Correction to: Supporting Aboriginal and Torres Strait islander cultural educators and cultural mentors in Australian general practice education.

Following publication of the original article [1], the author reported that Fig. 1 was missing.

Supporting aboriginal and Torres Strait islander cultural educators and cultural mentors in Australian general practice education.

BACKGROUND: Promoting cultural competence of health professionals working with Aboriginal and Torres Strait Islander communities is key to improving health outcomes. Cultural Educators and Cultural Mentors (CE/ CMs) have critical roles in Australian general practice training, yet these are not well understood. METHODS: Guided by a CE/CM Network, our research team including experienced CE/CMs, used surveys and semi-structured interviews to explore these roles and investigate best practice in employment and support. Participants sampled from stakeholders involved in general practice education across Australia included CE/CMs, Medical Educators, General Practice Supervisors and Registrars, and representatives of Regional Training Organisations, Indigenous Health Training Posts and other key organisations. We undertook thematic analysis using a framework approach, refined further in team discussions that privileged views of CE/ CM members. RESULTS: Participants comprised 95 interviewees and 55 survey respondents. We organised our findings under three overarching themes: understandings about cultural education and mentoring; employment and support of CE/CMs; and delivery and evaluation of cultural education and mentoring. Our findings supported a central role for Aboriginal and Torres Strait Islander CE/CMs in face-to-face Registrar education about culture and history and related impacts on health and healthcare. Cultural education was reported to provide base-line learning as preparation for clinical practice whilst cultural mentoring was seen as longitudinal, relationship-based learning. Mentoring was particularly valued by Registrars working in Aboriginal and Torres Strait Islander communities. Challenges described with employment and support included difficulties in finding people with skills and authority to undertake this demanding work. Remuneration was problematic, particularly for CMs whose work-time is difficult to quantify, and who are often employed in other roles and sometimes not paid. Other improved support recommended included appropriate employment terms and conditions, flexibility in role definitions, and professional development. Recommendations concerning implementation and evaluation included valuing of cultural education, greater provision of mentoring, partnerships with Medical Educators, and engagement of CE/CMs in rigorous evaluation and assessment processes. CONCLUSIONS: Our research highlights the importance of the unique CE/CM roles and describes challenges in sustaining them. Professional and organisational support is needed to ensure delivery of respectful and effective cultural education within general practitioner training.

Exome sequencing identifies rare variants in multiple genes in atrioventricular septal defect.

PURPOSE: The genetic etiology of atrioventricular septal defect (AVSD) is unknown in 40% cases. Conventional sequencing and arrays have identified the etiology in only a minority of nonsyndromic individuals with AVSD. METHODS: Whole-exome sequencing was performed in 81 unrelated probands with AVSD to identify potentially causal variants in a comprehensive set of 112 genes with strong biological relevance to AVSD. RESULTS: A significant enrichment of rare and rare damaging variants was identified in the gene set, compared with controls (odds ratio (OR): 1.52; 95% confidence interval (CI): 1.35-1.71; P = 4.8 × 10(-11)). The enrichment was specific to AVSD probands, compared with a cohort without AVSD with tetralogy of Fallot (OR: 2.25; 95% CI: 1.84-2.76; P = 2.2 × 10(-16)). Six genes (NIPBL, CHD7, CEP152, BMPR1a, ZFPM2, and MDM4) were enriched for rare variants in AVSD compared with controls, including three syndrome-associated genes (NIPBL, CHD7, and CEP152). The findings were confirmed in a replication cohort of 81 AVSD probands. CONCLUSION: Mutations in genes with strong biological relevance to AVSD, including syndrome-associated genes, can contribute to AVSD, even in those with isolated heart disease. The identification of a gene set associated with AVSD will facilitate targeted genetic screening in this cohort.

Is a planned caesarean section in women with cardiac disease beneficial?

OBJECTIVES: In the general population, planned caesarean section is thought to be safer in high-risk situations as it avoids the greater risk of an emergency caesarean section. Only limited data exist on the optimal mode of delivery in women with structural heart disease. We investigated the relationship between mode of delivery and pregnancy outcome in women with pre-existing heart disease. METHODS: The Registry on Pregnancy and Cardiac Disease is an on-going, global, prospective observational registry of women with structural heart disease. We report on 1262 deliveries, between January 2007 and June 2011. RESULTS: The caesarean section was planned in 393 women (31%): 172 (44%) for cardiac and 221 (56%) for obstetric reasons of whom 53 delivered by emergency caesarean section. Vaginal delivery was planned in 869 (69%) women, of whom 726 (84%) actually delivered vaginally and 143 (16%) had an emergency caesarean section. Perinatal mortality(1.1 vs 2.7, p=0.14) and low apgar score (11.9 vs 10.1, p=0.45) were not significantly different in women who had a caesarean section or vaginal delivery; gestational age(37 vs 38 weeks p=0.003) and birth weight (3073 vs 2870 g p<0.001) were lower in women delivered by caesarean section compared with women delivered by vaginal delivery. In those delivered by elective or emergency caesarean section, there was no difference in maternal mortality (1.8% vs 1.5%, p=1.0), postpartum heart failure (8.8% vs 8.2% p=0.79) or haemorrhage (6.2% vs 5.1% p=0.61). CONCLUSIONS: These data suggest that planned caesarean section does not confer any advantage over planned vaginal delivery, in terms of maternal outcome, but is associated with an adverse fetal outcome.

General Practitioner Supervisor assessment and teaching of Registrars consulting with Aboriginal patients - is cultural competence adequately considered?

BACKGROUND: General Practitioner (GP) Supervisors have a key yet poorly defined role in promoting the cultural competence of GP Registrars who provide healthcare to Aboriginal and Torres Strait Islander people during their training placements. Given the markedly poorer health of Indigenous Australians, it is important that GP training and supervision of Registrars includes assessment and teaching which address the well documented barriers to accessing health care. METHODS: A simulated consultation between a GP Registrar and an Aboriginal patient, which illustrated inadequacies in communication and cultural awareness, was viewed by GP Supervisors and Medical Educators during two workshops in 2012. Participants documented teaching points arising from the consultation which they would prioritise in supervision provided to the Registrar. Content analysis was performed to determine the type and detail of the planned feedback. Field notes from workshop discussions and participant evaluations were used to gain insight into participant confidence in cross cultural supervision. RESULTS: Sixty four of 75 GPs who attended the workshops participated in the research. Although all documented plans for detailed teaching on the Registrar's generic communication and consultation skills, only 72% referred to culture or to the patient's Aboriginality. Few GPs (8%) documented a plan to advise on national health initiatives supporting access for Aboriginal and Torres Strait Islander people. A lack of Supervisor confidence in providing guidance on cross cultural consulting with Aboriginal patients was identified. CONCLUSIONS: The role of GP Supervisors in promoting the cultural competence of GP Registrars consulting with Aboriginal and Torres Strait Islander patients could be strengthened. A sole focus on generic communication and consultation skills may lead to inadequate consideration of the health disparities faced by Indigenous peoples and of the need to ensure Registrars utilise health supports designed to decrease the disadvantage faced by vulnerable populations.

What do GPs need to work more effectively with Aboriginal patients? Views of Aboriginal cultural mentors and health workers.

BACKGROUND: Given the health inequity faced by Aboriginal people, it is important that interactions with general practitioners (GPs) are effective and that GPs are aware of issues affecting culturally competent practice. METHODS: Semi-structured interviews undertaken with Aboriginal people who had a role in cultural support or mentorship of GPs and registrars in urban or large rural centres were analysed thematically. RESULTS: Fourteen participants contributed to the study. They emphasised that a culturally respectful approach was the most important attribute. Other core knowledge and skills that promote effective relationships and healthcare were categorised as attitudes and approach, communication and consultation skills, culturally aware practices and applied knowledge. DISCUSSION: Respectful attitudes and good communication and consultation skills need to be combined with knowledge of the historical, cultural, social, medical and system factors that impact on healthcare delivery for Aboriginal patients. Access to cultural mentorship or support is important to promote culturally competent practice.

Factors influencing participation in a population-based biorepository for childhood heart disease.

BACKGROUND: Consenting minors for genetics research and biobanking involves ethical and social challenges. We examined factors influencing participation rates in a population-based biorepository for childhood heart disease. METHODS: Individuals were prospectively enrolled across 7 centers in Ontario by using a standardized consent form. Individuals were approached for consent for the donation of blood/saliva (DNA), tissue, and skin from the affected individual for future genomics and stem cell research. Consent rates were compared between pediatric and adult patients and factors affecting consent were analyzed by using multiple logistic regression analysis. RESULTS: From 2008 to 2011, 3637 patients were approached. A total of 2717 pediatric patients consented (90% consent rate); mean age was 8.5 ± 5.8 years (57% male; 76% white). A total of 561 adult patients consented (92% consent rate, P = .071 versus pediatric). Factors associated with lower pediatric consent rates included younger age, race, absence of complex defects, and location of consent; these were not associated with adult consent rates. Leading causes for refusal of consent were lack of interest in research (43%), overwhelmed clinically (14%), and discomfort with genetics (11%). Concerns related to privacy, insurability, indefinite storage, and ongoing access to medical records were not the leading causes for refusal. CONCLUSIONS: The high pediatric consent rate (90%) was comparable with that of adults. Ethical, social, or legal issues were not the leading reasons for refusal of consent.

Structure and process measures of quality of care in adult congenital heart disease patients: a pan-Canadian study.

BACKGROUND: There are more adults than children with congenital heart disease. Of over 96,000 ACHD patients in Canada, approximately 50% require ongoing expert care. In spite of published recommendations, data on the quality of care for ACHD patients are lacking. METHODS: Survey methodology targeted all Canadian Adult Congenital Heart (CACH) network affiliated ACHD centers. Clinics were asked to prospectively collect outpatient and procedural volumes for 2007. In 2008, centers were surveyed regarding infrastructure, staffing, patient volumes and waiting times. RESULTS: All 15 CACH network registered centers responded. The total number of patients followed in ACHD clinics was 21,879 (median per clinic=1132 (IQR: 585, 1816)). Of the total 80 adult and pediatric cardiologists affiliated to an ACHD clinic, only 27% had received formal ACHD training. Waiting times for non-urgent consultations were 4 ± 2 months, and 4 ± 3 months for percutaneous and surgical procedures. These were beyond Canadian recommended targets at 11 sites (73%) for non-urgent consultations, at 8 sites (53%) for percutaneous interventions and 13 sites (87%) for surgery. CONCLUSIONS: Of a minimum number of 96,000 ACHD patients in Canada, only 21,879 were being regularly followed in 2007. At most sites waiting times for ACHD services were beyond Canadian recommended targets. In spite of universal health care access, published guidelines for ACHD patient structure and process measures of health care quality are not being met.

Catheter Ablation of Hemodynamically Unstable Ventricular Tachycardia in a Patient with Dextro - Transposition of the Great Arteries and Mustard's Repair.

INTRODUCTION: A patient with D-TGA and surgical repair (Mustard's procedure) presented with appropriate ICD shocks due to monomorphic ventricular tachycardia, refractory to antiarrhythmic medications. METHODS AND RESULTS: The patient underwent an electrophysiological study and catheter ablation for the VT. Substrate and pace mapping techniques, with the help of an electroanatomical mapping system, was used to localize and ablate the tachycardia successfully. CONCLUSIONS: In patients with D-TGA and Mustard's repair, scar tissue resulting from VSD repair can act as a substrate for recurrent VT. Catheter ablation of VT is useful in management of VT that occurs despite antiarrhythmic therapy and/or when it is unstable.

Expanding roles of Aboriginal health workers in the primary care setting: seeking recognition.

The work of Aboriginal health workers (AHWs) in the primary care setting is discussed, emphasising that partnership with non-Indigenous health providers such as nurses and general practitioners brings complementary skills together to improve the health care available to Aboriginal clients, and to decrease the cultural and communication barriers to delivering such health care. The diverse skills and responsibilities of AHWs, which include clinical, health promotion, education and leadership roles, are illustrated. The current focus on increasing AHW educational opportunities, and the need to recognise AHWs as core health professionals and equal members of the health care team is presented. The increasing recognition of the role of AHWs in providing cultural mentorship for non-Indigenous colleagues is discussed.

Implantable cardioverter-defibrillators in tetralogy of Fallot.

BACKGROUND: Tetralogy of Fallot is the most common form of congenital heart disease in implantable cardioverter-defibrillator (ICD) recipients, yet little is known about the value of ICDs in this patient population. METHODS AND RESULTS: We conducted a multicenter cohort study in high-risk patients with Tetralogy of Fallot to determine actuarial rates of ICD discharges, identify risk factors, and characterize ICD-related complications. A total of 121 patients (median age 33.3 years; 59.5% male) were enrolled from 11 sites and followed up for a median of 3.7 years. ICDs were implanted for primary prevention in 68 patients (56.2%) and for secondary prevention in 53 (43.8%), defined by clinical sustained ventricular tachyarrhythmia or resuscitated sudden death. Overall, 37 patients (30.6%) received at least 1 appropriate and effective ICD discharge, with a median ventricular tachyarrhythmia rate of 213 bpm. Annual actuarial rates of appropriate ICD shocks were 7.7% and 9.8% in primary and secondary prevention, respectively (P=0.11). A higher left ventricular end-diastolic pressure (hazard ratio 1.3 per mm Hg, P=0.004) and nonsustained ventricular tachycardia (hazard ratio 3.7, P=0.023) independently predicted appropriate ICD shocks in primary prevention. Inappropriate shocks occurred in 5.8% of patients yearly. Additionally, 36 patients (29.8%) experienced complications, of which 6 (5.0%) were acute, 25 (20.7%) were late lead-related, and 7 (5.8%) were late generator-related complications. Nine patients died during follow-up, which corresponds to an actuarial annual mortality rate of 2.2%, which did not differ between the primary and secondary prevention groups. CONCLUSIONS: Patients with tetralogy of Fallot and ICDs for primary and secondary prevention experience high rates of appropriate and effective shocks; however, inappropriate shocks and late lead-related complications are common.

Sudden death and defibrillators in transposition of the great arteries with intra-atrial baffles: a multicenter study.

BACKGROUND: Transposition of the great arteries with intra-atrial baffle repair is among the congenital heart defects at highest risk of sudden death. Little is known about mechanisms of sudden death and the role of implantable cardioverter defibrillators. METHODS AND RESULTS: We conducted a multicenter cohort study in patients with transposition of the great arteries to determine actuarial rates of implantable cardioverter defibrillator shocks, identify risk factors, assess underlying arrhythmias, and characterize complications. Overall, 37 patients (age, 28.0+/-7.6 years; 89.2% male) were enrolled from 7 sites. Implantable cardioverter defibrillators were implanted for primary prevention in 23 (62.1%) patients and secondary prevention in 14 patients (37.8%). Annual rates of appropriate shocks were 0.5% and 6.0% in primary and secondary prevention, respectively (P=0.0366). Independent predictors were a secondary prevention indication (hazard ratio, 18.0; P=0.0341) and lack of beta-blockers (hazard ratio, 16.7; P=0.0301). In patients with appropriate shocks, intracardiac electrograms documented supraventricular tachycardia preceding or coexisting with ventricular tachycardia in 50%. No patient with inducible ventricular tachycardia received an appropriate shock in comparison with 37.5% of noninducible patients (P=0.0429). Inappropriate shocks occurred in 6.6% per year, more so in patients of lesser weight (hazard ratio, 0.91 per kg; P=0.0168). Additionally, 14 patients (37.8%) experienced complications: 5 (13.5%) acute, 1 (2.7%) late generator related, and 12 (32.4%) late lead related. CONCLUSIONS: In patients with transposition of the great arteries, high rates of appropriate shocks are noted in secondary but not primary prevention. Supraventricular arrhythmias may be implicated in the etiology of ventricular tachyarrhythmias; beta-blockers seem protective, and inducible ventricular tachycardia does not seem to predict future events. Inappropriate shocks and late lead-related complications are common.

Expanding roles of Aboriginal health workers in the primary care setting: seeking recognition.

The work of Aboriginal health workers (AHWs) in the primary care setting is discussed, emphasising that partnership with non-Indigenous health providers such as nurses and general practitioners brings complementary skills together to improve the health care available to Aboriginal clients, and to decrease the cultural and communication barriers to delivering such health care. The diverse skills and responsibilities of AHWs, which include clinical, health promotion, education and leadership roles, are illustrated. The current focus on increasing AHW educational opportunities, and the need to recognise AHWs as core health professionals and equal members of the health care team is presented. The increasing recognition of the role of AHWs in providing cultural mentorship for non-Indigenous colleagues is discussed.