Caring for adults with congenital heart disease in a regional setting.

OBJECTIVE: The number of adults with congenital heart disease has increased with improvements in surgical and medical management, posing a challenge for regional and rural settings, which might have difficulties accessing specialised professionals with congenital heart disease services. This study aims to ascertain the demographics and management of adults with congenital heart disease seen by a cardiology practice in regional Australia to better understand the needs of regional adults with congenital heart disease. DESIGN: A descriptive study using data from clinical notes collected between April 2013 and April 2016. SETTING: A private cardiology practice in Coffs Harbour, New South Wales. The practice has a treating cardiologist, senior sonographer, visiting cardiothoracic surgeon and visiting paediatric cardiologist. PARTICIPANTS: One-hundred-and-one adults with congenital heart disease (age: 16-88 years; 55 women). MAIN OUTCOME MEASURES: Congenital heart disease defect, lesion severity, referral reason, past surgery, pulmonary hypertension, cardiac surgery during the study, mortality, adherence with follow-up plans and specialist referral. RESULTS: Sixty-six patients had simple congenital heart disease, 24 had congenital heart disease of moderate complexity and 11 had congenital heart disease of great complexity. Most were referred for surveillance, seven were referred due to pregnancy and eight were new diagnoses. Six patients died, nine had cardiac operations and five were treated for pulmonary arterial hypertension. Overall adherence to the treating cardiologist's follow-up plans was 84%. All patients with congenital heart disease of great complexity were referred to congenital heart disease specialists. CONCLUSION: A substantial number of patients had complex pathology, new diagnoses or required surgery, highlighting the importance of developing integrated services with the close support of outside specialists in managing adults with congenital heart disease in regional settings.

Vascular ring: right aortic arch, mirror image branching with Kommerell's diverticulum.

Images of vascular ring-right aortic arch, mirror image branching with Kommerell's diverticulum and a brief comment on its management.

Soda bread provocation test for subjects with transient serology for coeliac disease 3 years after a population screening survey.

BACKGROUND: We have previously reported that IgA antigliadin antibodies (IgA-AGA) in the majority of healthy subjects are transient and do not indicate enteropathy, and also that an increased intake of gluten in the form of soda bread may be part of the explanation for this phenomenon. OBJECTIVE: The aim of the study was to determine whether gluten challenge with soda bread in subjects with transiently positive IgA-AGA could induce a significant titre of IgA-AGA. DESIGN: Food challenge study. METHODS: All subjects with positive IgA-AGA on screening at the time of the MONICA project in 1991 (T0) who developed negative serology at 3-year follow-up (T1) were invited to participate in a 'soda bread challenge' (1 loaf per day) for 1 month. Analysis of food intake was carried out prior to the challenge (T2) and compared to the analysis at the time of screening (T0). IgA-AGA and IgA antiendomysial antibodies (EMA) were checked pre- (T2) and post-challenge (T3). RESULTS: Ten subjects agreed to participate. Quantities of food ingested for the various categories did not differ significantly from T0 to T2. IgA-AGA titres did not differ significantly from T1 to T2 (20.2 versus 35.0, P=0.085). Mean IgA-AGA titres rose significantly between T2 and T3 (35.0 versus 40.3, P=0.005), although none of the subjects developed a significant titre of IgA-AGA. None of the subjects were positive for IgA-EMA. CONCLUSIONS: Intake of soda bread does not appear to be an important explanation as to why subjects may have a transient rise in IgA-AGA titre since none of the subjects developed a significant titre of IgA-AGA.