We sought to evaluate the risk and image quality from cardiovascular CT in patients across all stages of single-ventricle palliation, and to define accuracy by comparing findings with intervention and surgery.

Consecutive CT scans performed in patients with single-ventricle heart disease were retrospectively reviewed at a single institution. Diagnosis, sedation needs, estimated radiation dose, and adverse events were recorded. Anatomical findings, image quality (1–4, 1=optimal), and discrepancy compared with interventional findings were determined. Results are described as medians with their 25th and 75th percentiles.

From January, 2010 to August, 2015, 132 CT scans were performed in single-ventricle patients of whom 20 were neonates, 52 were post-Norwood, 15 were post-Glenn, and 45 were post-Fontan. No sedation was used in 76 patients, 47 were under minimal or moderate sedation, and nine were under general anaesthesia. The median image quality score was 1.2. The procedural dose–length product was 24 mGy-cm, and unadjusted and adjusted radiation doses were 0.34 (0.2, 1.8) and 0.82 (0.55, 1.88) mSv, respectively. There was one adverse event. No major and two minor discrepancies were noted at the time of 79 surgical and 10 catheter-based interventions.

Cardiovascular CT can be performed with a low radiation exposure in patients with single-ventricle heart disease. Its accuracy compared with that of interventional findings is excellent. CT is an effective advanced imaging modality when a non-invasive pathway is desired, particularly if cardiac MRI poses a high risk or is contraindicated.

Previous studies suggest that children with congenital cardiac diagnoses report lower quality of life when compared with healthy norms. A few studies have evaluated quality of life specifically in children born with hypoplastic left heart syndrome, a condition requiring several surgeries before age three. The aim of this study was to use an empirically validated and standardised measure – the Pediatric Quality of Life Inventory – to evaluate quality of life in children with hypoplastic left heart syndrome and compare the findings with similar, medically complicated samples.

The parent-report Pediatric Quality of Life Inventory was administered, and demographic information was collected through an internet portal. A total of 121 caregivers of children with hypoplastic left heart syndrome responded. The sample included children aged 2–18 years (M=10.81 years). Independent sample t-tests were used to compare our sample with published norms of healthy children and children with acute or chronic illnesses.

Children with hypoplastic left heart syndrome were rated as having significantly lower overall quality-of-life scores (M=59.69) compared with published norms of children without medical diagnoses (M=83.00) and those with acute (M=78.70) or chronic (M=77.19) illnesses (p<0.001). Children with hypoplastic left heart syndrome complicated by a stroke or seizure (15%) reported the lowest quality of life. The results held for all subscales (p<0.001).

Children with hypoplastic left heart syndrome appear to be a significantly vulnerable population with difficulties in functioning across psychosocial domains and across the age span. Further research is required to facilitate early identification of the need for resources for these children and families, especially for children who experience additional medical complications.

Few data are available regarding the use of metolazone in infants in cardiac intensive care. Researchers need to carry out further evaluation to characterise the effects of this treatment in this population.

This is a descriptive, retrospective study carried out in patients less than a year old. These infants had received metolazone over a 2-year period in the paediatric cardiac intensive care unit at our institution. The primary goal was to measure the change in urine output from 24 hours before the start of metolazone therapy to 24 hours after. Patient demographic variables, laboratory data, and fluid-balance data were analysed.

The study identified 97 infants with a mean age of 0.32±0.25 years. Their mean weight was 4.9±1.5 kg, and 58% of the participants were male. An overall 63% of them had undergone cardiovascular surgery. The baseline estimated creatinine clearance was 93±37 ml/minute/1.73 m2. Initially, the participants had received a metolazone dose of 0.27±0.10 mg/kg/day, the maximum dose being 0.43 mg/kg/day. They had also received other diuretics during metolazone initiation, such as furosemide (87.6%), spironolactone (58.8%), acetazolamide (11.3%), bumetanide (7.2%), and ethacrynic acid (1%). The median change in urine output after metolazone was 0.9 ml/kg/hour (interquartile range 0.15–1.9). The study categorised a total of 66 patients (68.0%) as responders. Multivariable analysis identified acetazolamide use (p=0.002) and increased fluid input in the 24 hours after metolazone initiation (p<0.001) as being significant for increased urine output. Changes in urine output were not associated with the dose of metolazone (p>0.05).

Metolazone increased urine output in a select group of patients. Efficacy can be maximised by strategic selection of patients.

Through this study we aimed to assess the educational level and employment status of adults with CHD in Germany.

Data were acquired from an online survey carried out in 2015 by the German National Register for Congenital Heart Defects. A total of 1458 adults with CHD participated in the survey (response rate: 37.6%). For 1198 participants, detailed medical information, such as main cardiac diagnosis and information from medical reports, was available.

Of the participants surveyed (n=1198), 54.5% (n=653) were female, and the mean age was 30 years. The majority of respondents (59.4%) stated that they had high education levels and that they were currently employed (51.1%). Patients with simple CHD had significantly higher levels of education (p<0.001) and were more likely to be employed (p=0.01) than were patients with complex CHD.

More than half of the participants had high education levels and the majority were employed. The association between CHD and its severity and individuals’ educational attainment should be investigated more closely in future studies.

We have previously shown that the minor alleles of vascular endothelial growth factor A (VEGFA) single-nucleotide polymorphism rs833069 and superoxide dismutase 2 (SOD2) single-nucleotide polymorphism rs2758331 are both associated with improved transplant-free survival after surgery for CHD in infants, but the underlying mechanisms are unknown. We hypothesised that one or both of these minor alleles are associated with better systemic ventricular function, resulting in improved survival.

This study is a follow-up analysis of 422 non-syndromic CHD patients who underwent neonatal cardiac surgery with cardiopulmonary bypass. Echocardiographic reports were reviewed. Systemic ventricular function was subjectively categorised as normal, or as mildly, moderately, or severely depressed. The change in function was calculated as the change from the preoperative study to the last available study. Stepwise linear regression, adjusting for covariates, was performed for the outcome of change in ventricular function. Model comparison was performed using Akaike’s information criterion. Only variables that improved the model prediction of change in systemic ventricular function were retained in the final model.

Genetic and echocardiographic data were available for 335/422 subjects (79%). Of them, 33 (9.9%) developed worse systemic ventricular function during a mean follow-up period of 13.5 years. After covariate adjustment, the presence of the VEGFA minor allele was associated with preserved ventricular function (p=0.011).

These data support the hypothesis that the mechanism by which the VEGFA single-nucleotide polymorphism rs833069 minor allele improves survival may be the preservation of ventricular function. Further studies are needed to validate this genotype–phenotype association and to determine whether this mechanism is related to increased vascular endothelial growth factor production.

Dilated cardiomyopathy is a rare but serious disorder in children. No effective diagnostic or treatment tools are readily available. This study aimed to evaluate the efficacy of intravenous immunoglobulins in children with new onset dilated cardiomyopathy.

In this retrospective cohort study, 94 children with new onset dilated cardiomyopathy were followed during a median period of 33 months. All patients with secondary dilated cardiomyopathy – for example, genetic, auto-immune or structural defects – had been excluded. Viral tests were performed in all patients and 18 (19%) children met the criteria for the diagnosis “probable or definite viral myocarditis”. Intravenous immunoglobulins were administered to 21 (22%) patients. Overall transplant-free survival was 75% in 5 years and did not differ between treatment groups. The treatment was associated with a higher recovery rate within 5 years, compared with non-treated children (70 versus 43%, log rank=0.045). After correction for possible confounders the hazard ratio for recovery with intravenous immunoglobulins was not significant (hazard ratio: 2.1; 95% CI: 1.0–4.6; p=0.056). Administration of intravenous immunoglobulins resulted in a greater improvement in the shortening fraction of the left ventricle.

In our population of children with new onset dilated cardiomyopathy, of either viral or idiopathic origin, intravenous immunoglobulins were administered to a minority of the patients and did not influence transplant-free survival, but were associated with better improvement of systolic left ventricular function and with better recovery. Our results support the concept that children with new onset dilated cardiomyopathy might benefit from intravenous immunoglobulins.

Cerebrovascular reactivity monitoring has been used to identify the lower limit of pressure autoregulation in adult patients with brain injury. We hypothesise that impaired cerebrovascular reactivity and time spent below the lower limit of autoregulation during cardiopulmonary bypass will result in hypoperfusion injuries to the brain detectable by elevation in serum glial fibrillary acidic protein level.

We designed a multicentre observational pilot study combining concurrent cerebrovascular reactivity and biomarker monitoring during cardiopulmonary bypass. All children undergoing bypass for CHD were eligible. Autoregulation was monitored with the haemoglobin volume index, a moving correlation coefficient between the mean arterial blood pressure and the near-infrared spectroscopy-based trend of cerebral blood volume. Both haemoglobin volume index and glial fibrillary acidic protein data were analysed by phases of bypass. Each patient’s autoregulation curve was analysed to identify the lower limit of autoregulation and optimal arterial blood pressure.

A total of 57 children had autoregulation and biomarker data for all phases of bypass. The mean baseline haemoglobin volume index was 0.084. Haemoglobin volume index increased with lowering of pressure with 82% demonstrating a lower limit of autoregulation (41±9 mmHg), whereas 100% demonstrated optimal blood pressure (48±11 mmHg). There was a significant association between an individual’s peak autoregulation and biomarker values (p=0.01).

Individual, dynamic non-invasive cerebrovascular reactivity monitoring demonstrated transient periods of impairment related to possible silent brain injury. The association between an impaired autoregulation burden and elevation in the serum brain biomarker may identify brain perfusion risk that could result in injury.

The aim of this study was to explore perinatal and early postnatal outcomes in fetuses with prenatally diagnosed d-transposition of the great arteries and impacts of standardised prenatal consultation.

All fetuses with prenatally diagnosed d-transposition of the great arteries prospectively enrolled at South China cardiac centre from 2011 to 2015. Standardised prenatal consultation was introduced in 2013 and comprehensive measures were implemented, such as establishing fetal CHD Outpatient Consultation Service, performing standard prenatal consultation according to specifications, and establishing a multidisciplinary team with senior specialists performing in-person consultations. Continuous follow-up investigation was conducted. Perinatal and postnatal outcomes were compared before and after consultation including live birth, elective termination of pregnancy, spontaneous fetal death, stillbirths, referral for surgery, and survival.

In all, 146 fetuses were enrolled with 41 (28%) lost to follow-up. Among 105 remaining fetuses, 29 (28%) were live births and 76 (72%) were terminated. After consultation, live birth rate was higher (50 versus 33%) and termination rate was lower (50 versus 76%), although there was no statistical significance. Excluding three live births without postnatal d-transposition of the great arteries, 65% (17/26) underwent arterial switch operation within 30 days. A total of three in-hospital deaths occurred and during the 10-month follow-up period, one death was observed. In one case, the switch procedure was performed at 13 months and the infant survived. Out of eight infants without arterial switch operation, two died.

Live birth rate increased after consultation; however, termination remained high. Combining termination, patients without arterial switch operation, and operative mortality, outcomes of d-transposition of the great arteries infants can be improved. Standard consultation, multidisciplinary collaboration, and improved perinatal care are important to improve outcomes.

Obesity increases the risk for various cardiovascular problems. Increase in body mass index is often an independent risk factor for the development of elevated blood pressure and clustering of various cardiovascular risk factors.

To determine early markers of left ventricular affection in obese patients before the appearance of left ventricular hypertrophy.

In this cross-sectional study, we evaluated 42 obese patients and 30 healthy controls. Their ages ranged from 6 to 19 years. Studied children were subjected to anthropometric, lipid profile, and serum Troponin I level measurements. Echocardiographic evaluation performed to assess the left ventricle included left ventricular dimension measurement using motion-mode echocardiography, based on which patients with left ventricular hypertrophy (10 patients) were eliminated, as well as conventional and tissue Doppler imaging.

Tissue Doppler findings in the study groups showed that the ratio of transmitral early diastolic filling velocity to septal peak early diastolic myocardial velocity (E/e′) was significantly higher in cases compared with controls [6.9±1.4 versus 9.0±1.6, p (Pearson’s coefficient)=0.001, respectively]. The level of cardiac troponin I was significantly higher in cases compared with controls [0.14±0.39 ng/ml versus 0.01±0.01 ng/ml, p (Pearson’s coefficient)=0.047, respectively] and there was a significant correlation between troponin I and transmitral early diastolic filling velocity to septal peak early diastolic myocardial velocity ratio (E/e′) [R (correlation coefficient)=0.6].

Tissue Doppler Imaging and Troponin I evaluation proved useful tools to detect early affection of the left ventricle in obese patients even in the absence of left ventricular hypertrophy.

The relationship between sildenafil dosing, exposure, and systemic hypotension in infants is incompletely understood.

The aim of this study was to characterise the relationship between predicted sildenafil exposure and hypotension in hospitalised infants.

We extracted information on sildenafil dosing and clinical characteristics from electronic health records of 348 neonatal ICUs from 1997 to 2013, and we predicted drug exposure using a population pharmacokinetic model.

We identified 232 infants receiving sildenafil at a median dose of 3.2 mg/kg/day (2.0, 6.0). The median steady-state area under the concentration–time curve over 24 hours (AUC24,SS) and maximum concentration of sildenafil (Cmax,SS,SIL) were 712 ng×hour/ml (401, 1561) and 129 ng/ml (69, 293), respectively. Systemic hypotension occurred in 9% of the cohort. In multivariable analysis, neither dosing nor exposure were associated with systemic hypotension: odds ratio=0.96 (95% confidence interval: 0.81, 1.14) for sildenafil dose; 0.87 (0.59, 1.28) for AUC24,SS; 1.19 (0.78, 1.82) for Cmax,SS,SIL.

We found no association between sildenafil dosing or exposure with systemic hypotension. Continued assessment of sildenafil’s safety profile in infants is warranted.

Norwood palliation for patients with single ventricle heart disease is associated with a significant risk for acute kidney injury, which portends a worse prognosis. We sought to investigate the impact of hybrid stage I palliation (Hybrid) on acute kidney injury risk.

This study is a single-centre prospective case–control study of seven consecutive neonates with single ventricle undergoing Hybrid palliation. Levels of serum creatinine and four novel urinary biomarkers, namely neutrophil gelatinase-associated lipocalin, interleukin-18, liver fatty acid-binding protein, and kidney injury molecule-1, were obtained before and after palliation. Acute kidney injury was defined as a ⩾50% increase in serum creatinine within 48 hours after the procedure. Data were compared with a contemporary cohort of 12 neonates with single ventricle who underwent Norwood palliation.

Patients who underwent Hybrid were more likely to be high-risk candidates (86 versus 25%, p=0.01) compared with those who underwent Norwood. Despite similar preoperative serum creatinine levels, there was a trend towards higher levels of postoperative peak serum creatinine (0.7 [0.63, 0.94] versus 0.56 [0.47, 0.74], p=0.06) and rate of acute kidney injury (67 versus 29%, p=0.17) in the Norwood cohort. Preoperative neutrophil gelatinase-associated lipocalin (58.4 [11, 86.3] versus 6.3 [5, 16.2], p=0.07) and interleukin-18 (30.6 [9.6, 167.2] versus 6.3 [6.3, 16.4], p=0.03) levels were higher in the Hybrid cohort. Nevertheless, longitudinal mixed-effect models demonstrated Hybrid palliation to be a protective factor against increased postoperative levels of neutrophil gelatinase-associated lipocalin (estimate −1.8 [−3.0, −9.0], p<0.001) and liver fatty acid-binding protein (−49.3 [−89.7, −8.8], p=0.018).

In this single-centre case–control study, postoperative acute kidney injury risk did not differ significantly by single ventricle stage I treatment strategy; however, postoperative elevation in novel urinary biomarkers, consistent with subclinical kidney injury, was encountered in the Norwood cohort but not in the Hybrid cohort.

Adults with CHD often exhibit complex cardiac abnormalities, whose management requires specific clinical and surgical expertise. To enable easier access of these patients to highly specialised care, we implemented a collaborative programme that incorporates medical and surgical specialists belonging to both paediatric and adult cardiovascular institutions.

The objective of this study was to review the experience gained and to analyse the surgical outcome of major cardiac surgery.

We retrospectively reviewed all consecutive patients admitted for major cardiac surgery using our network between January, 2010 and December, 2013. Analysis of surgical outcome was performed in patients selected for major cardiac surgery with cardiopulmonary bypass. Early and late outcomes were evaluated.

Out of a total of 433 inward patients, 86 were selected for surgery. The median age was 25.5 years, –64 patients (74.4%) had previously undergone heart surgery, and –55 patients (64%) had been subjected to at least one sternotomy. Abnormalities of the left ventricular and right ventricular outflow tract were the most frequent (37.2% and 30.2%, respectively), and despite high-surgical complexity only one death occurred (in-hospital mortality 1.1%). On a median follow-up time of 4 years no deaths and no heart-failure events have occurred; one patient underwent further cardiac surgery programmed at the time of discharge.

Low mortality and morbidity rates can be obtained in high-surgical complexity adults with CHD populations when paediatric and adult cardiac specialists operate in the same multidisciplinary environment.

Echocardiographic screening represents an opportunity for reduction in the global burden of rheumatic heart disease. A focussed single-view screening protocol could allow for the rapid training of healthcare providers and screening of patients.

The aim of this study was to determine the sensitivity and specificity of a focussed single-view hand-held echocardiographic protocol for the diagnosis of rheumatic heart disease in children.

A total of nine readers were divided into three reading groups; each interpreted 200 hand-held echocardiography studies retrospectively as screen-positive, if mitral regurgitation ⩾1.5 cm and/or any aortic insufficiency were observed, or screen-negative from a pooled study library. The performance of experts receiving focussed hand-held protocols, non-experts receiving focussed hand-held protocols, and experts receiving complete hand-held protocols were determined in comparison with consensus interpretations on fully functional echocardiography machines.

In all, 587 studies including 76 on definite rheumatic heart disease, 122 on borderline rheumatic heart disease, and 389 on normal cases were available for analysis. The focussed single-view protocol had a sensitivity of 81.1%, specificity of 75.5%, negative predictive value of 88.5%, and a positive predictive value of 63.2%; expert readers had higher specificity (86.1 versus 64.8%, p<0.01) but equal sensitivity. Sensitivity – experts, 96% and non-experts, 95% – and negative predictive value – experts, 99% and non-experts, 98% – were better for definite rheumatic heart disease. False-positive screening studies resulting from erroneous identification of mitral regurgitation and aortic insufficiency colour jets increased with shortened protocols and less experience (p<0.01).

Our data support a focussed screening protocol limited to parasternal long-axis images. This holds promise in making echocardiographic screening more practical in regions where rheumatic heart disease remains endemic.

To evaluate differences in interstage growth of pulmonary arteries between use of polytetrafluoroethylene and femoral vein homograft as Sano shunt during stage-I Norwood palliation.

A retrospective review of all patients who survived to the second stage following Norwood–Sano operation at two institutions was performed. Either polytetrafluoroethylene or the valved segment of femoral vein homograft was used for construction of the Sano shunt. The size of pulmonary arteries was compared at pre-Glenn catheterisation.

A total of 48 neonates with the diagnosis of hypoplastic left heart syndrome or its variants comprised the study population. Femoral vein homograft of 5–6 mm diameter was used in 14 and polytetrafluoroethylene graft of 5 mm was used in 34 patients. The two groups were comparable in terms of preoperative demographics and age at time of pre-Glenn catheterisation (3.9±0.7 versus 3.4±0.8 months, p=0.06). Patients who received femoral vein homograft demonstrated a significantly higher pre-Glenn Nakata index [264 (130–460) versus 165 (108–234) mm2/m2, p=0.004]. The individual branch pulmonary arteries were significantly larger in the femoral vein group (right, 7.8±3.6 versus 5.0±1.2, p=0.014; left, 7.2±2.1 versus 5.6±1.9, p=0.02). There were no differences in cardiac index, Qp:Qs, ventricular end-diastolic pressure or systemic oxygen saturations.

Utilisation of a valved segment of femoral vein homograft as right ventricle to pulmonary artery conduit during Norwood–Sano operation confers better interstage growth of the pulmonary arteries. Further studies are needed to evaluate the impact of femoral vein homograft on single ventricle function.

Children with hypoplastic left heart syndrome are at a risk for neurodevelopmental delays. Current guidelines recommend systematic evaluation and management of neurodevelopmental outcomes with referral for early intervention services. The Single Ventricle Reconstruction Trial represents the largest cohort of children with hypoplastic left heart syndrome ever assembled. Data on life events and resource utilisation have been collected annually. We sought to determine the type and prevalence of early intervention services used from age 1 to 4 years and factors associated with utilisation of services.

Data from 14-month neurodevelopmental assessment and annual medical history forms were used. We assessed the impact of social risk and geographic differences. Fisher exact tests and logistic regression were used to evaluate associations.

Annual medical history forms were available for 302 of 314 children. Greater than half of the children (52–69%) were not receiving services at any age assessed, whereas 20–32% were receiving two or more therapies each year. Utilisation was significantly lower in year 4 (31%) compared with years 1–3 (with a range from 40 to 48%) (p<0.001). Social risk factors were not associated with the use of services at any age but there were significant geographic differences. Significant delay was reported by parents in 18–43% of children at ages 3 and 4.

Despite significant neurodevelopmental delays, early intervention service utilisation was low in this cohort. As survival has improved for children with hypoplastic left heart syndrome, attention must shift to strategies to optimise developmental outcomes, including enrolment in early intervention when merited.

This study evaluates the morbidity, mortality, and cost differences between patients who underwent either a simple or a complex arterial switch operation.

A retrospective study of patients undergoing an arterial switch operation at a single institution was performed. Simple cases were defined as patients with d-transposition of the great arteries with usual coronary anatomy or circumflex artery originating from the right with either intact ventricular septum or ventricular septal defect. Complex cases included all other forms of coronary anatomy, aortic coarctation or arch hypoplasia, and Taussig–Bing anomalies. Costs were acquired using an institutional activity-based accounting system.

A total of 98 patients were identified, 68 patients in the simple group and 30 in the complex group. The mortality rate was 2% for the simple and 7% for the complex group, p=0.23. Major morbidities including cardiac arrest, extracorporeal membrane oxygenation, a major coronary event, surgical or catheter-based re-intervention, stroke, or permanent pacemaker placement, non-cardiac surgical procedures, mediastinitis, and sepsis did not differ between the simple and complex groups (16 versus 27%, p=0.16). The complex group had increased bleeding requiring re-exploration (0 versus 10%, p=0.04). Hospital and ICU length of stay did not differ. Complex patients had higher overall hospital costs (simple $80,749 versus complex $97,387, p=0.01) and higher postoperative costs (simple $60,192 versus complex $70,132, p=0.02). The operating room and supplies accounted for the majority of the cost difference.

Complex arterial switches can be safely performed with low rates of morbidity and mortality but at an increased cost.