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Background Adult congenital heart disease (ACHD) is a relatively new subspecialty in the cardiology field. The prevalence of ACHD is estimated at ∼ 3,000 per million adult population. The ACHD patient group is estimated to grow at ∼ 5% per year and in the next decade it is forecast that 1 in 150 young adults will carry some form of ACHD diagnosis. These estimates translate to ∼ 72,000 ACHD patients in Australia and ∼ 14,000 in New Zealand, although no current numbers are available. The Cardiac Society of Australia and New Zealand (CSANZ) has recently published Recommendations for Standards of Care for Adult Congenital Heart Disease (ACHD) in 2016. There is currently no long-term plan or proposal to address this huge health care burden within the federal government. This document details the size of the problem insofar as it is known and recommends solutions to be implemented. Methods This document was developed by the Adult Congenital Heart Disease Working Group of the Paediatric and Congenital Council (the Congenital Heart Alliance of Australia and New Zealand) as a response to the chronic under resourcing in this area, the risk this poses to patients and clinicians, and the clear need for long-term planning to develop safe care pathways. Results These issues were raised with the CSANZ Board in December 2015 and the document was developed in response to the Board’s request for more information. The current iteration was finalised on 14 November 2017. Conclusions The authorship group comprised participants in the CSANZ adult CHD standards of care recommendations from 2013 with the inclusion of some newly trained ACHD cardiologists, who represented most states and territories across ANZ. None of the authors has any academic or professional conflict of interest.

Aims Improved survival has resulted in increasing numbers and complexity of adults with congenital heart disease (ACHD). International guidelines recommend specialized care but many patients are still not managed at dedicated ACHD centres. This study analysed referral sources and appropriateness of management for patients referred to our tertiary ACHD Centre over the past 3 years. Methods and results We compared differences in care between patients referred from paediatric/ACHD-trained vs. general adult cardiologists, according to Adherence (A) or Non-Adherence (NA) with published guidelines. Non-Adherent cases were graded according to the severity of adverse outcome or risk of adverse outcome. Of 309 consecutively referred patients (28 ± 14 years, 51% male), 134 (43%) were from general cardiologists (19% highly complex CHD) and 115 (37%) were from paediatric cardiology or ACHD specialists (33% highly complex CHD). Sixty referrals (20%) were from other medical teams and of those, 31 had been lost to follow-up. Guideline deviations were more common in referrals from general compared to CHD-trained cardiologists (P

Although repair of Tetralogy of Fallot (TOF) and transposition of the great arteries (TGA) has facilitated survival into adulthood, many survivors have residual haemodynamic abnormalities, including exercise intolerance and late right ventricular (RV) failure.We studied 40 asymptomatic adult subjects (31.3+/-1.5 years) after congenital heart disease (CHD) surgery during childhood, using tissue Doppler echocardiography (TDE). We compared systolic (S') and diastolic (E' for early filling) RV and LV velocities, with 40 age matched controls (29.5+/-1.0 years).Both RV S' and E' velocities were significantly slower in the CHD group compared to controls (6.3+/-0.4 cm/s vs. 9.3+/-0.3 cm/s; 8.5+/-0.5 cm/s vs. 10.9+/-0.4 cm/s, respectively, p0.001 for both). By contrast, LV S' and E' velocities were similar in both groups. Interestingly, in 50% of CHD subjects where RV function was reported as 'normal', both RV S' and E' velocities were significantly slower compared with controls (6.5+/-0.6 cm/s vs. 9.3+/-0.3 cm/s, p0.0001 and 9.4+/-0.7 cm/s vs. 10.9+/-0.4 cm/s, p0.05 respectively).RV S' and E' velocities are frequently abnormal in asymptomatic survivors of TOF and TGA repair, even where RV function appears 'normal'. Hence TDE during follow up may be a sensitive means of detecting pre-clinical abnormalities in RV performance.

Background:There is concern about whether cardiac damage occurs as a result of prolonged strenuous exercise.Objective:To investigate whether competing in a triathlon is associated with cardiac damage based on a sustained increase in cardiac troponin T (cTnT), and whether such an increase correlates with echocardiographic changesMethods:cTnT and echocardiographic measurements were made in 38 participants in the 2001 Australian ironman triathlon. cTnT was measured the day before, immediately after, and the day following the race. Echocardiography was done the day before, immediately after, and two to six weeks later for measurement of ejection fraction, stroke volume, cardiac output, wall motion analysis, and global left ventricular function (LVF).Results:No subject had detectable cTnT in the pre-race sample. Following the race, 32 subjects (86.5%) had detectable levels of cTnT (>0.01 ng/ml), with six (16.2%) having >0.10 ng/ml. The day after the race, nine subjects (23.7%) still had detectable cTnT, with two recording a level >0.10 ng/ml. Previously described echocardiographic changes of “cardiac fatigue” were observed in the whole cohort. There was a modest but significant correlation between change in ejection fraction and peak cTnT level (p = 0.02,r = 0.39). Athletes with a post-race cTnT >0.10 ng/ml had a greater decrease in global LVF (p = 0.02) and a trend toward a greater fall in ejection fraction and stroke volume than athletes with cTnT levels 0.10 ng/ml (p>0.05).Conclusions:Participation in ironman triathlon often resulted in persistently raised cTnT levels, and the troponin rise was associated with echocardiographic evidence of abnormal left ventricular function. The clinical significance and long term sequelae of such damage remains to be determined.

The MRI findings of a case of coronary artery fistula occurring in a patient with pulmonary atresia and tricuspid atresia is presented.

Objective Survival of patients with repaired tetralogy of Fallot (rToF) into young adulthood is very good. Concerns exist, however, over long-term morbidity and mortality as these subjects reach middle age. We aimed to assess survival and the prevalence of complications in patients with rToF seen in our Adult Congenital Heart Disease (ACHD) service. Methods One hundred and sixty-eight consecutive patients with ‘simple rToF’, aged over 16 years, followed up at our tertiary-level ACHD service in Sydney, Australia since 2000, were included. We documented mortality and analysed the prospectively defined composite end points of (a) ‘Serious adverse events’, including death, heart failure hospitalisation and/or documented ventricular arrhythmia and (b) ‘Adverse events’ inclusive of the above and endocarditis, atrial arrhythmia, defibrillator and/or pacemaker implantation. Results Mean age at the last follow-up was 34±12 years, and 55% were men. There were 10 (6%) deaths, and 26 patients (16%) experienced a ‘serious adverse event’. Fifty-one patients (30%) experienced an ‘adverse event’ and 29 patients had atrial arrhythmias. One hundred and one (61%) patients had at least one pulmonary valve replacement. By age 40 years, 93% were free of serious adverse events, and 83% were free of any adverse event. By age 50 years, only 56% had not had an adverse event. Older age and history of atrial arrhythmia were predictive of serious adverse events. Conclusion Survival into mid-adulthood in patients with rToF is very good; however, a substantial number of survivors have adverse events by the age of 50 years.

Background Congenitally corrected transposition of the great arteries (ccTGA) is a rare congenital heart disease. There have been only few reports of sudden cardiac death (SCD) in patients with ccTGA and reasonable ventricular function. Methods A retrospective review of the medical records of all patients attending our adult congenital heart centre, with known ccTGA. Results From a database of over 3500 adult patients with congenital heart disease, we identified 39 (∼1%) with ccTGA and ‘two-ventricle’ circulations. 65% were male. The mean age at diagnosis was 12.4±11.4 years and the mean age at last time of review was 34.3±11.3 years. 24 patients (56%) had a history of surgical intervention. 8 (19%) had had pacemaker implantation and 2 had had a defibrillator implanted for non-sustained ventricular tachycardia (NSVT). In 544 years of patient follow-up, there had been five cases of SCD in our population; 1 death per 109 patient-years. Two of these patients had had previously documented supraventricular or NSVT. However, they were all classified as New York Heart Association (NYHA) class I or II, and systemic (right) ventricular function had been recorded as normal, mildly or mildly–moderately impaired, at most recent follow-up. Conclusions Our experience suggests the need for improved risk stratification and/or surveillance for malignant arrhythmia in adults with ccTGA, even in those with reasonable functional class on ventricular function.

Coronary pulmonary fistulae are rare cardiac anomalies. We present the case of a 46-year-old woman with multiple coronary-pulmonary fistulae involving all three coronary arteries. She presented with atypical chest pain and had no obstructive coronary lesion. SPECT thallium study demonstrated no perfusion defect. The fistulae were multiple but small with only a small left to right shunt (Qp:Qs = 1.2). The patient has remained well without intervention.

D. Jackson3, P. Bannon1,2,3,5, R. Cordina1,3, L. Pressley1,3, G. Sholler 1,4, D. Winlaw1,4, D. Celermajer 1,3,∗ 1 The Sydney Medical School, University of Sydney, Australia 2 Baird Institute for Applied Heart and Lung Surgical Research, Australia 3 The Cardiology and Cardiothoracic Surgery Department at Royal Prince Alfred Hospital, Australia 4 Heart Centre for Children & Adolph Basser Cardiac Institute, Children’s Hospital at Westmead, Australia 5 Sydney Cardiothoracic Surgeons, Suite 304 RPAH Medical Centre, Australia

Objectives To determine the relationship between age and aortic dilatation in patients with Marfan syndrome and to define the rate of progression of aortic dilatation in these patients. Design All patients were evaluated in a multidisciplinary clinic to establish a firm diagnosis of Marfan syndrome. Aortic dimensions were measured by echocardiography and patients with Marfan syndrome were followed up with annual physical and echocardiographic examinations to detect any change in aortic diameter over the subsequent four years. Patients One hundred and fifty-seven patients were referred to the clinic for assessment, of whom 40 exhibited diagnostic features of Marfan syndrome. Only 24 of these patients had previously been diagnosed with Marfan syndrome, while 17 other patients, previously diagnosed with Marfan syndrome, had insufficient clinical features to justify the diagnosis. Results Among the 40 patients (19 male, 21 female) with Marfan syndrome (mean age, 28 +/- 15 years), the prevalence of cardiovascular abnormalities was 90%. Aortic root dilatation was present in 78% of patients, aortic regurgitation in 28%, mitral valve prolapse in 65% and mitral regurgitation in 35%. Mean aortic root diameter in the Marfan patients (21.4 +/- 4.0 mm/m2 body surface area) markedly exceeded that of age and sex matched controls without Marfan syndrome (14.9 +/- 2.2 mm/m2) and that of first-degree relatives without Marfan syndrome (15.3 +/- 2.9 mm/m2). The occurrence of aortic dilatation in Marfan syndrome was variable, with patients as young as 20 years exhibiting severe dilatation. All patients with Marfan syndrome exhibiting aortic dilatation were advised to take beta-adrenergic blocking drugs, unless contraindicated, in an effort to retard the rate of aortic dilatation. Among 33 patients followed up for at least one year, 14 (42%) exhibited an increase in aortic diameter of at least 2 mm, while 16 of 23 patients (70%) followed up for at least three years exhibited similar progression of aortic dilatation. The overall mean rate of dilatation in the Marfan patients was 1.9 mm per year. Nine patients developed aortic dilatation of more than 50 mm diameter during four years' follow-up and required surgical repair of the aorta. Each of these patients is well at between three months' and four years' follow-up. Conclusions Aneurysmal dilatation of the aorta is a common complication of Marfan syndrome and may become manifest at an early age. Furthermore, aortic dilatation can progress rapidly, even in the absence of symptoms. Individuals with Marfan syndrome should have annual echocardiographic examinations to monitor aortic root dimensions, and those exhibiting rapid progression of aortic dilatation or an aortic root diameter in excess of 50 mm, should be considered for elective composite graft repair of the aorta.

Both glucocorticoid and mineralocorticoid hormones are known to be involved in the physiology and pathophysiology of the gastrointestinal tract. Studies on the mechanism of action of steroid hormones indicate an initial obligatory step of binding to stereospecific receptor proteins in the cytoplasm of target tissue cells. Mucosal cells from the gastrointestinal tract of adrenalectomized, gonadectomized rats contain cytoplasmic glucocorticoid receptors which bind tritiated dexamethasone with an affinity (Kdiss4C) of approximately 10(-8)M. The concentration of glucocorticoid receptors per unit cytoplasmic protein is in order duodenum greater than jejunum greater than ileum=stomach greater than colon, and their affinity for steroid hormones is in order dexamethasone greater than corticosterone greater than deoxycorticosterone=aldosterone. No glucocorticoid receptors could be demonstrated in esophageal mucosal cells. Binding sites for tritiated aldosterone, with affinity characteristics appropriate for physiological mineralocorticoid receptors, were demonstrated in duodenum, jejunum, ileum and colon. No similar sites could be shown in the mucosa of the gastric antrum.

It has been recently suggested that 16α,18-dihydroxydeoxycorticosterone (16α,18-diOHDOC) may act as a “positive allosteric effector” of the binding of aldosterone to mineralocorticoid receptors. To test this hypothesis, a series of in vitro and in vivo studies examining the effect of 16α,18-diOHDOC on tritiated aldosterone ( 3 HA) binding to mineralocorticoid receptors was performed. Using kidney slices from adrenalectomized rats, in vitro incubations were made for 20′ at 37C, over a range of concentrations of 3 HA plus tenfold dexamethasone to confine tracer binding to mineralocorticoid receptors. At no concentration of 3 HA did 16α,18-diOHDOC enhance binding; at all tracer concentrations a slight competing effect was observed. When 3 HA was injected into rats in vivo with and without 16α,18-diOHDOC, a similar insignificant displacement of 3 HA binding was seen in renal cytoplasmic fractions from adrenalectomized test rats. Additional in vitro studies were performed in an attempt to elucidate the mechanism of postulated action of 16α,18-diOHDOC. Neither renal cytoplasmic binding of oestradiol, postulated as a secondary pathway for steroid influenced Na + retention, nor the binding of dexamethasone to renal glucocorticoid receptors, was altered by 16α,18-diOHDOC. Binding of tritiated 16α,18-iOHDOC in renal cyto-plasmic fractions was shown to be non specific, in that it could not be displaced by excess unlabelled 16α,18-diOHDOC. Finally, in a series of in vivo experiments using adrenalectomized rats, we could not show any effect of 16α,18-diOHDOC on urinary electrolyte excretion, either alone or in combination with low doses of aldosterone. Accordingly, we can find no evidence for 16α-diOHDOC having a direct effect on the kidney: in particular, there would appear as yet no molecular evidence for 16α,18-diOHDOC being a positive allosteric effector of aldosterone.