Neonates undergoing heart surgery for CHD are at risk for postoperative gastrointestinal complications and aspiration events. There are limited data regarding the prevalence of aspiration after neonatal cardiothoracic surgery; thus, the effects of aspiration events on this patient population are not well understood. This retrospective chart review examined the prevalence and effects of aspiration among neonates who had undergone cardiac surgery at the time of their discharge.

This study examined the prevalence of aspiration among neonates who had undergone cardiac surgery. Demographic data regarding these patients were analysed in order to determine risk factors for postoperative aspiration. Post-discharge feeding routes and therapeutic interventions were extracted to examine the time spent using alternate feeding routes because of aspiration risk or poor caloric intake. Modified barium swallow study results were used to evaluate the effectiveness of the test as a diagnostic tool.

A retrospective study was undertaken of neonates who had undergone heart surgery from July, 2013 to January, 2014. Data describing patient demographics, feeding methods, and follow-up visits were recorded and compared using a χ2 test for goodness of fit and a Kaplan–Meier graph.

The patient population included 62 infants – 36 of whom were male, and 10 who were born with single-ventricle circulation. The median age at surgery was 6 days (interquartile range=4 to 10 days). Modified barium swallow study results showed that 46% of patients (n=29) aspirated or were at risk for aspiration, as indicated by laryngeal penetration. In addition, 48% (n=10) of subjects with a negative barium swallow or no swallow study demonstrated clinical aspiration events. Tube feedings were required by 66% (n=41) of the participants. The median time spent on tube feeds, whether in combination with oral feeds or exclusive use of a nasogastric or gastric tube, was 54 days; 44% (n=27) of patients received tube feedings for more than 120 days. Premature infants were significantly more likely to have aspiration events than infants delivered at full gestational age (OR p=0.002). Infants with single-ventricle circulation spent a longer time on tube feeds (median=95 days) than infants with two-ventricle defects (median=44 days); the type of cardiac defect was independent of prevalence of an aspiration event.

Aspiration is common following neonatal cardiac surgery. The modified barium swallow study is often used to identify aspiration events and to determine an infant’s risk for aspirating. This leads to a high proportion of infants who require tube feedings following neonatal cardiac surgery.

CHD occurs in about 1% of the United States population, and is now the most common cardiac condition affecting women during pregnancy.

An anonymous, single-site, cross-sectional, 50-question survey was performed at a national Adult Congenital Heart Disease conference to assess the level of knowledge, attitudes, and perceptions regarding cardiac care during pregnancy in women with CHD.

A total of 77 women completed the survey. Among them, 50% (n=39) had moderate and 38% (n=29) had severely complex disease; 30% (n=23) of women were told that pregnancy was contraindicated given their underlying cardiac condition. Almost two-thirds (n=50) report being categorised as high risk for adverse cardiovascular events. During pregnancy, 84% (n=65) preferred their cardiologist to have trained in adult CHD, 44% (n=34) were satisfied with adult cardiologists, and 36% (n=28) with paediatric cardiologists. Only 48% (n=37) were aware that a fetal echocardiogram was indicated. Only 35% (n=27) discussed modes of delivery with their providers, and 70% (n=54) preferred their prenatal cardiology visits at an adult hospital. Up to 85% (n=64) of them had discussed contraception with their cardiologists, and 72% (n=56) felt they needed high-risk maternal–fetal medicine to be involved with their care.

Despite seeking medical care, these pregnant women did not have a full understanding of their condition and their cardiovascular risk during pregnancy. On the basis of these results, further efforts are needed to improve the knowledge, attitudes, and perceptions of women with CHD in relation to their cardiac and obstetric management during pregnancy.

A few studies have evaluated the impact of clinical trial results on practice in paediatric cardiology. The Infant Single Ventricle (ISV) Trial results published in 2010 did not support routine use of the angiotensin-converting enzyme inhibitor enalapril in infants with single-ventricle physiology. We sought to assess the influence of these findings on clinical practice.

A web-based survey was distributed via e-mail to over 2000 paediatric cardiologists, intensivists, cardiothoracic surgeons, and cardiac advance practice nurses during three distribution periods. The results were analysed using McNemar’s test for paired data and Fisher’s exact test.

The response rate was 31.5% (69% cardiologists and 65% with >10 years of experience). Among respondents familiar with trial results, 74% reported current practice consistent with trial findings versus 48% before trial publication (p<0.001); 19% used angiotensin-converting enzyme inhibitor in this population “almost always” versus 36% in the past (p<0.001), and 72% reported a change in management or improved confidence in treatment decisions involving this therapy based on the trial results. Respondents familiar with trial results (78%) were marginally more likely to practise consistent with the trial results than those unfamiliar (74 versus 67%, p=0.16). Among all respondents, 28% reported less frequent use of angiotensin-converting enzyme inhibitor over the last 3 years.

Within 5 years of publication, the majority of respondents was familiar with the Infant Single Ventricle Trial results and reported less frequent use of angiotensin-converting enzyme inhibitor in single-ventricle infants; however, 28% reported not adjusting their clinical decisions based on the trial’s findings.

Automated external defibrillators can be life-saving in out-of-hospital cardiac arrest.

Our aim was to review our experience of prescribing automated external defibrillators for children at increased risk of sudden arrhythmic death.

We reviewed all automated external defibrillators issued by the Scottish Paediatric Cardiac Electrophysiology Service from 2005 to 2015. All parents were given resuscitation training according to the Paediatric Resuscitation Guidelines, including the use of the automated external defibrillator.

A total of 36 automated external defibrillators were issued to 36 families for 44 children (27 male). The mean age at issue was 8.8 years. Diagnoses at issue included long QT syndrome (50%), broad complex tachycardia (14%), hypertrophic cardiomyopathy (11%), and catecholaminergic polymorphic ventricular tachycardia (9%). During the study period, the automated external defibrillator was used in four (9%) children, and in all four the automated external defibrillator correctly discriminated between a shockable rhythm – polymorphic ventricular tachycardia/ventricular fibrillation in three patients with one or more shocks delivered – and non-shockable rhythm – sinus rhythm in one patient. Of the three children, two of them who received one or more shocks for ventricular fibrillation/polymorphic ventricular tachycardia survived, but one died as a result of recurrent torsades de pointes. There were no other deaths.

Parents can be taught to recognise cardiac arrest, apply resuscitation skills, and use an automated external defibrillator. Prescribing an automated external defibrillator should be considered for children at increased risk of sudden arrhythmic death, especially where the risk/benefit ratio of an implantable defibrillator is unclear or delay to defibrillator implantation is deemed necessary.

The relationship between interatrial communication, ductus arteriosus, and pulmonary flow in transposition of the great arteries and intact ventricular septum may help predict postnatal desaturation.

Echocardiographic data of 45 fetuses with transposition of the great arteries and intact ventricular septum and 50 age-matched controls were retrospectively reviewed. Interatrial communication, left and right ventricular output, flow in the ductus arteriosus, as well as effective pulmonary flow were measured. Patients were divided into two groups on the basis of postnatal saturations: group 1 had saturations ⩽50% and group 2 >50%.

Of 45 fetuses, 13 (26.7%) were classified into group 1. Compared with fetuses in group 2, they had a smaller interatrial communication (2.9 versus 4.0 mm, p=0.004) and more retrograde diastolic flow in the ductus arteriosus (92 versus 23%, p=0.002). Both groups showed a significant decrease in ductal flow compared with controls. Patients in group 2 had a higher effective pulmonary flow compared with controls. There was a mild correlation between left ventricular output and size of the interatrial communication (Spearman’s rank correlation 0.44).

A retrograde diastolic flow is present in most of the fetuses with postnatal desaturation. Fetuses with transposition of the great arteries have a lower flow through the ductus arteriosus compared with controls. Fetuses without restrictive foramen ovale have higher effective pulmonary flow. Peripheral pulmonary vasodilatation due to higher oxygen saturation in pulmonary arteries in the case of transposition of the great arteries could be one possible cause.

There is no consensus or theoretical explanation regarding the optimal location for the fenestration during the Fontan operation. We investigated the impact of the location of the fenestration on Fontan haemodynamics using a three-dimensional Fontan model in various physiological conditions.

A three-dimensional Fontan model was constructed on the basis of CT images, and a 4-mm-diameter fenestration was located between the extracardiac Fontan conduit and the right atrium at three positions: superior, middle, and inferior part of the conduit. Haemodynamics in the Fontan route were analysed using a three-dimensional computational fluid dynamic model in realistic physiological conditions, which were predicted using a lumped parameter model of the cardiovascular system. The respiratory effect of the caval flow was taken into account. The flow rate through the fenestration, the effect of lowering the central venous pressure, and wall shear stress in the Fontan circuit were evaluated under central venous pressures of 10, 15, and 20 mmHg. The pulse power index and pulsatile energy loss index were calculated as energy loss indices.

Under all central venous pressures, the middle-part fenestration demonstrated the most significant effect on enhancing the flow rate through the fenestration while lowering the central venous pressure. The middle-part fenestration produced the highest time-averaged wall shear stress, pressure pulse index, and pulsatile energy loss index.

Despite slightly elevated energy loss, the middle-part fenestration most significantly increased cardiac output and lowered central venous pressure under respiration in the Fontan circulation.

The objectives of this study were to compare the quality-of-life scores of Malaysian children with CHD and their healthy siblings, to determine the level of agreement between proxy-reports and child self-reports, and to examine variables that have an impact on quality of life in those with CHD.

Parental-proxy scores of the Pediatric Quality of Life Inventory 4.0 core scales were obtained for 179 children with CHD and 172 siblings. Intra-class coefficients were derived to determine the levels of proxy–child agreement in 66 children aged 8–18 years. Multiple regression analysis was used to determine factors that impacted Pediatric Quality of Life Inventory scores.

Proxy scores were lower in children with CHD than siblings for all scales except physical health. Maximum differences were noted in children aged 5–7 years, whereas there were no significant differences in the 2–4 and 13–18 years age groups. Good levels of proxy–child agreement were found in children aged 8–12 years for total, psychosocial health, social, and school functioning scales (correlation coefficients 0.7–0.8). In children aged 13–18 years, the level of agreement was poor to fair for emotional and social functioning. The need for future surgery and severity of symptoms were associated with lower scores.

Differences in proxy perception of quality of life appear to be age related. The level of proxy–child agreement was higher compared with other reported studies, with lower levels of agreement in teenagers. Facilitating access to surgery and optimising control of symptoms may improve quality of life in this group of children.

Percutaneous balloon valvuloplasty is the primary treatment for critical pulmonary valve stenosis in neonates. Thus far, a few studies have reported long-term results of this technique in neonatal critical pulmonary valve stenosis.

We carried out a retrospective study of all consecutive newborns with critical pulmonary valve stenosis subjected to percutaneous balloon valvuloplasty at a single centre, between 1994 and 2014, to assess its immediate and long-term safety and efficacy.

A total of 24 neonates presented with critical pulmonary valve stenosis. The mean diameter of the pulmonary annulus was 7 mm (±1.19); 33.3% had a dysplastic pulmonary valve, and 92% were started on prostaglandin E1 treatment. Percutaneous balloon valvuloplasty was performed at a mean age of 4.0±4.3 days using, on average, a balloon-to-pulmonary annulus ratio of 1.18 mm (with a range from 0.9 to 1.43). Immediate success was achieved in 22/24 patients (92%) with a reduction in the pulmonary transvalvular peak gradient (p<0.05) and in the right ventricle/systemic pressure ratio (p<0.05). There was one death (4%) 6 days after the procedure, and 29.2% of them had transient rhythm complications. For a mean follow-up time of 8.4 years, the re-intervention rate was 42.9%. In total, 14 re-interventions were performed in nine neonates, including surgery in six. Freedom from re-intervention was 50% at 8 years and 43% at 10 and 15 years.

This series, to the best of our knowledge, has had the longest follow-up of neonates with critical pulmonary valve stenosis. Percutaneous balloon valvuloplasty is a safe and effective treatment, and in our study 75% of the patients were exclusively treated using this technique.

We carried out a retrospective review of patients who underwent repair for ventricular septal defect and aortic arch obstruction – coarctation or interrupted aortic arch – between July, 2002 and June, 2013. Echocardiographic data were reviewed, and the need for re-intervention was evaluated.

A total of 89 patients were included in this study. A significant left ventricular outflow tract growth was noticed after surgical repair. Preoperatively, the mean left ventricular outflow tract Z-score was −1.46±1 (range −5.5 to 1.1) and increased to a mean value of −0.7±1.3 (range −2.7 to 3.2) at last follow-up (p=0.0001), demonstrating relevant growth of the left ventricular outflow tract after repair for ventricular septal defect and aortic arch obstruction. After primary repair, 11 patients (12.3%) required re-intervention with surgical repair for left ventricular outflow tract obstruction after a mean period of 36±21 months. There were no significant differences in age, weight, and indexed aortic valve and left ventricular outflow tract measurements between those who developed obstruction and those who did not.

Significant left ventricular outflow tract growth is expected after repair of ventricular septal defect and aortic arch obstruction. Small aortic valve and left ventricular outflow tract at diagnosis are not risk factors to predict the need for surgical re-intervention for left ventricular outflow tract obstruction in future.

The aim of this study was to determine whether the exercise tolerance test can provide diagnostic and prognostic information regarding children and young adults and help predict outcome.

A total of 87 patients, aged 7–29 years (median 13, mean 13.4) were selected retrospectively. They underwent exercise test at the Freeman Hospital from December, 2015 to May, 2016. There were two groups of patients – 46 had symptoms such as chest pain, palpitations, syncope, or dyspnoea on exertion and no cardiac diagnosis, and 40 patients had a cardiac diagnosis such as hypertrophic cardiomyopathy, transposition of the great arteries with post-arterial switch operation, aortic stenosis or regurgitation, tetralogy of Fallot, abnormal coronary arteries, Wolff–Parkinson–White syndrome, or supraventricular tachycardia.

In the group of patients with symptoms and no cardiac diagnosis, exercise test was negative and there was no exercise-induced arrhythmia; 31 patients were discharged from follow-up. In the group of patients with a cardiac diagnosis, four patients had to be treated – one had ablation, one the Ross procedure, one aortic valve repair, and one aortic valve ballooning; in addition, seven patients had to be further investigated – one had signal average electrocardiogram, one stress cardiac MRI, two cardiac MRI, one lung function test, one reveal device, and one 24 hours electrocardiogram. In all, 43 patients were further followed-up from both groups.

The exercise tolerance test is useful for clinical decision making in children and young adults with a cardiac diagnosis. In this study, the exercise tolerance test in patients with symptoms suggestive of cardiac disease but no cardiac diagnosis did not reveal any new diagnoses.

To evaluate the experiences of parenting a child with hypoplastic left heart syndrome after the child has been discharged home from hospital.

A study of the parents’ experiences using face-to-face interviews and psychometric measures with parents whose child had survived stage surgery.

Parents were interviewed within the home environment or within the hospital if that was their choice.

A total of 29 parents (16 mothers and 13 fathers) of surviving children.

A semi-structured face-to-face interview plus psychometric tests (parent demographics, Maslach Burnout Inventory, Impact on Family Scale, and the Psychological Check List – Civilian).

The parents’ experience in supporting a child with hypoplastic left heart syndrome is one of stress, of commitment, and of love. Although parents experienced joy in their child, they were also subjected to anxiety with four parents test positive to post-traumatic stress disorder and hypervigilance while monitoring their child’s condition. Parents lived with many difficulties, and demands.

Pulmonary lymphangiectasia associated with hypoplastic left heart syndrome with an intact or restrictive atrial septum may result from increased left atrial pressure, and is associated with worse outcomes following staged reconstruction due to lung dysfunction and significant hypoxaemia. Our objective was to characterise the incidence of pulmonary lymphangiectasia in cases of early mortality following stage 1 reconstructions.

An institutional cardiac surgical database was retrospectively searched for patients who died within 30 days following a stage 1 reconstruction between 1 January, 1984 and 31 December, 2013. During that period, 1669 stage 1 procedures were performed. Autopsy lung specimens were reviewed by a paediatric pathologist. Patients who died of suspected technical issues were excluded.

A total of 54 patients were included, and of these seven cases (8.5%) of pulmonary lymphangiectasia were identified. The mean estimated gestational age was 38.2±2.4 weeks, and the mean birth weight was 3.0±0.6 kg. The median interval between surgery and death was 1 day (with a range from 0 to 18 days). The atrial septum was intact in one patient (14.3%), restrictive in three patients (42.9%), and unrestrictive in three patients (42.9%).

Pulmonary lymphangiectasia may develop in hypoplastic left heart syndrome with or without a restrictive atrial septum. As standard prenatal diagnostic evaluations and treatment methods for pulmonary lymphangiectasia are limited, this may be an important contributor to early and late mortality following stage 1 reconstruction for hypoplastic left heart syndrome.

The aim of this study was to evaluate the safety and feasibility of a passive range of motion exercise programme for infants with CHD.

This non-randomised pilot study enrolled 20 neonates following Stage I palliation for single-ventricle physiology. Trained physical therapists administered standardised 15–20-minute passive range of motion protocol, for up to 21 days or until hospital discharge. Safety assessments included vital signs measured before, during, and after the exercise as well as adverse events recorded through the pre-Stage II follow-up. Feasibility was determined by the percent of days that >75% of the passive range of motion protocol was completed.

A total of 20 infants were enrolled (70% males) for the present study. The median age at enrolment was 8 days (with a range from 5 to 23), with a median start of intervention at postoperative day 4 (with a range from 2 to 12). The median hospital length of stay following surgery was 15 days (with a range from 9 to 131), with an average of 13.4 (with a range from 3 to 21) in-hospital days per patient. Completion of >75% of the protocol was achieved on 88% of eligible days. Of 11 adverse events reported in six patients, 10 were expected with one determined to be possibly related to the study intervention. There were no clinically significant changes in vital signs. At pre-Stage II follow-up, weight-for-age z-score (−0.84±1.20) and length-for-age z-score (−0.83±1.31) were higher compared with historical controls from two earlier trials.

A passive range of motion exercise programme is safe and feasible in infants with single-ventricle physiology. Larger studies are needed to determine the optimal duration of passive range of motion and its effect on somatic growth.

Mitral valve anatomy has a significant impact on potential surgical options for patients with hypoplastic or borderline left ventricle. Papillary muscle morphology is a major component regarding this aspect. The purpose of this study was to use cardiac magnetic resonance to describe the differences in papillary muscle anatomy between normal, borderline, and hypoplastic left ventricles.

We carried out a retrospective, observational cardiac magnetic resonance study of children (median age 5.36 years) with normal (n=30), borderline (n=22), or hypoplastic (n=13) left ventricles. Borderline and hypoplastic cases had undergone an initial hybrid procedure. Morphological features of the papillary muscles, location, and arrangement were analysed and compared across groups.

All normal ventricles had two papillary muscles with narrow pedicles; however, 18% of borderline and 46% of hypoplastic cases had a single papillary muscle, usually the inferomedial type. In addition, in borderline or hypoplastic ventricles, the supporting pedicle occasionally displayed a wide insertion along the ventricular wall. The length ratio of the superolateral support was significantly different between groups (normal: 0.46±0.08; borderline: 0.39±0.07; hypoplastic: 0.36±0.1; p=0.009). No significant difference, however, was found when analysing the inferomedial type (0.42±0.09; 0.38±0.07; 0.39±0.22, p=0.39). The angle subtended between supports was also similar among groups (113°±17°; 111°±51° and 114°±57°; p=0.99). A total of eight children with borderline left ventricle underwent biventricular repair. There were no significant differentiating features for papillary muscle morphology in this subgroup.

The superolateral support can be shorter or absent in borderline or hypoplastic left ventricle cases. The papillary muscle pedicles in these patients often show a broad insertion. These changes have important implications on surgical options and should be described routinely.

We explored left ventricular mechanics using speckle-tracking echocardiography in 35 patients with a history of Kawasaki disease at least 6 months after the acute phase. We also included 30 healthy children as controls. Strain data sets were acquired for the quantification of left ventricular global strain, segmental strain, and left ventricular ejection fraction.

The mean age of our patients was 25.6±15.4 months. At a median follow-up of 57.5 months (16.5–98.2), although both values were in the normal range, the mean left ventricular ejection proportion of patients (57.3%) was a little lower than that of controls (p⩽0.05). Patient strain values at the basal inferoseptal (20.0), basal anterolateral (19.5), apical septal (23.3), and apical inferior (24.0) segments were lower compared with controls. In all, seven patients had coronary aneurysms during follow-up. Kawasaki disease patients with pyuria had lower left ventricular strain at the mid anterior, mid anteroseptal, apical anterior, and apical inferior segments and global longitudinal strain compared with patients with no pyuria (p⩽0.05).

In children with a history of Kawasaki disease, impairment of left ventricular mechanics occurs especially within the left anterior descending artery territories.