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This study sought to investigate the characteristics, morbidity (including the rate of infective endocarditis and valve replacement) and mortality of individuals undergoing percutaneous pulmonary valve implantation in Australia and New Zealand since the procedure has been performed.The outcomes of percutaneous pulmonary valve implantation in Australia and New Zealand have not been evaluated. Recent international data, including patients from New Zealand, suggests the rate of infective endocarditis is not insignificant.A retrospective multi-site cohort study was undertaken via medical record review at the centres where percutaneous pulmonary valve implantation has been performed. All procedures performed from 2009-March 2018 were included. Individuals were identified from local institution databases. Data was collected and analysed including demographics, details at the time of intervention, haemodynamic outcome, post procedure morbidity and mortality. Multi-site ethics approval was obtained.One hundred and seventy-nine (179) patients attended the cardiac catheter laboratory for planned percutaneous pulmonary valve implantation. Of these patients, 172 underwent successful implantation. Tetralogy of Fallot and pulmonary atresia were the most common diagnoses. The median age at procedure was 19 years (range 3-60 yrs). There was a significant improvement in the acute haemodynamics in patients undergoing percutaneous pulmonary valve implantation for stenosis. Seven (7) patients (3.9%) experienced a major procedural/early post procedure complication (death, conversion to open procedure, cardiac arrest), including two deaths. The annualised rates of infective endocarditis and valve replacement were 4.6% and 3.8% respectively. There was one death related to infective endocarditis in follow-up.Percutaneous pulmonary valve replacement is a relatively safe method of rehabilitating the right ventricular outflow tract.

In a male patient with D-transposition of the great arteries post Mustard repair, we report the inadvertent placement of an atrial pacemaker lead across the baffle into the pulmonary venous atrium managed with extraction and re-implantation.

Background: For patients with d-loop transposition of the great arteries (d-TGA) with a systemic right ventricle after an atrial switch operation, there is a need to identify risks for end-stage heart failure outcomes. Objectives: The authors aimed to determine factors associated with survival in a large cohort of such individuals. Methods: This multicenter, retrospective cohort study included adults with d-TGA and prior atrial switch surgery seen at a congenital heart center. Clinical data from initial and most recent visits were obtained. The composite primary outcome was death, transplantation, or mechanical circulatory support (MCS). Results: From 1,168 patients (38% female, age at first visit 29 ± 7.2 years) during a median 9.2 years of follow-up, 91 (8.8% per 10 person-years) met the outcome (66 deaths, 19 transplantations, 6 MCS). Patients experiencing sudden/arrhythmic death were younger than those dying of other causes (32.6 ± 6.4 years vs 42.4 ± 6.8 years; P < 0.001). There was a long duration between sentinel clinical events and end-stage heart failure. Age, atrial arrhythmia, pacemaker, biventricular enlargement, systolic dysfunction, and tricuspid regurgitation were all associated with the primary outcome. Independent 5-year predictors of primary outcome were prior ventricular arrhythmia, heart failure admission, complex anatomy, QRS duration >120 ms, and severe right ventricle dysfunction based on echocardiography. Conclusions: For most adults with d-TGA after atrial switch, progress to end-stage heart failure or death is slow. A simplified prediction score for 5-year adverse outcome is derived to help identify those at greatest risk.

Background More women with congenital heart disease (CHD) are surviving to childbearing age. In this population, pregnancy results in a higher rate of adverse events for the mother and offspring. We sought to evaluate outcomes in our cohort and relate these to previously developed risk classifications. Method We retrospectively reviewed all pregnancies occurring in our tertiary referral centre CHD cohort between 2007 and 2019 resulting in data from 128 pregnancies in 89 women. The mean age was 29±6 years. Underlying cardiac diagnoses were grouped according to the ESC Registry of Pregnancy and Cardiac disease (ROPAC) classification and baseline risk assessed as per the modified WHO classification. Results There were a wide range of underlying diagnoses and large number of moderate to high risk pregnancies with 57 (44.5%) classified as mWHO III or IV. There were no maternal deaths. The mean gestation at delivery was 37 weeks. The majority delivered vaginally. Adverse events occurred in 80 pregnancies (63%). Cardiovascular events in 21 (16%), obstetric 54 (42%) and neonatal 52 (41%). Common events included premature labour and delivery in 21 pregnancies (16%); post-partum haemorrhage in 33 (26%), small for gestational age infants in 38 (30%) and admission to the NICU in 23 (18%). Event rates increased in women classified as higher risk by mWHO group. Conclusion Women with CHD have increased rates of adverse cardiovascular, obstetric and neonatal events in pregnancy. As expected, adverse outcomes occur more frequently in higher risk mWHO groups.

PURPOSE To analyze the short-term (up to 1 month) clinical outcomes in patients undergoing corneal laser refractive surgery and the impact on dry eye disease (DED) metrics and corneal nerves using in vivo confocal microscopy (IVCM). METHODS The unaided distance visual acuity, corrected distance visual acuity, and spherical equivalent refraction (SEQ) were determined in 16 and 13 patients undergoing FS-LASIK and SMILE, respectively. DED metrics assessed were Ocular Surface Disease Index, Dry Eye Questionnaire 5-items (DEQ-5), tear film osmolarity, tear meniscus height, noninvasive keratograph breakup time (NIKBUT), ocular staining, and meibomian gland atrophy. An automated analysis of corneal nerve fiber density, corneal nerve branch density, corneal nerve fiber length (CNFL), and corneal nerve fiber fractal dimension were obtained from the IVCM scans using ACCMetrics software (University of Manchester). RESULTS Both surgical techniques provided good refractive and visual outcomes. DED symptoms were found to be higher after FS-LASIK compared with SMILE (P < 0.05). A decrease in tear meniscus height (∼31%) and NIKBUT (∼40%) was reported after FS-LASIK (P = 0.005 and P = 0.001, respectively) but not after SMILE. Both procedures affected corneal nerve fiber density, corneal nerve branch density, CNFL, and corneal nerve fiber fractal dimension, but the impact was significantly greater with FS-LASIK (P = 0.001). Only CNFL correlated with the reported symptoms (DEQ-5) after FS-LASIK (r = -0.545, P = 0.029). CONCLUSIONS FS-LASIK and SMILE provided good refractive and visual outcomes. There was an increased impact on DED symptoms after FS-LASIK compared with SMILE, although there were no significant differences between the procedures for most of the other ocular surface metrics assessed. The IVCM findings showed that SMILE had less impact on corneal nerves compared with FS-LASIK.

Accurate risk stratification is important in pregnant women including the growing population with congenital heart disease (CHD). We meta-analysed the performance of risk models for CHD women. Six studies with 3426 pregnancies were studied, with cardiac complication rates of 6.7-20.6%. Pooled c-statistics (95% confidence interval) for scores were mWHO 0.71 (0.65-0.76), CARPREG 0.66 (0.61-0.71) and ZAHARA 0.71 (0.65-0.76). Current risk models had at best moderate discrimination for CHD women with significant room for improvement.

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.

Background: Although several factors have been cited for risk stratification in patients with simple transposition of the great arteries (dTGA), no single predictor emerges consistently. We aimed to assess survival and determine factors associated with survival in a large cohort of dTGA adults with atrial switch. Methods and Results: We included 1,169 dTGA adults (median age 28.1 years, 38.7% female) under regular follow-up at 28 institutions between 2002 and 2019. The primary outcome was a composite of death, mechanical circulatory support (MCS) and heart transplant. During a median follow-up of 9.2 [IQR 5.5-14.2] years, 67 (5.7%) patients died, six (0.5%) patients underwent MCS and 21 (1.8%) had a heart transplant. Cumulative incidence of composite endpoint at 15 years was 12.8% [95% CI 9.8 - 15.7], see Figure). Median age at time of primary outcome was 39.5 [IQR 33.9 - 45.1] years. Leading causes of death were worsening of heart failure (34%), non-cardiac (21%) and sudden unexplained death (12%). In multivariable Cox analyses for baseline variables, age, VSD, ventricular arrhythmia and heart failure admission were each associated with increased risk of the outcome, whereas prior pacemaker (26% of patients) was not. New pacemaker implantation was performed in 107 (9.1%), ICD in 109 (9.3%), and cardiac surgery in 35 (3%) patients. Patients who died were more likely to develop arrhythmias, be admitted for heart failure or require surgery during follow-up. Conclusion: In this large contemporary cohort of dTGA adults after atrial switch, late survival was excellent and seemed to be determined by arrhythmia and heart failure-related complications. Few patients underwent advanced heart failure therapies. Figure. Cumulative incidence of the composite primary outcome (MCS, heart transplant or death) over a period of 15 years from first visit at an adult congenital heart disease clinic since 2002. Shading represents upper and lower 95% confidence limits.

Background: Occasionally patients with congenitally corrected transposition of the great arteries (ccTGA) exhibit little clinical evidence of cardiovascular limitation even to their 8th decade. We aimed to assess survival prospects in a large cohort of ccTGA adults. Methods & Results: We included 555 ccTGA adults (median age 33.0 years, 48.3% female) under regular follow-up at 28 institutions between 2002 and 2019. The primary outcome was a composite of death, mechanical circulatory support (MCS) and heart transplant. During a median follow-up of 8.1 [IQR 4.4 - 13.3] years, 56 (10.1%) patients died, 10 (1.8%) patients underwent MCS and 14 (2.5%) had a heart transplant. Median age at time of primary outcome was 51.1 [IQR 37.5 - 63.2] years and cumulative incidence at 15 years from baseline was 21.5% [95% CI 16.1 - 26.5]. Leading causes of death were worsening of heart failure (43%) and sudden death (10%). Patients who died were more likely to use heart failure (HF) medications. In multivariable Cox analyses for baseline variables, age, prior atrial arrhythmia and HF admission were each associated with an increased risk of the primary outcome. Figure shows cumulative incidence according to history of atrial arrhythmia. During follow-up, 91 (16.4%) were admitted for HF, pacemaker implantation was performed in 68 (12.3%) patients, ICD in 82 (14.7%), and major cardiac surgery (mostly for systemic AV-valve) in 89 (15.8%) patients. Conclusion: In this large cohort of ccTGA adults, survival seemed to be primarily determined by heart failure-related complications. Prior atrial arrhythmia also seems to be a harbinger for adverse outcome. Few patients underwent advanced HF therapies. Figure: Cumulative incidence of the composite primary outcome (MCS, heart transplant or death) over a period of 14 years from first visit at an adult congenital heart disease clinic since 2002 stratified according to history of atrial arrhythmia. Shading represents upper and lower 95% confidence limits.

The Fontan circulation describes the circulatory state resulting from an operation in congenital heart disease where systemic venous return is directed to the lungs without an intervening active pumping chamber. As survival increases, so too does recognition of the potential health challenges. This document aims to allow clinicians, people with a Fontan circulation, and their families to benefit from consensus agreement about management of the person with a Fontan circulation. The document was crafted with input from a multidisciplinary group of health care providers as well as individuals with a Fontan circulation and families. It is hoped that the shared common vision of long-term wellbeing will continue to drive improvements in care and quality of life in this patient population and eventually translate into improved survival. KEYPOINTS.

BackgroundInfective endocarditis has been reported post Melody percutaneous pulmonary valve implant; the incidence and risk factors, however, remain poorly defined. We identified four cases of endocarditis from our first 25 Melody implants. Our aim was to examine these cases in the context of postulated risk factors and directly compare endocarditis rates with local surgical valves.MethodsWe conducted a retrospective review of patients post Melody percutaneous pulmonary valve implant in New Zealand (October, 2009–May, 2015) and also reviewed the incidence of endocarditis in New Zealand among patients who have undergone surgical pulmonary valve implants.ResultsIn total, 25 patients underwent Melody implantation at a median age of 18 years. At a median follow-up of 2.9 years, most were well with low valve gradient (median 27 mmHg) and only mild regurgitation. Two patients presented with life-threatening endocarditis and obstructive vegetations at 14 and 26 months post implant, respectively. Two additional patients presented with subacute endocarditis at 5.5 years post implant. From 2009 to May, 2015, 178 surgical pulmonic bioprostheses, largely Hancock valves and homografts, were used at our institution. At a median follow-up of 2.9 years, four patients (2%) had developed endocarditis in this group compared with 4/25 (16%) in the Melody group (p=0.0089). Three surgical valves have been replaced.ConclusionsThe Melody valve offers a good alternative to surgical conduit replacement in selected patients. Many patients have excellent outcomes in the medium term. Endocarditis, however, can occur and if associated with obstruction can be life threatening. The risk for endocarditis in the Melody group was higher in comparison with that in a contemporaneous surgical pulmonary implant cohort.

In the first of two reports, Dr Irene Siso-Fuertes and Dr Clare O’Donnell review days one and two of the ESCRS 2020 virtual conference

In the second report from Dr Irene Siso-Fuertes and Dr Clare O’Donnell of the ESCRS 2020 virtual conference, discussion turns to myopia and surgical developments

Purpose Dry eye is known to impact on clinical outcomes after laser vision correction and the use of a newer ‘all femtosecond laser’ surgical approach may be associated with less impact on the ocular surface post-operatively. The purpose of this study was to evaluate the early clinical outcomes and tear instability after the first small incision lenticule extraction (SMILE) cases undertaken by three surgeons at a single site in the UK. Methods Retrospective audit. Seventy-one eyes of 37 patients underwent SMILE surgery using the Zeiss VisuMax laser system (Carl Zeiss Meditec, Germany). Uncorrected and corrected distance visual acuity, spherical equivalent refraction, fluorescein enhanced tear break up time, simulated keratometry and complications were evaluated pre- and post-operatively where applicable. Results The study population consisted of 21 males and 16 females. The mean ± standard deviation age was 33 ± 8 years. The results showed that 100% of eyes achieved 20/40 or better and 88% achieved 20/20 or better uncorrected distance visual acuity. The spherical equivalent refraction after surgery was within ±0.50D in 82% of eyes at three months. There was no significant difference in tear break up time from pre-operative levels at three months. Complications were infrequent. Conclusions This early data from surgeons’ first SMILE procedures suggest SMILE provides good outcomes in terms of refractive predictability and visual acuity with minimal impact on the tear film. Longitudinal research will further improve our understanding of the longer-term impact of SMILE on clinical outcomes, ocular surface metrics and patient reported outcomes.

Purpose Cataract surgery can lead to the temporary development or worsening of dry eye symptoms. Contributing factors may include corneal incisions, agents used before, during or after surgery, light from the operating microscope, disruption of ocular surface tissues and inflammatory processes. The purpose of this study was to observe the effect of light exposure on conjunctival fibroblasts in order to determine whether light has an effect on wound healing closure, assuming that operating microscopes might have an effect on the ocular surface. Method An in vitro scratch assay was performed on porcine conjunctival fibroblasts. Ten minutes of light exposure from a light microscope with a halogen bulb was performed after the scratch assay. Fibroblasts were kept in culture for 48 hours post-exposure and the wound closure rates were visualized by live/dead staining. The fibroblasts which were exposed to light were compared to those without light exposure. Cell viability was also analysed by MTT assay. Results A Slower wound closure rate was found when fibroblasts were exposed to light compared to the non-light exposed controls. Cell viability reduced by 20% with light exposure compared to controls in p3 cells (p = 0.04; however, the trend was not observed with p4 and p5 cells (p > 0.05). Conclusions These results suggest that light exposure might be one of the possible contributory factors for dry eye after ophthalmic surgery. Further evaluation of light effects should be carried out with different ocular surface cells.

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.

The number of adults with congenital heart disease (CHD) has increased markedly over the past few decades as a result of astounding successes in pediatric cardiac care. Nevertheless, it is now well understood that CHD is not cured but palliated, such that life-long expert care is required to optimize outcomes. All countries in the world that experience improved survival in CHD must face new challenges inherent to the emergence of a growing and aging CHD population with changing needs and medical and psychosocial issues. Founded in 1992, the International Society for Adult Congenital Heart Disease (ISACHD) is the leading global organization of professionals dedicated to pursuing excellence in the care of adults with CHD worldwide. Recognizing the unique and varied issues involved in caring for adults with CHD, ISACHD established a task force to assess the current status of care for adults with CHD across the globe, highlight major challenges and priorities, and provide future direction. The writing committee consisted of experts from North America, South America, Europe, South Asia, East Asia, and Oceania. The committee was divided into subgroups to review key aspects of adult CHD (ACHD) care. Regional representatives were tasked with investigating and reporting on relevant local issues as accurately as possible, within the constraints of available data. The resulting ISACHD position statement addresses changing patterns of worldwide epidemiology, models of care and organization of care, education and training, and the global research landscape in ACHD.

Background There is increasing use of portable echocardiography as a screening test for rheumatic heart disease (RHD). The prevalence of valvular regurgitation in healthy populations as determined using portable echocardiography has not been well defined. Minimal echocardiographic criteria for RHD have recently been clarified, but the overlap of normal and abnormal valvular regurgitation warrants further study. The aim of this study was to determine the spectrum of echocardiographic findings using portable echocardiography in children from a population with low prevalence of RHD. Methods Screening echocardiography was conducted in 396 healthy students aged 10 to 12 years using portable echocardiographic equipment. Echocardiograms were assessed according to 2012 World Heart Federation criteria for RHD. The prevalence of physiologic valvular regurgitation was compared with that found in previous studies of children using large-platform machines. Results Physiologic mitral regurgitation (MR) was present in 14.9% of subjects (95% CI, 11.7%–18.7%) and pathologic MR in 1.3% (95% CI, 0.6%–2.9%). Two percent (95% CI, 1.0%–3.9%) had physiologic aortic regurgitation, and none had pathologic aortic valve regurgitation. Physiologic tricuspid regurgitation was present in 72.7% of subjects (95% CI, 68.1%–76.9%) and physiologic pulmonary regurgitation in 89.6% (95% CI, 85.7%–91.8%). After cardiology review, no cases of definite RHD were found, but 0.5% of patients (95% CI, 0.1%–1.8%) had pathologic MR meeting World Heart Federation criteria for borderline RHD. Two percent (95% CI, 1.4%–4.6%) of the cohort had minor forms of congenital heart disease. Conclusions The spectrum of physiologic cardiac valvular regurgitation in healthy children as determined using portable echocardiography is described and is within the range of previous studies using large-platform echocardiographic equipment. The finding of two children with pathologic-grade MR, likely representing the upper limit of physiologic regurgitation, has implications for echocardiographic screening for RHD in high-prevalence regions.

To assess the biomechanical changes of collagen cross-linking on keratoconic corneas in vitro.Six keratoconic corneal buttons were included in this study. Each cornea was divided into two halves, where one half was cross-linked and the other half was treated with riboflavin only and served as control. The biomechanical changes of the corneal tissue were measured across the stroma using scanning acoustic microscopy (SAM).In the cross-linked corneas, there was a steady decrease in the magnitude of speed of sound from the anterior region through to the posterior regions of the stroma. The speed of sound was found to decrease slightly across the corneal thickness in the control corneas. The increase in speed of sound between the cross-linked and control corneas in the anterior region was by a factor of 1.039×.A higher speed of sound was detected in cross-linked keratoconic corneal tissue when compared with their controls, using SAM. This in vitro model can be used to compare to the cross-linking results obtained in vivo, as well as comparing the results obtained with different protocols.

Aim Ileocolonic neuroendocrine tumours (NETs) are diagnosed as part of bowel cancer screening programmes (BCSPs). The aim of this study was to identify and characterize NETs diagnosed within the English BCSP, a double-screen programme that uses guaic faecal occult blood test (gFOBT) screening and colonoscopy, by interrogating the national colorectal screening database and validating the findings with individual BCSP centres. Method The Exeter database was interrogated by running queries to identify participants with coded NETs (from the start of the programme in July 2006 - 1 December 2014). A written proforma was sent to the responsible BCSP clinician for validation and characterization. Results During this period, 13 061 716 participants were adequately screened using gFOBTs, and 259 765 participants had definitively abnormal results. There were 146 unique participants with NET-related codes from 216 707 BCSP colonoscopies. The diagnosis rates per 100 000 colonoscopies were 29 rectal, 18 colonic and 11 ileal NETs. The majority of rectal NETs had Grade 1 (80%) and Stage T1 (85.1%) disease. Over half of ileal NETs (53.6%) in this study had invasive disease, with 85.2% having nodal and 36.1% having metastastatic disease. Conclusion The current study highlights the rate of colorectal NETs diagnosed in the English BCSP. These data highlight a higher-than-anticipated incidence, and the potential additional benefit of BCSPs in identifying occult NETs.

ObjectiveEisenmenger syndrome (ES) is a severe form of pulmonary hypertension in adults with congenital heart disease (CHD) and has a poor prognosis. We aimed to understand factors associated with survival in ES and particularly to assess the potential benefits of advanced pulmonary vasodilator therapy (AT).MethodsFrom January 2004, when AT became generally available for patients with ES, we followed 253 ES adults from 12 adult congenital heart disease centres across Australia and New Zealand. Demographic, medical and outcome data were collected and analysed prospectively and retrospectively.ResultsThe patients with ES were predominantly female (60%), aged 31 (SD 12) years. At diagnosis of ES, 64% were WHO functional class ≥3. The most common underlying lesion was ventricular septal defect (33%) with 21% having ‘complex’ anatomy. Over a median follow-up time of 9.1 years, the majority (72%) had been prescribed at least one AT (49% single agent), mostly bosentan (66%, 168 patients). The mean time on AT was 6 (SD 3.6) years. Those on AT were more functionally impaired at presentation (69% WHO ≥3 vs 51%, p=0.007) and more likely to have been prescribed anticoagulation (47% vs 27%, p=0.003). The risk of death/transplant was 4.8 %/year in AT exposed versus 8.4% in those never exposed. On multivariable analysis, exposure to AT was independently associated with greater survival (survival HR 2.27, 95% CI 1.49 to 3.45; pConclusionTreatment with AT was independently associated with greater survival in patients with ES, even though they were comparatively sicker prior to treatment.

BackgroundTranscatheter device closure has become the established standard of care for suitable atrial septal defects. Device erosion has been a recent focus and has prompted changes in the Instructions for Users documentation released by device companies. We reviewed our entire local experience with atrial septal defect device closure, focussing on the evolution of this procedure in our centre and particularly on complications.MethodsWe carried out a retrospective review of 581 consecutive patients undergoing attempted transcatheter device closure of an atrial septal defect in Auckland from December 1997 to June 2014. We reviewed all complications recorded and compared our outcomes with the current literature. We sought to understand the impact of the evolution in recommendations and clinical practice on patient outcomes in our programme.ResultsThere were a total of 24 complications (4.1%), including 10 device embolisations (1.7%), nine arrhythmias (1.5%), two significant vascular access-related complications (0.3%), one device erosion (0.2%), one malposed device (0.2%), and one probable wire perforation of the left atrial appendage (0.2%). There was one mortality related to device embolisation. All device embolisations occurred following the change in Instructions for Users after publication of the first device erosion report in 2004. This increase in embolisation rate was statistically significant (p-value 0.015).ConclusionsIn our series, the incidence of device embolisation was higher than that anticipated, with a significant increase following changes to the Instructions for Users. This highlights the need for ongoing data collection on complication incidence and for ongoing review of the impact of changes in clinical practice on complication rates.

Purpose: This study investigated how aberration-controlling, customised soft contact lenses corrected higher-order ocular aberrations and visual performance in keratoconic patients compared to other forms of refractive correction (spectacles and rigid gas-permeable lenses). Methods: Twenty-two patients (16 rigid gas-permeable contact lens wearers and six spectacle wearers) were fitted with standard toric soft lenses and customised lenses (designed to correct 3rd-order coma aberrations). In the rigid gas-permeable lens-wearing patients, ocular aberrations were measured without lenses, with the patient's habitual lenses and with the study lenses (Hartmann-Shack aberrometry). In the spectacle-wearing patients, ocular aberrations were measured both with and without the study lenses. LogMAR visual acuity (high-contrast and low-contrast) was evaluated with the patient wearing their habitual correction (of either spectacles or rigid gas-permeable contact lenses) and with the study lenses. Results: In the contact lens wearers, the habitual rigid gas-permeable lenses and customised lenses provided significant reductions in 3rd-order coma root-mean-square (RMS) error, 3rd-order RMS and higher-order RMS error (p ≤ 0.004). In the spectacle wearers, the standard toric lenses and customised lenses significantly reduced 3rd-order RMS and higher-order RMS errors (p ≤ 0.005). The spectacle wearers showed no significant differences in visual performance measured between their habitual spectacles and the study lenses. However, in the contact lens wearers, the habitual rigid gas-permeable lenses and standard toric lenses provided significantly better high-contrast acuities compared to the customised lenses (p ≤ 0.006). Conclusions: The customised lenses provided substantial reductions in ocular aberrations in these keratoconic patients; however, the poor visual performances achieved with these lenses are most likely to be due to small, on-eye lens decentrations. © 2014 The College of Optometrists.

Dr Clare O’Donnell provides an overview of contemporary solutions offered to patients by refractive surgery

Photodynamic collagen crosslinking (CXL) using ultraviolet-A (UVA) irradiation combined with the photosensitizer riboflavin has been introduced as a new treatment for progressive keratoconus. The results of clinical studies are promising, but the efficacy of the treatment in halting the progression depends on the stability of the induced biomechanical effects. The effects of corneal CXL on corneal rigidity; collagen fiber diameter; and resistance to heat degradation, enzymatic digestion, and swelling due to hydration are reviewed in this paper. The collective results indicate that CXL using UVA and riboflavin enhances the biomechanical properties of the corneal tissue, which remain stable over time. Therefore, this treatment could become the future standard therapy for keratoconus or used to halt the progression of keratoconus and postpone the need for corneal transplantation. The increase in availability and popularity of the CXL technique accentuates the requirement for reliable and accurate techniques for measuring corneal biomechanical properties before and after treatment.

Purpose: To assess the feasibility of applying scanning acoustic microscopy (SAM) on UV cross-linked corneal tissue for mapping and analyzing its biomechanical properties. Materials and Methods: Five corneal pairs (10 corneas) were used. In each pair, one cornea was cross-linked (epithelium removed, riboflavin application for 45 min and UVA irradiation for 30 min) and the contralateral control cornea was epithelial debrided and treated only with riboflavin for 45 min. Histological sections were prepared and their mechanical properties were examined using SAM. A line profile technique and 2D analysis was used to analyze the mechanical properties of the corneas. Then the corneal paraformaldehyde and unfixed sections were examined histologically using hematoxylin and eosin (H&E) staining. Results: In the frozen fresh corneal tissue, the speed of sound of the treated corneas was 1672.5 � 36.9 ms � 1 , while it was 1584.2 � 25.9 ms � 1 in the untreated corneas. In the paraformaldehyde fixed corneal tissue, the speed of sound of the treated corneas was 1863.0 � 12.7 ms � 1 , while it was 1739.5 � 30.4 ms � 1 in the untreated corneas. The images obtained from the SAM technique corresponded well with the histological images obtained with H&E staining. Conclusion: SAM is a novel tool for examining corneal tissue with a high spatial resolution, providing both histological and mechanical data.

Background: There is no specific quality of life (QoL) measurement tool to quantify QoL in patients with biliary tract cancer. Quality of life measurement is an increasingly crucial trial end point and is now being incorporated into clinical practice. Methods: This International Multicentre Phase IV Validation Study assessed the QLQ-BIL21 module in 172 patients with cholangiocarcinoma and 91 patients with cancer of the gallbladder. Patients completed the questionnaire at baseline pretherapy and subsequently at 2 months. Following this, the psychometric properties of reliability, validity, scale structure and responsiveness to change were analysed. Results: Analysis of the QLQ-BIL21 scales showed appropriate reliability with Cronbach's α-coefficients >0.70 for all scales overall. Intraclass correlations exceeded 0.80 for all scales. Convergent validity >0.40 was demonstrated for all items within scales, and discriminant validity was confirmed with values

Purpose: To compare monochromatic aberrations of keratoconic eyes when uncorrected, corrected with spherically-powered RGP (rigid gas-permeable) contact lenses and corrected using simulations of customised soft contact lenses for different magnitudes of rotation (up to 15°) and translation (up to 1mm) from their ideal position. Methods: The ocular aberrations of examples of mild, moderate and severe keratoconic eyes were measured when uncorrected and when wearing their habitual RGP lenses. Residual aberrations and point-spread functions of each eye were simulated using an ideal, customised soft contact lens (designed to neutralise higher-order aberrations, HOA) were calculated as a function of the angle of rotation of the lens from its ideal orientation, and its horizontal and vertical translation. Results: In agreement with the results of other authors, the RGP lenses markedly reduced both lower-order aberrations and HOA for all three patients. When compared with the RGP lens corrections, the customised lens simulations only provided optical improvements if their movements were constrained within limits which appear to be difficult to achieve with current technologies. Conclusions: At the present time, customised contact lens corrections appear likely to offer, at best, only minor optical improvements over RGP lenses for patients with keratoconus. If made in soft materials, however, these lenses may be preferred by patients in term of comfort.

Purpose: To assess visual performance and ocular aberrations in keratoconic patients using toric soft contact lenses (SCL), rigid-gas-permeable (RGP) contact lenses and spectacle lens correction. Methods: Twenty-two keratoconus patients (16 RGP lens wearers and six spectacle wearers) were fitted with toric SCL. Ocular aberrations were measured with and without the patient's habitual RGP lenses and with the SCL in place. In the spectacle wearers, aberrations were measured with and without the SCL. Visual performance (high- and low-contrast visual acuity) was evaluated with the patient's habitual correction and with the SCL. Results: In the RGP lens wearers both the habitual lenses and the toric SCL significantly reduced coma, trefoil, 3rd-order, 4th-order cylinder and higher-order root-mean-square (RMS) aberrations (p≤0.015). In the spectacle wearers the toric SCL significantly reduced coma, 3rd-order and higher-order RMS aberrations (p≤0.01). The patients' habitual RGP lenses gave better low-contrast acuity (p≤0.006) compared to the toric SCL; however, no significant difference was found between lens types for high-contrast acuity (p=0.10). In the spectacle wearers no significant differences in visual performance measurements were found between the patients' spectacles and the toric SCL (p≥0.06). Conclusion: The results show that RGP lenses provided superior visual performances and greater reduction of 3rd-order aberrations compared to toric SCL in this group of keratoconic patients. In the spectacle-wearing group, visual performance with the toric SCL was found to be comparable to that measured with spectacles. Nevertheless, with the exception of spherical aberration, the toric SCL were successful in significantly reducing uncorrected higher-order aberrations. Ophthalmic & Physiological Optics

PURPOSE: To explore the repeatability of lower-order and higher-order ocular aberrations measured in patients with keratoconus. METHODS: The IRX-3 (Imagine Eyes, Paris, France) aberrometer was used to record lower-order and higher-order aberrations in 31 eyes of 31 patients with keratoconus. Four monocular measurements were taken consecutively for each patient. The aberrometry data were analysed up to the 5th Zernike order for a 4-mm pupil diameter. The data were evaluated using repeated-measures anova and Friedman analyses. Repeatability was analysed using within-subject standard deviation (S(W) ) and the repeatability limit (r) calculated as 1.96 �?2 � S(w). RESULTS: Of the 11 aberration terms evaluated, the repeatability of Z (2,0) (mean = 1.36 ?m; S(W) = 0.09 ?m; r = 0.26 ?m); Z (2,�2) RMS (mean = 1.05 ?m; S(W) = 0.09 ?m; r = 0.24 ?m) and Z (4,0) aberrations (mean = 0.34 ?m; S(W) = 0.09 ?m; r = 0.24 ?m) showed the highest variability. In contrast, Z (3,�1) RMS aberrations (mean = 0.85 ?m; S(W) = 0.06 ?m; r = 0.16 ?m) and Z (4,�2) RMS aberrations (mean = 0.40??m; S(W) = 0.07 ?m; r = 0.18 ?m) showed comparatively better repeatability. CONCLUSIONS: The lower-order and higher-order aberrations measured in this group of keratoconic patients showed higher levels of variability compared to previous investigations of visually-normal subjects. These results may be of interest to eyecare practitioners involved in the design and fitting of aberration-controlling contact lenses for patients with keratoconus.

PURPOSE: To explore the possible association between myopia and corneal biomechanical properties in a Caucasian population, and the correlations between the properties of right and left eyes. METHODS: Corneal hysteresis (CH) and corneal resistance factor (CRF) were measured using the Ocular Response Analyser (ORA) in both eyes of 95 normal adult subjects aged between 19 and 48 years. The spherical equivalent refractive errors of the participants ranged from 0.25 to -14.00 D. The mean CH and CRF values for the right and left eyes were recorded for each subject. CH and CRF data were compared between different refractive groups. RESULTS: CH was found to be slightly lower in high myopes (>-6.00 D, mean CH 10.0 � 1.2 mmHg) in comparison to moderate myopes (>-3.00 to -6.00 D, 10.1 � 1.4 mmHg) and emmetropes and low myopes (+0.25 to -2.75 D, 10.9 � 1.5 mmHg). The decrease in CH with the degree of myopia was about 0.13 mmHg per D or roughly 1% per D (r(2) = 0.084, p < 0.001). Inter-subject variations were much greater than any systematic changes. CRF was not correlated with refractive error (r(2) = 0.001, p = 0.66). Although the refractive error was highly correlated between the two eyes (r(2) = 0.89, p < 0.001), CH and CRF showed a lower inter-ocular correlation (r(2) = 0.68, p < 0.001 and r(2) = 0.77, p < 0.001 respectively). CONCLUSIONS: These findings may indicate that the viscoelastic properties of the cornea are altered to a minor extent in myopia. However, in this normal population, any overall systematic changes in CH and CRF with refractive error were small in comparison with the considerable inter-subject scatter at any level of refraction.

Pellucid marginal corneal degeneration (PMD) is a rare ectatic disorder which typically affects the inferior peripheral cornea in a crescentic fashion. The condition is most commonly found in males and usually appears between the 2nd and 5th decades of life affecting all ethnicities. The prevalence and aetiology of this disorder remain unknown. Ocular signs and symptoms of patients with PMD differ depending on the severity of the condition. Unless corneal topography is evaluated, early forms of PMD may often be undetected however, in the later stages PMD can often be misdiagnosed as keratoconus. Visual signs and symptoms include longstanding reduced visual acuity or increasing against-the-rule irregular astigmatism leading to a slow reduction in visual acuity. In rare cases, patients may present with a sudden loss of vision and excruciating ocular pain due to corneal hydrops or spontaneous perforation. The vast majority of PMD patients are managed using spectacles and contact lenses. Several surgical procedures have been used in an attempt to improve visual acuity when spectacles and contact lenses do not provide adequate vision correction. Since patients with PMD make poor candidates for laser vision correction, an awareness of the topographical and slit-lamp features of PMD will be useful to clinicians screening for signs of corneal abnormality before corneal refractive surgery. This review describes the clinical features of PMD, its differential diagnosis and various management strategies presently available.

This case study reports on how refraction, visual acuity, and ocular higher-order aberrations changed after rigid gas-permeable (RGP) lens wear was suspended by a patient with early pellucid marginal corneal degeneration (PMD) and by a patient with moderate keratoconus. Alterations in central corneal power and axes, central corneal thickness, and corneal topography were also explored.Ocular aberrations and Scheimpflug photography were measured at 2 visits, 7 days apart, after the patients had removed their contact lenses. Subjective refraction and logarithm of the minimum angle of resolution visual acuities were also recorded at both visits.In contrast to the keratoconic patient, the patient with early PMD showed changes in subjective refraction (approximately -1.75 diopter cylinder of astigmatism), front surface central powers (∼1.3 diopter [D] horizontally and 2 D vertically), and anterior surface topography (∼1.2 D) between visits. Both patients showed an increase in total higher-order root mean square (HORMS) aberrations after suspending lens wear (∼0.40 μm for the moderately keratoconic patient and 0.22 μm for the early PMD patient).Changes in the optical and structural parameters of the cornea after suspending lens wear are likely to be dependent on a multitude of factors, such as the lens fitting and the biomechanical properties of the cornea. The findings of this report may be useful to practitioners when refracting or refitting existing RGP lens wearers with ectasia or to those involved in prescribing aberration-controlling contact lenses for conditions such as keratoconus and PMD.