Sacubitril/Valsartan in Adult Congenital Heart Disease Patients With Chronic Heart Failure - A Single Centre Case Series and Call for an International Registry.

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.

Adult congenital heart disease nurse coordination: Essential skills and role in optimizing team-based care a position statement from the International Society for Adult Congenital Heart Disease (ISACHD).

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.

Outcomes Following Melody Transcatheter Pulmonary Valve Implantation for Right Ventricular Outflow Tract Dysfunction in Repaired Congenital Heart Disease: First Reported Australian Single Centre Experience.

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).

A retrospective study of judicial encounters of young adults with congenital heart disease.

Young people with congenital heart disease are often marginalised and may compensate for restricted physical and social abilities by substance abuse or risk-taking behaviour. We report on the judicial encounters of patients in an Adult Congenital Heart unit. Fifteen patients had court appearances and all were male.

Intrathoracic gastric volvulus mimicking pyloric stenosis.

A 5-week-old-infant presented to hospital following the acute onset of non-bilious vomiting with clinical and acid-base features suggestive of pyloric stenosis. A chest radiograph obtained because of intercurrent infection unexpectedly revealed a left-sided congenital diaphragmatic hernia. A barium meal demonstrated the presence of an intrathoracic gastric volvulus, requiring urgent surgical management.

Distribution of R- and S-methadone into human milk during multiple, medium to high oral dosing.

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.

Audiological screening of neonatal intensive care unit graduates at high risk of sensorineural hearing loss.

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.

Neonatal abstinence syndrome following abrupt cessation of breastfeeding.

Neonatal abstinence syndrome is seen commonly in infants born to women on methadone maintenance. Many of these infants are breast-fed but few data are available on the distribution of methadone in breast milk, particularly for women receiving doses greater than 60 mg daily. We report two infants who appeared to develop neonatal abstinence syndrome, after abrupt discontinuation of breast-feeding by women receiving 70 mg and 130 mg of methadone.

How safe is antenatal transfer between level 3 units?

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.

The relationship of selenium status to respiratory outcome in the very low birth weight infant.

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.

Maternal methadone dosage and neonatal withdrawal.

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.

Ulnar club-hand and constriction-ring syndrome.

An infant presented with ulnar club hand and constriction ring syndrome involving all four limbs. The former abnormality was diagnosed by ultrasound at 23 weeks gestation. This association has not been previously described.

Diagnosis of intraventricular hemorrhage in the newborn: value of sonography via the posterior fontanelle.

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).

Prenatal diagnosis of unilateral hydrocephalus.

We report a case of unilateral hydrocephalus diagnosed at 20 weeks' gestation, at which time marked facial and cranial asymmetry was present already. Brain mantle reconstitution was incomplete following ventriculo-peritoneal shunting, and the child has significant neurodevelopmental disability.

Prenatal diagnosis of colon atresia.

We report a case of colon atresia diagnosed pre-natally at 32 weeks' gestation. The fetal transverse colon was dilated. Complete atresia of the middle transverse colon was confirmed after birth.