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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.

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.

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.

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.

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.

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.

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 explore the biomechanical changes induced by repeated cross-linking using scanning acoustic microscopy (SAM). METHODS: Thirty human corneas were divided into three groups. In group A, five corneas were cross-linked once. In group B, five corneas were cross-linked twice, 24 hours apart. In group C, five corneas were cross-linked three times, 24 hours apart. The contralateral controls in all groups had similar treatment but without UV-A. The speed of sound, which is directly proportional to the square root of the tissue's elastic modulus, was assessed using SAM. RESULTS: In group A, the speed of sound of the treated corneas was 1677.38 � 10.70 ms(-1) anteriorly and 1603.90 � 9.82 ms(-1) posteriorly, while it was 1595.23 � 9.66 ms(-1) anteriorly and 1577.13 � 8.16 ms(-1) posteriorly in the controls. In group B, the speed of sound of the treated corneas was 1746.33 � 23.37 ms(-1) anteriorly and 1631.60 � 18.92 ms(-1) posteriorly, while it was 1637.57 � 22.15 ms(-1) anteriorly and 1612.30 � 22.23 ms(-1) posteriorly in the controls. In group C, the speed of sound of the treated corneas was 1717.97 � 18.92 ms(-1) anteriorly and 1616.62 � 17.58 ms(-1) posteriorly, while it was 1628.69 � 9.37 ms(-1) anteriorly and 1597.68 � 11.97 ms(-1) posteriorly in the controls. The speed of sound in the anterior (200 � 200 ?m) region between the cross-linked and control corneas in groups A, B, and C was increased by a factor of 1.051 (P = 0.005), 1.066 (P = 0.010), and 1.055 (P = 0.005) respectively. However, there was no significant difference among the cross-linked corneas in all groups (P = 0.067). CONCLUSIONS: A significant increase in speed of sound was found in all treated groups compared with the control group; however, the difference among the treated groups is not significant, suggesting no further cross-links are induced when collagen cross-linking treatment is repeated.

OBJECTIVES: Transannular patching is used to relieve significant pulmonary annular stenosis during tetralogy of Fallot repair. Although recent literature has focused on the deleterious effects of pulmonary regurgitation, inadequate relief of stenosis may increase postoperative mortality and the re-intervention rate. Patching criteria based on pulmonary annular z-scores are ambiguous because of the use of varied z-score data sets. This study aimed to generate data that could be used to optimize z-score use for patch insertion. METHODS: A retrospective review was made of medical records of patients who had a valve-sparing repair of tetralogy of Fallot between 1 January 2000 and 31 December 2010. In a selected group in which the residual gradient was confined to the pulmonary valve, the post-repair peak pressure gradient was determined by trans-thoracic echocardiography and was correlated with the intraoperative pulmonary valve annulus (PVA) diameter z-score. Regression analysis was used to examine this relationship. RESULTS: Data from 46 patients with valve-sparing repair were reviewed; the median age and median weight were 6.5 months and 6.6 kg, respectively; the 30-day mortality was 2.2%. Analysis of these data implies that 25% of the time, all patients with a PVA z-score of −1.3 would have a PVA gradient ≥30 mmHg. CONCLUSIONS: Criteria that recommend a transannular patch insertion only when the PVA diameter z-score is significantly smaller than −1.3 (e.g. −3) may result in a significant number of patients with an unacceptable post-repair PVA gradient.

This article corrects the following:Optical quality for keratoconic eyes with conventional RGP lens and simulated, customised contact lens corrections: a comparisonAmit Jinabhai, W. Neil Charman, Clare O’Donnell, Hema RadhakrishnanVolume 32, Issue 3, Ophthalmic and Physiological Optics, pages: 200-212, First Published online: April 18, 2012

The literature suggests that diabetic patients may have altered tear chemistry and tear secretion as well as structural and functional changes to the corneal epithelium, endothelium and nerves. These factors, together with a reported increased incidence of corneal infection, suggest that diabetic patients may be particularly susceptible to developing ocular complications during contact lens wear. Reports of contact lens-induced complications in diabetic patients do exist, although a number of these reports concern patients with advanced diabetic eye disease using lenses on an extended wear basis. Over the past decade or so, there have been published studies documenting the response of the diabetic eye to more modern contact lens modalities. The results of these studies suggest that contact lenses can be a viable mode of refractive correction for diabetic patients. Furthermore, new research suggests that the measurement of tear glucose concentration could, in future, be used to monitor metabolic control non-invasively in diabetic patients. This could be carried out using contact lenses manufactured from hydrogel polymers embedded with glucose-sensing agents or nanoscale digital electronic technology. The purpose of this paper is to review the literature on the anterior ocular manifestations of diabetes, particularly that pertaining to contact lens wear.

PURPOSE: ? The aim was to study the link between refractive error and corneal biomechanical properties. METHODS: ? Corneal hysteresis and corneal resistance factor were measured using the Ocular Response Analyser in 117 participants. The spherical equivalent refractive error of the participants ranged between -9.00 and +3.00?D. RESULTS: ? Corneal hysteresis and corneal resistance factor showed a considerable degree of variability between individuals. Corneal hysteresis was not found to correlate significantly with refractive error (p = 0.82). Corneal resistance factor showed a weak but significant correlation with spherical equivalent refractive error (r(2) = 0.04; p = 0.03), with myopic participants exhibiting a higher corneal resistance factor compared with non-myopes. CONCLUSIONS: ? Refractive error accounted for four per cent of the variance in corneal resistance factor measurements, indicating that patients with mild to moderate myopia have higher corneal resistance compared with non-myopes.

Purpose: This work investigates how short-term changes in blood glucose concentration affect the refractive components of the diabetic eye in patients with long-term Type 1 and Type 2 diabetes.\ud \ud Methods: Blood glucose concentration, refractive error components (mean spherical equivalent MSE, J0, J45), central corneal thickness (CCT), anterior chamber depth (ACD), crystalline lens thickness (LT), axial length (AL) and ocular aberrations were monitored at two-hourly intervals over a 12-hour period in: 20 T1DM patients (mean age ± SD) 38±14 years, baseline HbA1c 8.6±1.9%; 21 T2DM patients (mean age ± SD) 56±11 years, HbA1c 7.5±1.8%; and in 20 control subjects (mean age ± SD) 49±23 years, HbA1c 5.5±0.5%. The refractive and biometric results were compared with the corresponding changes in blood glucose concentration.\ud \ud Results: Blood glucose concentration at different times was found to vary significantly within (p0.05). Minor changes of marginal statistical or optical significance were observed in some biometric parameters. Similarly there were some marginally significant differences between the baseline biometric parameters of well-controlled and poorly-controlled diabetic subjects.\ud \ud Conclusion: This work suggests that normal, short-term fluctuations (of up to about 6 mM/l on a timescale of a few hours) in the blood glucose levels of diabetics are not usually associated with acute changes in refractive error or ocular wavefront aberrations. It is therefore possible that factors other than refractive error fluctuations are sometimes responsible for the transient visual problems often reported by diabetic patients.

Background: The aim was to investigate the fluctuations in monochromatic ocular aberrations with accommodation and tear-film changes in moderate keratoconic eyes. Methods: We measured the changes in ocular higher-order aberrations in 10 moderate keratoconic and 10 visually normal eyes to accommodative stimuli ranging from zero to 5.00 DS using a Hartmann-Shack aberrometer. In addition, the changes in ocular higher-order aberrations were measured for up to 15 seconds after a blink in eight keratoconic and eight visually normal eyes. Results: These results show that ocular spherical (p = 0.68) and coma-like (p = 0.71) aberrations did not change significantly with accommodation from zero to 5.00 DS in keratoconic eyes. In contrast to normal eyes, the ocular higher-order RMS error tended to decrease in magnitude after a blink in keratoconic eyes. Vertical coma became less negative with time after a blink in the keratoconic group, therefore, reducing the manifest ocular higher order RMS error by counteracting the negative vertical coma of the cornea. Conclusions: Compared to the manifest monochromatic higher-order aberrations, any dynamic fluctuations in ocular aberrations with accommodation and tear film changes are relatively small in moderate keratoconic eyes. This implies that the correction of monochromatic higher-order aberrations in keratoconus using customised soft contact lenses will not be significantly hindered by such dynamic aberrational changes.

PURPOSE. To assess and compare changes in the biomechanical properties of the cornea following different corneal collagen cross-linking protocols using scanning acoustic microscopy (SAM). METHODS. Ten donor human corneal pairs were divided into two groups consisting of five corneal pairs in each group. In group A, five corneas were treated with low-fluence (370 nm, 3 mW/cm(2)) cross-linking (CXL) for 30 minutes. In group B, five corneas were treated with high-fluence (370 nm, 9 mW/cm2) CXL for 10 minutes. The contralateral control corneas in both groups had similar treatment but without ultraviolet A. The biomechanical properties of all corneas were tested using SAM. RESULTS. In group A, the mean speed of sound in the treated corneas was 1677.38 +/- 10.70 ms(-1) anteriorly and 1603.90 +/- 9.82 ms(-1) posteriorly, while it was 1595.23 +/- 9.66 ms(-1) anteriorly and 1577.13 +/- 8.16 ms(-1) posteriorly in the control corneas. In group B, the mean speed of sound of the treated corneas was 1665.06 +/- 9.54 ms(-1) anteriorly and 1589.89 +/- 9.73 ms(-1) posteriorly, while it was 1583.55 +/- 8.22 ms(-1) anteriorly and 1565.46 +/- 8.13 ms(-1) posteriorly in the untreated control corneas. The increase in stiffness between the crosslinked and control corneas in both groups was by a factor of 1.051X. CONCLUSIONS. SAM successfully detected changes in the corneal stiffness after application of collagen cross-linking. A higher speed-of-sound value was found in the treated corneas when compared with the controls. No significant difference was found in corneal stiffness between the corneas cross-linked with low-and high-intensity protocols.

This study investigates whether systematic differences exist between the accommodation response/stimulus curves of emmetropes and myopes when the targets are sinusoidal gratings, in particular whether myopic accommodation is relatively less effective when presented with targets of high spatial frequency due to increased tolerance to defocus blur. Ten emmetropes (overall mean sphere +0.19 D, range -0.37 to +1.37 D) and 10 myopes (overall mean sphere -2.89 D, range -1.13 to -6.63 D) viewed Gabor targets with dominant frequencies 1, 4, 8 and 16 c/deg. Maximal grating contrast was 80% and the full, green, stimulus field was 6 deg. Subjects were aged between 18 and 37 years. A further high-contrast 6/30 optotype target was included for comparison purposes. Viewing was monocular, the other eye being occluded. Stimulus demand was varied with trial lenses over the nominal range 0 to 6.0 D and the corresponding accommodation responses were recorded with an open-view, Shin-Nippon SRW-5000 auto-refractor. The resulting accommodation response/stimulus curves were characterized by their slopes over the stimulus range 1.5 to 6.0 D and by an "error index" indicating the extent to which the responses differed from the ideal 1:1 response/stimulus line. No significant differences were found between the mean accommodative behavior of the two refractive groups for any target. There were, however, substantial inter-subject differences. Some subjects in both groups showed more accurate responses with the higher spatial-frequency targets, while others showed optimal response at intermediate frequencies. Although it has been reported in the literature that, in comparison to emmetropes, myopes have reduced sensitivity to blur and response/stimulus curves of lower slope, the present study failed to demonstrate any reduction in their responses to gratings of relatively high spatial frequency. For each target the two refractive groups showed similar accommodative behavior.

Corneal confocal microscopy is a reiterative, rapid, noninvasive in vivo clinical examination technique capable of imaging corneal nerve fibers. Nerve fiber tortuosity may indicate a degenerative and attempted regenerative response of nerve fibers to diabetes. The purpose of this study was to define alterations in the tortuosity of corneal nerve fibers in relation to age, duration of diabetes, glycemic control, and neuropathic severity.The cornea and collected images of the subbasal nerve plexus of 18 diabetic patients (stratified into mild, moderate, and severe neuropathic groups using conventional clinical measures of neuropathy) and 18 age-matched nondiabetic control subjects were scanned, and a novel mathematical paradigm was applied to quantify the extent of nerve tortuosity, which was termed the tortuosity coefficient (TC).TC was significantly different between the four clinical groups (F(3) = 12.2, P0.001). It was significantly greater in the severe neuropathic group than in control subjects (P0.003) and in the mild (P0.004) and moderate (P0.01) neuropathic groups. TC did not correlate significantly with the age (r = -0.003, P0.05), duration of diabetes (r = -0.219, P0.05), or hemoglobin A1c (HbA1c; r = 0.155, P0.05) of diabetic patients.Corneal confocal microscopy allows rapid, noninvasive in vivo evaluation of corneal nerve tortuosity. This morphologic abnormality relates to the severity of somatic neuropathy and may reflect an alteration in the degree of degeneration and regeneration in diabetes.