the institution's main teaching hospital(s). This configuration was associated with a 70% increase in the median proportion of students choosing emergency medicine as a career when compared to the 73 schools with no closely affiliated emergency medicine residency (5.1% vs 3.0%, P < .0001). When institutions were stratified by overall commitment to emergency medicine, the median proportion of students choosing emergency medicine as a career was 2.9% for institutions with a minimal commitment to emergency medicine (neither an academic department of emergency medicine nor a closely affiliated emergency medicine residency), 4.1% for institutions with a moderate commitment to emergency medicine (either a department of emergency medicine or an emergency medicine residency, but not both), and 5.7% for institutions with a substantial commitment to emergency medicine (a department of emergency medicine and an emergency medicine residency) (P < .0001). When institutional commitment to emergency medicine was examined in a simple multivariate model, only the presence of an emergency medicine residency was associated independently with student career choice (P < .001). CONCLUSION: An emergency medicine residency program that is closely affiliated with a medical school is strongly and independently associated with a quantitatively and statistically significant increase in the proportion of students from that school who choose a career in emergency medicine. These data support the proposition that, if emergency medicine is to meet national manpower shortage needs by attracting students to the specialty, it must establish residency programs within the primary teaching hospital(s) of medical schools. Such a configuration does not currently exist in the majority of schools.

Current status of academic emergency medicine within academic medicine in the United States.

OBJECTIVE: To characterize the status of emergency medicine within U.S. academic medical centers. METHODS: All accredited emergency medicine residency programs and all four-year allopathic medical schools in the United States were identified. Institutions were defined as academic medical centers based upon NIH research grant funding. These institutions were ranked using five measures of academic stature: a survey of medical school deans, a survey of internal medicine residency directors, level of research funding, characteristics of the student body, and an unweighted composite variable reflecting overall academic stature. The relationship between institutional academic stature and an empiric scale of institutional affiliation with emergency medicine was assessed. RESULTS: Sixty-two institutions were designated academic medical centers. These medical schools captured 90% of all NIH grant monies awarded in fiscal year 1990. Twenty-six of 87 emergency medicine residency programs (30%) were closely affiliated with one of these medical schools. Within academic medical centers, the presence of a residency or an academic department of emergency medicine was inversely associated with the medical school deans' ranking (p < 0.005), research rank (p < 0.001), and composite academic rank (p < 0.001). CONCLUSION: The majority of emergency medicine residency programs (70%) are not closely affiliated with institutions receiving the bulk (90%) of NIH resources for research. Within the institutions receiving the majority of NIH funding, there is a quantitatively and statistically significant inverse association of institutional emergency medicine affiliation and institutional academic rank.

Kong toys for laboratory primates: are they really an enrichment or just fomites?

Simple toys as enrichment devices have been associated with a rapid decline in their use by nonhuman primates. Other facets of toy presentation have not been described previously. For example, a comparison of the effect(s) of an enrichment device between two facilities should be validated if enrichment recommendations are to be made that affect diverse research facilities across the country. Additionally, a comparison of two methods of presentation (one highly accessible to the animal and the other less accessible) of the same enrichment device for potential differences in efficacy could provide direction in implementing an enrichment program based on simple toys. The handling of enrichment devices by nonhuman primates can lead to the spread of microbial contamination. The typical enrichment program rotates enrichment devices among animals to maximize the variety of stimuli available to each primate in the most economic manner. An adequate sanitation program is therefore pivotal to minimizing the potential for enrichment devices to be fomites. We conducted three experiments that addressed these issues. The results confirmed that, although the presence of a simple toy reduced behavioral pathology, there was variability in behavioral effect for an enrichment technique between facilities. Two methods of presentation (on floor and suspended) of a simple toy did not produce any significant differences in use. Finally, we demonstrated that microbial growth can persist on enrichment devices after they have been sanitized in a commercial cagewasher.

Use of emergency departments by elderly patients: projections from a multicenter data base.

STUDY OBJECTIVES: To assess the use of emergency medical care by the elderly in the United States, including emergency department visits, level of ED care required, ambulance services, and hospital admission rate. SETTING AND PARTICIPANTS: A multicenter computerized data base of 70 hospitals in 25 states. DESIGN: A retrospective review of elderly patients seeking ED care and comparison of elderly and nonelderly patients. The data were then used to estimate the use of emergency medical services nationally. MEASUREMENTS AND MAIN RESULTS: Fifteen percent of the 1,193,743 ED visits were made by patients 65 years or older. Thirty-two percent of elderly patients seen in EDs were admitted to the hospital, compared with 7.5% of nonelderly patients. Seven percent of elderly patients were admitted to ICUs, compared with 1% of nonelderly patients. Thirty percent of elderly patients seeking emergency care used ambulance transports compared with 8% of nonelderly. It is estimated that 13,693,400 elderly patients were seen in EDs in 1990, with more than 4 million patients admitted to hospitals. Compared with the nonelderly, the elderly are 4.4 times more likely to use ambulance transport, 5.6 times more likely to be admitted to the hospital, 5.5 times more likely to be admitted to an intensive care bed, and 6.1 times more likely to be classified as a comprehensive ED level of service. In our sample, 36% of all patients arriving by ambulance to the ED, 43% of all ED admissions, and 48% of all intensive care admissions were geriatric patients. CONCLUSION: With the rapid growth of the size of the elderly population, it is important that we assess the emergency medical resources needed to care for the geriatric population.

A prospective study of the impact of helmet usage on motorcycle trauma.

STUDY OBJECTIVE: To determine the effect of the use of a motorcycle helmet on reducing the mortality, morbidity, and health care costs resulting from motorcycle crashes. DESIGN: A prospective, multicenter study of all eligible motorcycle crash victims. SETTING: The emergency departments of eight medical centers across the state of Illinois, including representatives from urban, rural, teaching, and community facilities. TYPE OF PARTICIPANTS: All motorcycle crash victims presenting less than 24 hours after injury for whom helmet information was known. Data were collected from April 1 through October 31, 1988. MEASUREMENTS AND MAIN RESULTS: Fifty-eight of 398 patients (14.6%) were helmeted, and 340 (85.4%) were not. The nonhelmeted patients had higher Injury Severity Scores (11.9 vs 7.02), sustained head/neck injuries more frequently (41.7 vs 24.1%), and had lower Glasgow Coma Scores (13.73 vs 14.51). Twenty-five of the 26 fatalities were nonhelmeted patients. By logistic regression, the lack of helmet use was found to be a major risk factor for increased severity of injury. A 23% increase in health care costs was demonstrated for nonhelmeted patients (average charges $7,208 vs $5,852). CONCLUSION: Helmet use may reduce the overall severity of injury and the incidence of head injuries resulting from motorcycle crashes. A trend toward higher health care costs was demonstrated in the nonhelmeted patients.

Computerized tracking of emergency medicine resident clinical experience.

Although we commonly assume that because residents spend a given number of months in the emergency department they achieve adequate exposure to all necessary clinical entities, this has never been shown. We suspect, rather, that great variability exists among residents in the number and variety of patients they see; and that with respect to the ED, there are important diagnoses that are rare or absent in the clinical pathology of a training program. To confirm these hypotheses, we implemented a computerized system of recording patients and diagnoses managed in the ED by the 33 residents of the University of Illinois Affiliated Hospitals Emergency Medicine Residency. We collected data for nine months and accumulated 2,152 shifts of clinical experience. These data confirm our hypotheses. We found that senior residents managed an average of 11.9 +/- 2.3 patients per ten-hour shift, but the quickest resident saw almost twice as many patients as the slowest. Junior residents saw fewer patients, 8.5 +/- 1.4 patients per shift, but maintained a twofold difference between the fastest and slowest. Furthermore, there are important diagnoses that present too rarely for each resident to become facile in their management. We found that 22.7% of the 554 diagnoses listed in the Emergency Medicine Core Content never once presented to the ED. An additional 34.7% of these diagnoses did present, but so rarely that each resident could not possibly manage one case during a residency. The Length of Training Report of the American College of Emergency Physicians provides objective guidelines for the number of encounters a resident should have with 283 clinical entities. In this study, residents fell short of these guidelines with 50.5% of diagnoses. While absolute quantity of exposure does not assure competence in management, we recommend that each residency monitor the experience of its residents. This allows a residency to change its curriculum to make optimum use of available pathology, as well as to supplement deficiencies in clinical experience with case simulations.