End-stage heart failure in congenitally corrected transposition of the great arteries: a multicentre study.

BACKGROUND AND AIMS: For patients with congenitally corrected transposition of the great arteries (ccTGA), factors associated with progression to end-stage congestive heart failure (CHF) remain largely unclear. METHODS: This multicentre, retrospective cohort study included adults with ccTGA seen at a congenital heart disease centre. Clinical data from initial and most recent visits were obtained. The composite primary outcome was mechanical circulatory support, heart transplantation, or death. RESULTS: From 558 patients (48% female, age at first visit 36 ± 14.2 years, median follow-up 8.7 years), the event rate of the primary outcome was 15.4 per 1000 person-years (11 mechanical circulatory support implantations, 12 transplantations, and 52 deaths). Patients experiencing the primary outcome were older and more likely to have a history of atrial arrhythmia. The primary outcome was highest in those with both moderate/severe right ventricular (RV) dysfunction and tricuspid regurgitation (n = 110, 31 events) and uncommon in those with mild/less RV dysfunction and tricuspid regurgitation (n = 181, 13 events, P < .001). Outcomes were not different based on anatomic complexity and history of tricuspid valve surgery or of subpulmonic obstruction. New CHF admission or ventricular arrhythmia was associated with the primary outcome. Individuals who underwent childhood surgery had more adverse outcomes than age- and sex-matched controls. Multivariable Cox regression analysis identified older age, prior CHF admission, and severe RV dysfunction as independent predictors for the primary outcome. CONCLUSIONS: Patients with ccTGA have variable deterioration to end-stage heart failure or death over time, commonly between their fifth and sixth decades. Predictors include arrhythmic and CHF events and severe RV dysfunction but not anatomy or need for tricuspid valve surgery.

Identification and Subsequent Extraction of an Atrial Pacing Lead Inadvertently Positioned Within the Pulmonary Venous Atrium of a Patient With D-Transposition of the Great Arteries.

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.

Ten Years of Percutaneous Pulmonary Valve Implantation in Australia and New Zealand.

OBJECTIVE: 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. BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: Percutaneous pulmonary valve replacement is a relatively safe method of rehabilitating the right ventricular outflow tract.

Global multi-site, prospective analysis of cataract surgery outcomes following ICHOM standards: the European CAT-Community.

PURPOSE: To evaluate in a large sample of patients from 10 different European centers the results of cataract surgery, characterizing the relationship between patient-reported outcomes (PROMs) and clinician-reported outcome measures (CROMs). METHODS: Prospective non-interventional multicenter observational descriptive study analyzing the clinical outcomes of a total of 3799 cases undergoing cataract surgery (mean age: 72.7 years). In all cases, the cataract surgery standard developed by the International Consortium for Health Outcomes Measurements (ICHOM) was used to register the clinical data. Three-month postoperative visual acuity and refraction data were considered CROMs, whereas Rasch-calibrated item 2 (RCCQ2) and total Catquest-9SF score (CQ) were considered PROMs. RESULTS: Postoperative corrected distance visual acuity (CDVA) was 0.3 logMAR or better in 88.7% (2505/2823) of eyes. Mean differences between preoperative and postoperative RCCQ2 and CQ scores were -3.09 and -2.39, respectively. Visual function improvement with surgery was reported by 91.5% (2163/2364) of patients. Statistically significant, although weak, correlations of postoperative CDVA with postoperative refraction, PROMs, and complications were found (0.133 ≤ r ≤0.289, p < 0.001). A predictive model (R2: 0.254) of postoperative CDVA considering 10 variables was obtained, including preoperative CDVA, different ocular comorbidities, age, gender and intraoperative complications. Likewise, another predictive model (R2: 0.148) of postoperative CQ considering a total of 14 variables was obtained, including additionally preoperative CQ, target refraction and previous surgeries. CONCLUSIONS: Cataract surgery provides an improved functional vision in most of patients although this improvement can be limited by ocular comorbidities and complications. The relationship between PROMs and CROMs is multifactorial and complex.

Pregnancy Outcomes and Risk Evaluation in a Contemporary Adult Congenital Heart Disease Cohort.

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.

Performance of risk models predicting cardiac complications in pregnant women with congenital heart disease: a meta-analysis.

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.

Living With, and Caring for, Congenital Heart Disease in Australia: Insights From the Congenital Heart Alliance of Australia and New Zealand Online Survey.

BACKGROUND: There is a paucity of data describing the day-to-day experiences of adult Australians personally living with or caring for a child born with congenital heart disease (CHD). Such data would be of great practical importance to inform health care initiatives to improve outcomes. METHODS: 588 men (38.3 ± 11.9 years) and women (39.6 ± 12.6 years, 78% of respondent patients) living with CHD and 1,091 adult carers (93% mothers) of children with CHD (median age 7.3 [IQR 3.5-13.3 years], 54% male), representing all Australian states and territories, responded to a comprehensive online survey designed and hosted by the Congenital Heart Alliance of Australia and New Zealand. Data on demographic factors, the nature of underlying CHD, interactions with health care services, psychological wellbeing and wider impacts of CHD were collected. RESULTS: Most respondents were able to identify the type of CHD they (29% with a simple lesion such atrial septal defect, 17% tetralogy of Fallot) or their child had (21% with a simple lesion, 15% tetralogy of Fallot), whilst 90% cases of CHD had undergone cardiac surgery. Patients with CHD were mostly employed (70%) or studying (8.8%), whilst 9.1% were receiving disability benefits. In terms of transition care, 52% of adult patients had been referred by a paediatric to adult cardiologist with 84% still actively managed by a specialist. Overall, 31% of patients with CHD sought emergency care and required >10 days sick leave in the past 12 months. Moreover, 71% and 55% of patients, respectively, reported recent feelings of anxiety/worry or depressive thoughts related to their CHD (61% sought professional assistance). Consistent with high levels of disruption to daily living, 59% of carer respondents (24%>10 days) had taken carer's leave in the past 12 months. CONCLUSIONS: These contemporary, self-reported, Australian data reveal the burden of living and caring for CHD from an adult's perspective. Survey respondents highlighted the potential disconnect between paediatric and adult CHD services and suggest an important, unmet need for dedicated health services/community care to cost-effectively manage high levels of health care utilisation coupled with associated psychological distress.

Management of People With a Fontan Circulation: a Cardiac Society of Australia and New Zealand Position statement.

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.

Adult Congenital Heart Disease in Australia and New Zealand: A Call for Optimal Care.

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.

Transcatheter closure of secundum atrial septal defects: has fear of device erosion altered outcomes?

BACKGROUND: Transcatheter 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. METHODS: We 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. RESULTS: There 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). CONCLUSIONS: In 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.

Infective endocarditis following Melody valve implantation: comparison with a surgical cohort.

BACKGROUND: Infective 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. METHODS: We 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. RESULTS: In 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. CONCLUSIONS: The 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.

Optical quality and visual performance with customised soft contact lenses for keratoconus.

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.

Visual performance and optical quality with soft lenses in keratoconus patients.

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.

Optical quality for keratoconic eyes with conventional RGP lens and simulated, customised contact lens corrections: a comparison.

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.

Long-Term Outcomes After Atrial Switch Operation for Transposition of the Great Arteries.

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.

Another case of multiple juxtasutural hyperostoses, cervical exostoses, and fatty infiltration of myocardium.

We report on an individual affected by multiple juxtasutural cranial hyperostoses who later developed cervical spine hyperostoses with associated fusion anomalies. The propositus was not affected by a disorder of generalized overgrowth. An echocardiogram revealed areas of abnormal signal within the myocardium which were consistent with fatty infiltration on magnetic resonance imaging of the heart. We compare our case with a few previously reported cases that share phenotypic similarities but differ in some other clinical aspects. It is possible that these patients represent a phenotypic continuum resulting from a somatic mutation in an unknown gene.

Repeatability of ocular aberration measurements in patients with keratoconus.

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 µm; S(W) = 0.09 µm; r = 0.26 µm); Z (2,±2) RMS (mean = 1.05 µm; S(W) = 0.09 µm; r = 0.24 µm) and Z (4,0) aberrations (mean = 0.34 µm; S(W) = 0.09 µm; r = 0.24 µm) showed the highest variability. In contrast, Z (3,±1) RMS aberrations (mean = 0.85 µm; S(W) = 0.06 µm; r = 0.16 µm) and Z (4,±2) RMS aberrations (mean = 0.40 µm; S(W) = 0.07 µm; r = 0.18 µm) 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.

Corneal biomechanical properties measured with the Ocular Response Analyser in a myopic population.

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.

Corneal changes after suspending contact lens wear in early pellucid marginal corneal degeneration and moderate keratoconus.

OBJECTIVES: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

Optimising echocardiographic screening for rheumatic heart disease in New Zealand: not all valve disease is rheumatic.

AIMS: Echocardiography detects a greater prevalence of rheumatic heart disease than heart auscultation. Echocardiographic screening for rheumatic heart disease combined with secondary prophylaxis may potentially prevent severe rheumatic heart disease in high-risk populations. We aimed to determine the prevalence of rheumatic heart disease in children from an urban New Zealand population at high risk for acute rheumatic fever. METHODS AND RESULTS: To optimise accurate diagnosis of rheumatic heart disease, we utilised a two-step model. Portable echocardiography was conducted on 1142 predominantly Maori and Pacific children aged 10-13 years. Children with an abnormal screening echocardiogram underwent clinical assessment by a paediatric cardiologist together with hospital-based echocardiography. Rheumatic heart disease was then classified as definite, probable, or possible. Portable echocardiography identified changes suggestive of rheumatic heart disease in 95 (8.3%) of 1142 children, which reduced to 59 (5.2%) after cardiology assessment. The prevalence of definite and probable rheumatic heart disease was 26.0 of 1000, with 95% confidence intervals ranging from 12.6 to 39.4. Portable echocardiography overdiagnosed rheumatic heart disease with physiological valve regurgitation diagnosed in 28 children. A total of 30 children (2.6%) had non-rheumatic cardiac abnormalities, 11 of whom had minor congenital mitral valve anomalies. CONCLUSIONS: We found high rates of undetected rheumatic heart disease in this high-risk population. Rheumatic heart disease screening has resource implications with cardiology evaluation required for accurate diagnosis. Echocardiographic screening for rheumatic heart disease may overdiagnose rheumatic heart disease unless congenital mitral valve anomalies and physiological regurgitation are excluded.