Coronary artery anomalies are a heterogeneous group of congenital disorders presenting with a wide spectrum of symptoms, ranging from vague chest pain to sudden cardiac death. Despite available data, there is no consensus about the classification, nomenclature, and outcomes of coronary anomalies in the normally connected heart. In this study, we aimed to investigate clinical and angiographic characteristics of coronary arterial anomalies, as well as the frequency of atherosclerotic involvement in anomalous coronaries, diagnosed at a tertiary referral centre.

We retrospectively reviewed coronary angiograms performed between 2011 and 2015 for the presence of a coronary anomaly. A total of 111 patients with a final diagnosis of coronary anomaly were included in the study group. We also recruited 110 age- and sex-matched patients who underwent coronary angiography because of symptomatic coronary artery disease as controls.

Among 36,893 coronary angiograms, 111 (0.30%) major coronary anomalies were found. Compared with controls, the prevalence of significant atherosclerotic coronary disease was lower in patients with coronary anomalies and stable symptoms (p=0.02); however, the prevalence of significant coronary atherosclerosis was similar among patients admitted with unstable angina or myocardial infarction (p>0.05). Compared with controls, patients with an anomalous left anterior descending coronary artery had significantly less atherosclerotic involvement than those in whom the left anterior descending artery was not anomalous (p=0.005).

Although coronary artery anomalies are cited as a cause for myocardial ischaemia, atherosclerotic coronary artery disease is also frequent and may offer an alternative explanation to ischaemic symptoms. No predisposition to accelerated atherosclerosis was found, however, and atherosclerotic involvement was less frequent in some anomalous vessels.

The improved survival of patients born with CHD has led to increasing interest in research on quality of life of adult survivors. We report the findings of the first study in Malta carried out to investigate quality of life in adults with CHD under follow-up.

A self-reporting questionnaire modelled on the basis of the European Health Interview Survey 2008, including questions on mental health and vitality, was administered to consecutive adult CHD outpatients, aged 16 years and over, between May, 2013 and May, 2014. Foreigners and patients with learning difficulties or cognitive impairment were excluded. Quality-of-life data were compared with that from 371 age- and sex-matched 2008 survey responders – general population cohort. The impact of congenital lesion complexity, hospitalisation in the preceding 12 months, arrhythmias, co-morbidities, and cardiac medication use on quality of life of the CHD cohort was also investigated.

There were a total of 120 patient responders (63 males; mean age 30.53, SD 12.77 years). Overall, there were no significant differences in mental health and vitality between patient and general population cohorts, although older patients had better mental health scores compared with age-matched controls. Within the adult CHD cohort, hospitalisation in the preceding 12 months was the only factor associated with a poorer quality of life.

Overall, CHD has no negative impact on mental health and vitality in Maltese adult patients under follow-up. Patients needing frequent hospitalisations might warrant closer attention by clinical psychologists.

The aim of this study was to analyse whether changes in physical activity and body fatness are related to modifications in cardiovascular risk factors among adolescents.

A sample of 89 healthy adolescents was recruited for this study. We assessed habitual physical activity, body fat percentage, arterial thickness, blood sample, and biological maturation. Multivariate models were used to analyse the relationships between independent and dependent variables.

Physical activity (mean difference: 429.4 steps [95% confidence interval=−427 to 1286]) and body fatness (mean difference: −0.7% [95% confidence interval=−1.6–0.2]) remained stable during the study period. Independent of changes in physical activity, for each percentage increase in body fatness, femoral intima-media thickness increased by 0.007 mm (β=0.007 [95% confidence interval=0.001–0.013]). Longitudinal relationships were found for high-density lipoprotein-cholesterol (β=−0.477 mg/dl [95% confidence interval=−0.805 to −0.149]) and triacylglycerol (β=2.329 mg/dl [95% confidence interval=0.275–4.384]).

Changes in body fatness are more important than the amount of physical activity on cardiovascular and metabolic risks.

Acute kidney injury after cardiac surgery is a frequent and serious complication among children with congenital heart disease (CHD) and adults with acquired heart disease; however, the significance of kidney injury in adults after congenital heart surgery is unknown. The primary objective of this study was to determine the incidence of acute kidney injury after surgery for adult CHD. Secondary objectives included determination of risk factors and associations with clinical outcomes.

This single-centre, retrospective cohort study was performed in a quaternary cardiovascular ICU in a paediatric hospital including all consecutive patients ⩾18 years between 2010 and 2013.

Data from 118 patients with a median age of 29 years undergoing cardiac surgery were analysed. Using Kidney Disease: Improving Global Outcome creatinine criteria, 36% of patients developed kidney injury, with 5% being moderate to severe (stage 2/3). Among higher-complexity surgeries, incidence was 59%. Age ⩾35 years, preoperative left ventricular dysfunction, preoperative arrhythmia, longer bypass time, higher Risk Adjustment for Congenital Heart Surgery-1 category, and perioperative vancomycin use were significant risk factors for kidney injury development. In multivariable analysis, age ⩾35 years and vancomycin use were significant predictors. Those with kidney injury were more likely to have prolonged duration of mechanical ventilation and cardiovascular ICU stay in the univariable regression analysis.

We demonstrated that acute kidney injury is a frequent complication in adults after surgery for CHD and is associated with poor outcomes. Risk factors for development were identified but largely not modifiable. Further investigation within this cohort is necessary to better understand the problem of kidney injury.

The prevalence of overweight in children with CHD is about 26.9%. Increase in adipose tissue is related to the secretion of proinflammatory markers such as C-reactive protein. Assuming that children with CHD are exposed to other inherent risk factors for heart disease, our objective was to evaluate the correlation between levels of C-reactive protein and body mass index in children and adolescents with CHD.

A cross-sectional study with 377 children and adolescents with CHD in a clinical setting of a reference hospital was carried out. C-reactive protein data were collected after 12 hours of fasting. Nutritional status was classified according to body mass index. The patients were divided into three groups: cyanotic, acyanotic, and minimal heart defects (controls).

The mean age was 9.9±4.2 years, and 53.6% of the sample included males. The cyanotic group represented 22.3%, acyanotic 42.2%, and minimal defects 35.5% of the sample. The average body mass index percentile was 57.23±32.06. The median values of C-reactive protein were as follows: cyanotic 0.340, acyanotic with clinical repercussion 0.203, and minimal defects 0.128. There was a significant difference between the minimal defects and the cyanotic groups (p=0.023). There was a significant correlation between C-reactive protein and body mass index percentile (r=0.293, p<0.01). C-reactive protein levels were higher in girls (p=0.034). There were no significant correlations between C-reactive protein and age or birth weight.

The correlation between body mass index percentile and C-reactive protein was confirmed in this population. The prevention of overweight is paramount to avoid overlapping modifiable risk factors to those already inherent to the CHD.

The aim of this study was to describe previously unrecognised or under-recognised adverse events associated with Melody® valve implantation.

In rare diseases and conditions, it is typically not feasible to conduct large-scale safety trials before drug or device approval. Therefore, post-market surveillance mechanisms are necessary to detect rare but potentially serious adverse events.

We reviewed the United States Food and Drug Administration’s Manufacturer and User Facility Device Experience (MAUDE) database and conducted a structured literature review to evaluate adverse events associated with on- and off-label Melody® valve implantation. Adverse events were compared with those described in the prospective Investigational Device Exemption and Post-Market Approval Melody® transcatheter pulmonary valve trials.

We identified 631 adverse events associated with “on-label” Melody® valve implants and 84 adverse events associated with “off-label” implants. The most frequent “on-label” adverse events were similar to those described in the prospective trials including stent fracture (n=210) and endocarditis (n=104). Previously unrecognised or under-recognised adverse events included stent fragment embolisation (n=5), device erosion (n=4), immediate post-implant severe valvar insufficiency (n=2), and late coronary compression (n=2 cases at 5 days and 3 months after implantation). Under-recognised adverse events associated with off-label implantation included early valve failure due to insufficiency when implanted in the tricuspid position (n=7) and embolisation with percutaneous implantation in the mitral position (n=5).

Post-market passive surveillance does not demonstrate a high frequency of previously unrecognised serious adverse events with “on-label” Melody® valve implantation. Further study is needed to evaluate safety of “off-label” uses.

Sequential nephron blockade using intravenous chlorothiazide is often used to enhance urine output in patients with inadequate response to loop diuretics. A few data exist to support this practice in critically ill infants.

We included 100 consecutive patients <1 year of age who were administered intravenous chlorothiazide while receiving furosemide therapy in the cardiac ICU in our study. The primary end point was change in urine output 24 hours after chlorothiazide administration, and patients were considered to be responders if an increase in urine output of 0.5 ml/kg/hour was documented. Data on demographic, clinical, fluid intake/output, and furosemide and chlorothiazide dosing were collected. Multivariable regression analyses were performed to determine variables significant for increase in urine output after chlorothiazide administration.

The study population was 48% male, with a mean weight of 4.9±1.8 kg, and 69% had undergone previous cardiovascular surgery. Intravenous chlorothiazide was initiated at 89 days (interquartile range 20–127 days) of life at a dose of 4.6±2.7 mg/kg/day (maximum 12 mg/kg/day). Baseline estimated creatinine clearance was 83±42 ml/minute/1.73 m2. Furosemide dose before chlorothiazide administration was 2.8±1.4 mg/kg/day and 3.3±1.5 mg/kg/day after administration. A total of 43% of patients were categorised as responders, and increase in furosemide dose was the only variable significant for increase in urine output on multivariable analysis (p<0.05). No graphical trends were noted for change in urine output and dose of chlorothiazide.

Sequential nephron blockade with intravenous chlorothiazide was not consistently associated with improved urine output in critically ill infants.

Most patients born with CHD nowadays reach adulthood, and thus quality of life, life situation, and state of medical care aspects are gaining importance in the current era. The present study aimed to investigate whether patients’ assessment depends on their means of occupation. The findings are expected to be helpful in optimising care and for developing individual treatment plans.

The present study was based on an online survey conducted in cooperation with patient organisations. Participants were recruited from the database of the German National Register for Congenital Heart Defects. In total, 1828 individuals (777 males, 1051 females) took part. Participants were asked to rate aspects such their state of health on a six-tier scale (1=worst specification). Response behaviour was measured against the background of occupational details.

Training for or pursuing a profession was found to be significantly associated with participants’ rating of five of the six examined aspects (p<0.05). Sex seemed to play an important part in four of the six aspects.

An optimal treatment plan for adults with CHD should always consider aspects such as sex and employment status. To work out such an optimal and individual treatment plan for each adult CHD patient, an objective tool to measure patients’ actual CHD-specific knowledge precluding socially accepted response bias would be very useful.

The goal of the present prospective study was to assess health-related quality of life in children and adolescents with CHD before and after cardiac catheter treatment.

The study enrolled 96 children/adolescents with CHD from the north-east of China who underwent cardiac catheter intervention treatment between March, 2013 and January, 2014. The health-related quality of life at 24 hours before treatment, 1 month after treatment, and 6 months after treatment was evaluated using Pediatric Quality of Life Inventory Measurement Models 4.0 (PedsQLTM 4.0) generic core scales, and the scores were further compared.

Before treatment, each HRQOL domain score and the total score were obviously decreased than the post-treatment scores (1 month and 6 months). The total score and the scores in physical functioning and psychological functioning components were further increased 6 months after treatment than the scores 1 month after treatment.

The present study suggests that cardiac catheter interventional treatment improves the life quality of children or adolescents with CHD as time increases after the intervention.

Echocardiographic screening for rheumatic heart disease in asymptomatic children may result in early diagnosis and prevent progression. Physician-led screening is not feasible in Malawi. Task shifting to mid-level providers such as clinical officers may enable more widespread screening.

With short-course training, clinical officers can accurately screen for rheumatic heart disease using focussed echocardiography.

A total of eight clinical officers completed three half-days of didactics and 2 days of hands-on echocardiography training. Clinical officers were evaluated by performing screening echocardiograms on 20 children with known rheumatic heart disease status. They indicated whether children should be referred for follow-up. Referral was indicated if mitral regurgitation measured more than 1.5 cm or there was any measurable aortic regurgitation. The κ statistic was calculated to measure referral agreement with a paediatric cardiologist. Sensitivity and specificity were estimated using a generalised linear mixed model, and were calculated on the basis of World Heart Federation diagnostic criteria.

The mean κ statistic comparing clinical officer referrals with the paediatric cardiologist was 0.72 (95% confidence interval: 0.62, 0.82). The κ value ranged from a minimum of 0.57 to a maximum of 0.90. For rheumatic heart disease diagnosis, sensitivity was 0.91 (95% confidence interval: 0.86, 0.95) and specificity was 0.65 (95% confidence interval: 0.57, 0.72).

There was substantial agreement between clinical officers and paediatric cardiologists on whether to refer. Clinical officers had a high sensitivity in detecting rheumatic heart disease. With short-course training, clinical officer-led echo screening for rheumatic heart disease is a viable alternative to physician-led screening in resource-limited settings.

Paediatricians with Expertise in Cardiology assess children with a full history, examination, and often perform an echocardiogram. A minority are then referred to an outreach clinic run jointly with a visiting paediatric cardiologist. The accuracy of the echocardiography diagnosis made by the Paediatrician with Expertise in Cardiology is unknown.

We conducted a retrospective review of clinic letters for children seen in the outreach clinic for the first time between March, 2004 and March, 2011. Children with CHD diagnosed antenatally or elsewhere were excluded. We recorded the echocardiography diagnosis made by the paediatric cardiologist and previously by the Paediatrician with Expertise in Cardiology.

The Paediatrician with Expertise in Cardiology referred 317/3145 (10%) children seen in the local cardiac clinics to the outreach clinic over this period, and among them 296 were eligible for inclusion. Their median age was 1.5 years (range 1 month–15.1 years). For 244 (82%) children, there was complete diagnostic agreement between the Paediatrician with Expertise in Cardiology and the paediatric cardiologist. For 29 (10%) children, the main diagnosis was identical with additional findings made by the paediatric cardiologist. The abnormality had resolved in 17 (6%) cases by the time of clinic attendance. In six (2%) patients, the paediatric cardiologist made a different diagnosis. In total, 138 (47%) patients underwent a surgical or catheter intervention.

Paediatricians with Expertise in Cardiology can make accurate diagnoses of CHD in children referred to their clinics. This can allow effective triage of children attending the outreach clinic, making best use of limited specialist resources.

The aim of the present study was to explore and compare the association between a new vasoactive score – the Total Inotrope Exposure Score – and outcome and the established Vasoactive Inotrope Score in children undergoing cardiac surgery with cardiopulmonary bypass

The present study was a single-centre, retrospective study.

The study was carried out at a 21-bed cardiovascular ICU in a Tertiary Children’s Hospital between September, 2010 and May, 2011

The Total Inotrope Exposure Score is a new vasoactive score that brings together cumulative vasoactive drug exposure and incorporates dose adjustments over time. The performance of these scores – average, maximum Vasoactive Inotrope Score at 24 and 48 hours, and Total Inotrope Exposure Score – to predict primary clinical outcomes – either death, cardiopulmonary resuscitation, or extra-corporeal membrane oxygenation before hospital discharge – and secondary outcomes – length of invasive mechanical ventilation, length of ICU stay, and hospital stay – was calculated.

The study cohort included 167 children under 18 years of age, with 37 (22.2%) neonates and 65 (41.3%) infants aged between 1 month and 1 year. The Total Inotrope Exposure Score best predicted the primary outcome (six of 167 cases) with an unadjusted odds ratio for a poor outcome of 42 (4.8, 369.6). Although the area under curve was higher than other scores, this difference did not reach statistical significance. The Total Inotrope Exposure Score best predicted prolonged invasive mechanical ventilation, length of ICU stay, and hospital stay as compared with the other scores.

The Total Inotrope Exposure Score appears to have a good association with poor postoperative outcomes and warrants prospective validation across larger numbers of patients across institutions.

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.

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.

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).

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.

This study aimed to determine fetal echocardiographic features of tetralogy of Fallot in association with postnatal outcomes.

The Z-scores of the main and bilateral pulmonary arteries and the aorta were measured, and the following variables were calculated in 13 fetuses with tetralogy of Fallot: pulmonary artery-to-aorta ratio and main pulmonary artery cross-section ratio – the main pulmonary artery diameter squared divided by the sum of the diameter squared of the left and right pulmonary arteries. Fetuses were classified as having ductus arteriosus-dependent or ductus arteriosus-independent pulmonary circulation.

We included two infants with pulmonary atresia and six infants with ductus-dependent pulmonary circulation, who underwent systemic-to-pulmonary shunt surgeries at ⩽1 month of age. The Z-scores of the main pulmonary artery and the pulmonary artery-to-aorta ratio in fetuses with ductus-dependent pulmonary circulation were lesser than those in fetuses with ductus independence, but not significantly. The main pulmonary artery cross-section ratio in fetuses with ductus dependence was significantly lesser (0.65±0.44 versus 1.56±0.48, p<0.005). Besides, the flow of the ductus arteriosus was directed from the aorta to the pulmonary artery in the ductus arteriosus-dependent group during the fetal period.

The main pulmonary artery cross-section ratio was the most significant variable for predicting postnatal outcomes in fetuses with tetralogy of Fallot.

The postnatal period in preterm infants involves multiple physiological changes occurring immediately after birth and continuing for days or weeks. To recognise and treat compromise, it is important to measure cardiovascular function. The aim of this study was to describe longitudinal left ventricular function using conventional and novel echocardiography techniques in preterm infants who did not experience significant antenatal or postnatal complications and treatments.

We prospectively obtained cardiac ultrasound images at days 3, 7, 14, 21, and 28 in 25 uncomplicated, preterm infants <30 weeks of gestation. Speckle tracking analysis of the four chambers and short-axis images provided parameters of left ventricular volume, deformation, and basal myocardial velocities. The patent ductus arteriosus, cardiac dimensions, and atrial volume were also measured.

Stroke volume increased by 24% during the study period (1.05–1.30 ml/kg, p<0.05). Cardiac length, diameter, and systolic basal myocardial velocity increased with unchanged wall stress and deformation parameters. Diastolic function parameters resembled that of the fetus with predominance of atrial contraction compared with early diastolic velocities. Blood pressure and estimates of left ventricular filing pressure increased, suggesting that left ventricular compliance did not change in this period.

Stroke volume increased in the first 28 days after preterm birth. The preterm heart adapted by increasing its size, while maintaining systolic and atrial function, independent of early diastolic maturation. Longitudinal deformation of the left ventricle remained unchanged, suggesting relatively preserved function with maturation.

CHD is defined as structural defect(s) in the heart and proximal blood vessels present at birth. The National Cardiothoracic Center of Excellence, University of Nigeria Teaching Hospital (UNTH), Enugu, through the aid of visiting Cardiac Missions has managed a significant number of patients within the last 3.5 years.

The objective of this study was to review surgical options and outcome of complex CHD among patients attending UNTH, Enugu, Enugu.

During the period of 3.5 years (March, 2013 to June, 2016), a total of 20 cases of complex CHD were managed by cardiac missions that visited UNTH, Enugu. Their case notes and operating register were retrieved, reviewed, and analysed using SPSS version 19 (Chicago).

There were eight females and 12 males, with a ratio of 2:5. The age range was from 5 months to 34 years with a mean of 1.7. Among all, five patients died giving a mortality rate of 25%. The operative procedures ranged from palliative shunts to complete repair. The outcome was relatively good.

Complex CHD are present in our environment. Their surgical management in our centre is being made possible by periodic visits of foreign cardiac missions.