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Competing Interests: Competing interests: None declared.

Background: An improvement in life expectancy in patients suffering from adult congenital heart disease (ACHD) has corresponded with a rise in heart failure incidence within this group. An area that has not been addressed in ACHD heart failure guidelines has been the use of combined inhibition of angiotensin receptor-neprolysin pathways. This case series sought to demonstrate tolerability and 6-month outcome measures of sacubitril/valsartan use in ACHD patients with a severely impaired systemic ventricle., Methods: A prospective cohort analysis of ACHD patients between December 2016 and September 2017 with severe systemic ventricular systolic dysfunction, New York Heart Association (NYHA) class II-III symptoms and eligible for commencement on a sacubitril/valsartan regime was undertaken., Results: Five (5) consecutive patients were included in this cohort review, 80% male, mean age 41.8 (±19) years and mean systemic ventricular ejection fraction 27% (±3.6%). Two (2) patients with pre-existing D-TGA and atrial baffle repair, one patient with Tetralogy of Fallot repair and pulmonary valve replacement (PVR), one patient with left atrial isomerism and partial atrioventricular (AV) canal defect repair and mitral valve replacement (MVR) and the last patient had biventricular repair for pulmonary atresia with MVR and PVR. Forty per cent (40%) of patients had a systemic right ventricle. All patients had NYHA functional class of ≥II, were on optimal tolerated doses ACE-I or ARB prior to sacubitril/valsartan combination. Six (6) months post commencement of sacubitril/valsartan patients experienced a mean improvement of one functional class., Conclusions: Our experience suggests that sacubitril/valsartan therapy is well tolerated in ACHD heart failure patients and is associated with improvement in functional class., (Crown Copyright © 2018. Published by Elsevier B.V. All rights reserved.)

Background: Transcatheter pulmonary valve implantation (TPVI) with the Melody® transcatheter pulmonary valve (TPV) has demonstrated good haemodynamic and clinical outcomes in the treatment of right ventricular outflow tract (RVOT) conduit dysfunction in patients with repaired congenital heart disease CHD. We present the first Australian single centre experience of patients treated with Melody TPV., Method: A prospective, observational registry was developed to monitor clinical and haemodynamic outcomes in patients with RVOT dysfunction treated with the Melody TPV (Medtronic Inc, Minneapolis, United States)., Results: Seventeen patients underwent TPVI with Melody TPV at The Prince Charles Hospital between January 2009 and February 2016 with a median (range) age of 34 (R: 15-60). Fifteen (88%) were NYHA Class 2 dyspnoea and 11 (59%) had corrected Tetralogy of Fallot. Indication for TPVI was stenosis in eight (47%), regurgitation in two (12%) and mixed dysfunction in seven (41%). Device implantation was successful in all patients. Peak RVOT gradient was significantly reduced and there was no significant regurgitation post procedure. There was one (6%) major procedural adverse event and two (12%) major adverse events at last recorded follow-up. There were no patient deaths. Follow-up cardiac magnetic resonance imaging revealed a significant reduction in indexed right ventricular end diastolic volume., Conclusion: This study confirms the safety and effectiveness of TPVI with Melody TPV for RVOT dysfunction in repaired CHD., (Crown Copyright © 2017. Published by Elsevier B.V. All rights reserved.)

Background: 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 congenital heart disease (CHD) worldwide. Among ISACHD's objectives is to "promote a holistic team-based approach to the care of the adult with CHD that is comprehensive, patient-centered, and interdisciplinary" (http://www.isachd.org). This emphasis on team-based care reflects the fact that adults with CHD constitute a heterogeneous population with a wide spectrum of disease complexity, frequent association with other organ involvement, and varied co-morbidities and psychosocial issues., Methods: Recognizing the vital role of the adult CHD (ACHD) nurse coordinator (ACHD-NC) in optimizing team-based care, ISACHD established a task force to elucidate and provide guidance on the roles and responsibilities of the ACHD-NC. Acknowledging that nursing roles can vary widely from region to region based on factors such as credentials, scopes of practice, regulations, and local culture and tradition, an international panel was assembled with experts from North America, Europe, East Asia, and Oceania. The writing committee was tasked with reviewing key aspects of the ACHD-NC's role in team-based ACHD care., Results/conclusion: The resulting ISACHD position statement addresses the ACHD-NC's role and skills required in organizing, coordinating, and facilitating the care of adults with CHD, holistic assessment of the ACHD patient, patient education and counseling, and support for self-care management and self-advocacy., (Crown Copyright © 2016. Published by Elsevier B.V. All rights reserved.)

Aims: To measure the interdose milk to plasma ratio (M/P) of R- and S-methadone during multiple dosing in lactating mothers taking medium to high doses of methadone (> 40 mg daily), and to assess likely infant exposure., Methods: Eight mother/child pairs were studied, initially during their postpartum hospital stay (immature milk), and where possible again after 15 days (mature milk). The women were on a methadone maintenance programme with daily doses of >or=40 mg day-1. Venous blood was collected at 0, 1, 2, 4, 6, 8, 12, and 24 h and milk was collected from both breasts at 0-4, 4-8, 8-12, 12-16, 16-20, and 20-24 h after dose. R- and S-methadone were quantified by h.p.l.c. The areas under the plasma and milk concentration-time curves (AUC) were estimated and M/P(AUC) was calculated. The relative infant dose of both enantiomers was estimated as the product of drug concentration in milk and an average daily milk intake of 0.15 l kg(-1)., Results: For immature milk (n = 8) the M/P(AUC) for R-methadone was 0.68 (95% CI 0.48, 0.89) and for S-methadone 0.38 (0.26, 0.50). For mature milk (n = 2) the M/P(AUCs) for R-methadone were 0.39 and 0.54 and for S-methadone 0.24 and 0.30, respectively. The estimated doses of R- and S-methadone that would be received by the infant via immature milk were 3.5% (2.05, 5.03%) and 2.1% (1.3, 2.8%), respectively, of the maternal dose (assuming 50% of each enantiomer in the dose). The relative infant dose for R- plus S-methadone together was 2.8% (1.7, 3.9%)., Conclusions: Breastfeeding during medium to high dose methadone appears to be 'safe' according to conventional criteria because the dosage is < 10%. However because the absolute dose received by the infant is dependent on the maternal dose rate, the risk-benefit ratio should be considered for each individual case. The doses of methadone received via milk are unlikely to be sufficient to prevent the neonatal abstinence syndrome.

Aim: To audit the identification and screening of graduates from a neonatal intensive care unit with risk factors for sensorineural hearing loss., Methods: Hospital medical records of newborn infants discharged from the neonatal intensive care unit, Christchurch Womens Hospital, between 1 July 1994 and 30 June 1995 (n=564), were examined to identify those at risk for sensorineural hearing loss according to the American Speech-Language Hearing Association risk criteria 1991. Auditory brainstem response test results were obtained from the Christchurch Hospital Audiology Department. Outcome measures were: presence of hearing loss risk factors, numbers tested with auditory brainstem response, age at test and presence and degree of hearing impairment., Results: Of 5,215 live births in Christchurch, 564 infants were discharged through the neonatal intensive care unit. Of these, 86 had risk factors for sensorineural hearing loss. There were 72 (84%) infants tested at audiology, with fifteen (17%) having abnormal test results. There were fourteen with risk factors who did not get audiology screening., Conclusion: A high proportion (84%) of high risk newborn infants had auditory brainstem response testing. Further improvement would require strict implementation of standard procedures. Auditory brainstem response screening is part of a wider population surveillance approach to identify hearing loss as early as possible.

The effect of antenatal transport of women between level 3 units in New Zealand on maternal and infant well-being was examined in a retrospective case-controlled study. The outcomes of women transferred to other level 3 centres because of lack of neonatal unit space were compared with women who were able to be delivered at Christchurch Women's Hospital (CWH). Of 34 women transferred out of CWH from 1991-194, 20 (59%) were delivered at the receiving centre, the remained returning undelivered. Women transferred from Christchurch were more likely to be of lower parity (p < 0.05), and were less likely to be in premature labour than those who remained in Christchurch (p < 0.05). There was a trend for the initial risk of mortality as assessed by the Clinical Risk Index for Babies (CRIB) score to be lower for those infants born out of Christchurch but this was not statistically significant. Mortality, oxygen requirement at 36 week, periventricular haemorrhage, retinopathy of prematurity, and the time to regain birth-weight were not statistically different between the 2 groups, despite significantly more outborn children being male (p < 0.05). Antenatal transfer therefore seems to be a safe procedure, although this study did not consider the potentially serious side effect of social factors on transferred families.

Objective: To examine the relationship between plasma and erythrocyte selenium and glutathione peroxidase (GPx) levels in premature infants and outcome measures., Design: Prospective observational longitudinal study., Setting: Two regional neonatal intensive care units in the South Island of New Zealand, an area with low soil selenium., Patients: Seventy-nine infants with birth weights less than 1500 g or gestation less than 32 weeks admitted within 48 hours of birth from November 1992 through November 1993., Main Outcome Measures: Oxygen requirement at 28 days (chronic lung disease), or 36 weeks postmenstrual age and for all or most of the time from birth (bronchopulmonary dysplasia), total days in oxygen, retinopathy of prematurity, periventricular hemorrhage, or ventricular dilatation., Results: Initial infant plasma selenium and GPx levels were about two thirds of maternal levels and fell a further 30% in 28 days. In contrast to adults, there was a poor correlation in infant plasma between selenium and GPx at birth and 28 days. Plasma selenium at 28 days was significantly lower in infants with chronic lung disease and bronchopulmonary dysplasia. After controlling for gestational age and age when fully fed orally, 28-day plasma selenium was significantly associated with the log of total days of oxygen requirement, each drop of 0.1 mumol/L in 28-day selenium being associated with a 58% increase in days of oxygen dependency. No significant associations of other parameters of selenium status and respiratory outcome were found, and there were no significant associations of any parameters of selenium status with other outcome measures., Conclusions: This study demonstrates for the first time in human infants that low plasma selenium levels are significantly associated with an increased respiratory morbidity. Whether selenium deficiency is etiologically important in determining the respiratory outcome or the result of sickness in the infant should be investigated in a randomized, controlled trial.

The aim of this study was to assess the influence of maternal methadone dosage on the severity of neonatal withdrawal. The charts of 67 drug-abusing mothers and their 70 infants were examined to determine documented patterns of drug usage and the severity of neonatal withdrawal. Of these, 40 women were on a methadone programme. There was a strong relationship between maternal methadone dose at delivery and severity of neonatal withdrawal as assessed by the Neonatal Abstinence Score, length of stay and duration of treatment. Children whose mothers received methadone had mean peak symptom scores greater than 10 whereas the group receiving no methadone had mean scores of less than 4 (p < 0.001). These effects tended to increase with increasing doses of methadone. Length of stay and duration of neonatal treatment showed highly statistically significant increases (p < 0.001) with increasing methadone dose. Maternal methadone dose appears to be strongly related to the severity of neonatal withdrawal.

Objective: Intraventricular hemorrhage in neonates with normally sized ventricles is overlooked when sonograms obtained via the anterior fontanelle fail to show a small amount of blood in the occipital horns of the lateral ventricle. Because visualization of the occipital horns is improved when sonograms are obtained via the posterior fontanelle, we studied the efficacy of posterior fontanelle sonography in establishing the diagnosis of intraventricular hemorrhage for 259 neonates., Materials and Methods: We compared cranial sonograms obtained via both the anterior and the posterior fontanelles for 34 infants who had intraventricular hemorrhage and whose mean age at birth was 28 weeks (range, 23-40 weeks) with sonograms for 225 neonates who did not have hemorrhage and whose mean age at birth was 31 weeks (range, 24-42 weeks). Sonograms were assessed for satisfactory visualization of the occipital horns of the lateral ventricles, for the characteristics of intraventricular hemorrhage, if present, and for ventricular size, assessed as normal or showing mild, moderate, or marked dilatation. The technique of posterior fontanelle sonography involves obtaining parasagittal views of the occipital horns of the lateral ventricles and coronal scans of the occipital horns at the level of the calcarine fissure. The data from the contrasting groups of neonates were analyzed with Student's t-test or 2 x 2 tables as appropriate. Five autopsies were done; two confirmed intraventricular hemorrhage, and three confirmed the absence of intraventricular hemorrhage., Results: Sonograms obtained via the posterior fontanelle were satisfactory for 92% of neonates born at less than 32 weeks' gestation and 88% of all neonates scanned. The mean age at birth of those with satisfactory sonograms obtained via the posterior fontanelle was 29 weeks (SD, 4 weeks); in comparison, a mean age at birth of 35 weeks (SD, 5 weeks) was associated with unsatisfactory sonograms (p < .0001). Intraventricular hemorrhage was detected via the posterior fontanelle but not via the anterior fontanelle on the initial diagnostic scan for 14 neonates. The ventricles were more likely to be normally sized when intraventricular hemorrhage was seen via the posterior fontanelle only (86%) than when intraventricular hemorrhage was seen via the anterior fontanelle as well (50%) (Fisher's exact test; p = .04)., Conclusion: Our results show that sonograms obtained via the posterior fontanelle increase the rate of detection of intraventricular hemorrhage in neonates with normally sized ventricles.

At Makthlawaiya, in the Paraguayan Chaco, the prevalence of Trypanosoma (Schizotrypanum) cruzi infection among both domestic Triatoma infestans and domestic dogs was 38%, and IgG anti-T. cruzi antibody was detected by the quantitative enzyme-linked immunosorbent assay (ELISA) in 80% (105/133) of human sera. Ninety percent (25/28) of T. cruzi strains isolated from both T. infestans and dogs showed heterozygous isoenzyme profiles for glucose phosphate isomerase, phosphoglucomutase and 6-phosphogluconate dehydrogenase. These strains appeared to be closely related to Bolivian zymodeme 2. Three Paraguayan T. cruzi strains showed homozygous isoenzyme profiles, similar to those of major Brazilian zymodemes. It was concluded that T. cruzi strains with heterozygous isoenzyme profiles predominate in domestic transmission cycles in this highly endemic area of the Paraguayan Chaco.