Tetralogy of Fallot with absent pulmonary valve syndrome is a rare form of tetralogy of Fallot with dilatation of large pulmonary arteries. Prognosis is related to the severity of the cardiac malformation and to bronchial tree compression by dilated pulmonary arteries. This study analyses the prenatal echographic lung appearance in fetuses with tetralogy of Fallot with absent pulmonary valve and discusses its significance.

We carried out a retrospective review of fetal and postnatal files of nine fetuses diagnosed with tetralogy of Fallot with absent pulmonary valve syndrome in our institution. Correlations of prenatal ultrasound and cardiac imaging findings were obtained with outcome.

Abnormal heterogeneous fetal lung echogenicity was detected in eight cases out of nine, always associated with significant lobar arterial dilatation. This aspect was well correlated with postnatal imaging and outcome in the four neonatal cases. The only fetus with normal lung echogenicity also had lower degree of pulmonary artery dilatation in the series.

This study demonstrates that a heterogeneous ultrasound appearance of the fetal lungs can be detected in utero in the most severe cases. This aspect suggests an already significant compression of the fetal bronchial tree by the dilated arteries that may have prognostic implications.

“Athlete’s heart” is a cardiac adaptation to long-term intensive training. The aims of this study were to show the prevalence of left ventricular hypertrophy in teenagers who participate in sports, to define the different types of cardiac re-modelling, and to differentiate between physiological and pathological hypertrophy.

Echocardiographic measurements were obtained by M-mode, two dimensional, and Doppler techniques of participants from sports and control groups.

The echocardiographic examinations included 100 healthy teenagers taking part in dynamic sports such as football and basketball and 100 healthy teenagers taking part in static sports such as karate and judo. The control group (n=100) included healthy, sedentary teenagers. Sports participants had significantly higher left ventricular mass when compared with the control group, (p<0.001). In the dynamic sports group, 29% of the respondents had left ventricular mass above the 95th percentile, whereas 71% had left ventricular mass below the 95th percentile (p<0.001). The cardiac re-modelling was eccentric (79.4 versus 20.6%, p<0.001). In the group of static sports participants, 37% had left ventricular mass above the 95th percentile, whereas 63% had left ventricular mass below the 95th percentile (z score 0.74±0.82, p<0.001). The prevalence of concentric and eccentric types of re-modelling was equally manifested (54.05 versus 45.95%, p>0.05). Respondents from both groups had E/A ratios (transmitral flow velocity ratio)>1, preserved diastolic function, and statistically they did not differ from the control group.

Echocardiographic parameters show that physiological hypertrophy and cardiac re-modelling are present in teenagers who play sports. Unexpectedly, the prevalence of concentric and eccentric types of re-modelling is equally possible in the group of static sports participants.

Sickle cell disease is known to cause various degrees of vasculopathy, including impact on heart function. The aims of this single-centre, retrospective study were to assess cardiac chamber size and function and the relationship with haematological indices such as haemoglobin, aspartate aminotransferase, reticulocytosis and bilirubin, lactate dehydrogenase in sickle cell disease.

Right ventricle and left ventricle diastolic diameters, left ventricle mass estimate, left ventricle shortening fraction, myocardial performance index, and an index of myocardial relaxation (E/E’) were calculated and correlated with haematological parameters.

A total of 110 patients (65% haemoglobin SS, 29% haemoglobin SC) were studied at a mean age of 12.14±5.26 years. Right ventricle dilatation and left ventricle dilatation were present in 61.5 and 42.9%, respectively. Left ventricle mass was abnormal in 21.9%; all patients had normal myocardial performance index, 31.4% had abnormal E/E’, and left ventricle shortening fraction was low in 38.1%. Cardiac dilatation was best correlated with haemoglobin, aspartate aminotransferase, reticulocytosis and bilirubin. Best subset regression analysis yielded significant additional prediction for right ventricle or left ventricle dilatation with haemoglobin, bilirubin, and lactate dehydrogenase. Abnormal E/E’ was solely predictable with haemoglobin level. Hydroxyurea-treated patients had improved diastolic function.

Right ventricle dilatation was more prevalent than left ventricle dilatation. The long-term consequences of right ventricular dilatation, clinical consequences, and association with pulmonary vasculopathy need to be further determined.

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.

In CHD, three-dimensional strain analysis may overcome limitations of Doppler and two-dimensional strain of the left ventricle. The aims of this study were to evaluate feasibility and reproducibility of three-dimensional longitudinal, circumferential, and radial systolic strain by three-dimensional speckle-tracking echocardiography compared with two-dimensional echocardiography.

Patients with CHD, biventricular circulation with a systemic left ventricle, and who had two- and three-dimensional imaging performed on the same day from 2010 to 2014 were included. Quantitative two- and three-dimensional strain analyses were performed (two-dimensional cardiac performance analysis version 1.2 and four-dimensional left ventricular analysis version 3.1). Intra- and inter-observer variabilities were calculated on 25 studies.

A total of 30 patients, including 19 (61%) males, with a median age of 3.6 years (0.1–22 years) were included. The mean fractional shortening was 34.6±5.3%, and the mean ejection fraction was 62.0±6.4%. Measurement of two- and three-dimensional strain was feasible in >95% of segments. Good correlation was observed between longitudinal and circumferential strain (r=0.92, p⩽0.001 and r=0.87, p⩽0.001), but not radial strain (r=0.29, p=0.2). Intra- and inter-observer agreements were better for three-dimensional compared with two-dimensional strain, and better for both two- and three-dimensional longitudinal and circumferential strains compared with radial strain.

Left ventricular three-dimensional strain analysis is feasible in children with CHD. The reproducibility of longitudinal and circumferential strain by three-dimensional analyses is better. Further longitudinal studies are warranted for the potential clinical application of this new technology.

The aim of the present study was to determine the role of toxic elements and trace elements in the pathogenesis of conotruncal heart defects by measuring their concentrations in the first meconium specimens of the affected newborns.

Concentrations of lead, cadmium, iron, zinc, and copper were measured in 1st-day meconium specimens that were collected from 60 newborns with conotruncal heart defects (Group I) and 72 healthy newborns (Group II).

The newborns with conotruncal defects and the healthy newborns had statistically similar demographic and clinical characteristics. When compared with healthy newborns, mean concentrations of lead, cadmium, iron, zinc, and copper were significantly higher in newborns with conotruncal heart defects (p=0.001 for each). In total, 51 newborns with conotruncal heart defects had normal karyotype. These newborns had significantly higher concentrations of lead, cadmium, iron, zinc, and copper when compared with healthy newborns. There were significant and positive correlations between the concentrations of lead and cadmium (r=0.618, p=0.001), lead and iron (r=0.368, p=0.001), lead and zinc (r=0.245, p=0.005), lead and copper (r=0.291, p=0.001), cadmium and iron (r=0.485, p=0.001), cadmium and zinc (r=0.386, p=0.001), and cadmium and copper (r=0.329, p=0.001).

Toxic metals and trace elements may disturb DNA repair mechanisms by impairing DNA methylation profiles, and thus have a role in the pathogenesis of conotruncal heart defects.

The aim of this study was to evaluate the anxiety and depression status, family functions, parenting attitudes, and quality of life in the mothers of children with CHD.

The study enrolled 120 mothers: 40 of children with cyanotic CHD, 40 of children with non-cyanotic CHD, and 40 of healthy controls. Short Form-36 for quality of life, Hospital Anxiety–Depression Scale for anxiety and depression, Family Assessment Device for the detection of problems affecting family functions, and Parental Attitude Research Instrument for measuring child-rearing attitudes were used in the study.

Statistically significant decreases were found in the general health standards of mothers of non-cyanotic children (p=0.035) and in the emotional and physical role difficulty of mothers of cyanotic children (p=0.006, p=0.010). When anxiety and depression levels of the parents were examined, the anxiety level of the cyanotic group was found to be significantly higher than that of the other groups (p=0.031). When family behaviours were assessed, there was a statistically significant decrease in role status in the families having a child with cyanotic CHD (p=0.035). In the Parental Attitude Research Instrument test, the husband and wife incompatibility sub-scale was found to be statistically significantly lower in the cyanotic CHD group (p=0.030).

When there is a diseased person in the family, the focus should not be solely on the problems of the patient but also on preventive methods to be implemented in order to protect the mental health of all family members.

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.

Ibuprofen is used widely to close patent ductus arteriosus in preterm infants. The anti-inflammatory activity of ibuprofen may also be partly due to its ability to scavenge reactive oxygen species and reactive nitrogen species. We evaluated the interaction between oxidative status and the medical treatment of patent ductus arteriosus with two forms of ibuprofen.

This study enrolled newborns of gestational age ⩽32 weeks, birth weight ⩽1500 g, and postnatal age 48–96 hours, who received either intravenous or oral ibuprofen to treat patent ductus arteriosus. Venous blood was sampled before ibuprofen treatment from each patient to determine antioxidant and oxidant concentrations. Secondary samples were collected 24 hours after the end of the treatment. Total oxidant status and total antioxidant capacity were measured using Erel’s method.

This prospective randomised study enrolled 102 preterm infants with patent ductus arteriosus. The patent ductus arteriosus closure rate was significantly higher in the oral ibuprofen group (84.6 versus 62%) after the first course of treatment (p=0.011). No significant difference was found between the pre- and post-treatment total oxidant status and total antioxidant capacity in the groups.

Ibuprofen treatment does not change the total oxidant status or total antioxidant capacity. We believe that the effect of ibuprofen treatment in inducing ischaemia overcomes the scavenging effect of ibuprofen.

This study aimed to (a) examine eHealth literacy, beliefs, and behaviours in parents of children with complex CHD, and (b) identify parents’ preferences for the content, format, features, and functions of eHealth resources for CHD.

Families (n=198) of children born between 2008 and 2011 and diagnosed with CHD requiring surgery were mailed a survey assessing a range of variables including eHealth literacy, beliefs, and behaviours as well as preferences for the format, functions, features, and content of eHealth resources for CHD.

A total of 132 parents (83 mothers, 49 fathers) completed the survey (response rate: 50%). Mothers (96%) were more likely to access eHealth resources than fathers (83%, χ2=6.74, p=0.009). Despite high eHealth resource use, eHealth literacy was relatively low, with results demonstrating considerable and widespread gaps in awareness of, access to, and communication about eHealth resources. Over 50% of parents reported that decisions regarding their child’s healthcare were influenced, to some extent, by web-based resources. Barriers to doctor–patient communication about eHealth included limited consultation time and concern about doctors’ disapproval. Participants demonstrated a strong desire for “eHealth prescriptions” from their child’s healthcare team, and perceived a wide range of eHealth topics as highly important, including treatment-related complications as well as physical, cognitive, and emotional development in children with CHD.

Results suggest a need for stronger, more proactive partnerships between clinicians, researchers, educators, technologists, and patients and families to bring about meaningful innovations in the development and implementation of eHealth interventions in paediatric cardiology.

Epidemiological patterns of cardiac disease differ between developed countries and African nations. Despite the collaborative efforts of developed countries, several obstacles hinder the implementation of successful programmes for the management of children with heart disease in Africa.

This study is a retrospective analysis of a bi-national two-institution partnership programme for the treatment of children with congenital and acquired heart disease. In April, 2011, a continuous medical-surgical programme was inaugurated at Clínica Girassol in Luanda. The main goals were to initiate permanent and local delivery of services while training local teams, allowing autonomous medical and surgical management of children with heart disease.

Between April, 2011 and August, 2015, a total of 1766 procedures were performed on 1682 children. Of them, 1539 had CHD and 143 had acquired heart disease; 94 children underwent interventional treatment. A total of 1672 paediatric surgeries were performed on 1588 children: 1087 (65%) were performed with extracorporeal circulation and 585 (35%) were off-pump. The age distribution of the children was 4.5% (n=76) neonatal, 40.4% (n=675) between 30 days and 1 year, and 55.1% (n=921) over 1 year. There were 76 re-operations (4.5%) due to complications. The 30-day mortality rate was 4.2% (71 patients). Education-wise, several Angolan medical and surgical specialists were trained, allowing near-autonomous cardiac care delivery in children with heart disease.

An innovative cooperation model between a European and an African centre based on permanent delivery of care and education allowed for effective training of local teams and treatment of children with heart disease in their own environment.

We carried out this study in order to evaluate the causes of chest pain in teenagers and the role of anxiety and depression in this age group compared with the normal population.

In this prospective case–control study, all patients aged 11–18 years with chest pain and no history of trauma and referred to a paediatric cardiology clinic from March, 2009–April, 2010 were selected. A chest pain protocol including a detailed history, full physical examination, required blood tests, electrocardiography, and echocardiography was performed for all. The presence of depression and anxiety and their severity were assessed by Beck questionnaires. The patients were compared with age- and sex-matched, randomly selected healthy controls.

In total, 194 patients with a mean age of 14±2 years were selected. The most frequent presentation was idiopathic chest pain (43.3%), followed by the psychological group (29.9%). These groups had no abnormal points in history, physical, and para-clinical tests. Moderate-to-severe depression was found in 45.9% in the patients group, compared with 17.6% of controls, which was statistically significant (p=0.016). Moreover, anxiety was detected in 67.5% of patients versus 15.4% in controls, which is a statistically significant difference (p=0.009). Cardiac chest pain with 9.27% was the most common type of organic causes.

Chest pain during teenage is more prevalent, but not risky. Undergoing a detailed history and full physical examination can help diagnose the causes in the majority of cases. Given the prevalence of a psychological group as well as role of anxiety and depression in most patients, referring to a psychiatrist is suggested.