The Pediatric Heart Network designed a career development award to train the next generation of clinician scientists in paediatric-cardiology-related research, a historically underfunded area. We sought to identify the strengths/weaknesses of the programme and describe the scholars’ academic achievements and the network’s return on investment.

Survey questions designed to evaluate the programme were sent to applicants – 13 funded and 19 unfunded applicants – and 20 mentors and/or principal investigators. Response distributions were calculated. χ2 tests of association assessed differences in ratings of the application/selection processes among funded scholars, unfunded applicants, and mentors/principal investigators. Scholars reported post-funding academic achievements.

Survey response rates were 88% for applicants and 100% for mentor/principal investigators. Clarity and fairness of the review were rated as “clear/fair” or “very clear/very fair” by 98% of respondents, but the responses varied among funded scholars, unfunded applicants, and mentors/principal investigators (clarity χ2=10.85, p=0.03; fairness χ2=16.97, p=0.002). Nearly half of the unfunded applicants rated feedback as “not useful” (47%). “Expanding their collaborative network” and “increasing publication potential” were the highest-rated benefits for scholars. Mentors/principal investigators found the programme “very” valuable for the scholars (100%) and the network (75%). The 13 scholars were first/senior authors for 97 abstracts and 109 manuscripts, served on 22 Pediatric Heart Network committees, and were awarded $9,673,660 in subsequent extramural funding for a return of ~$10 for every scholar dollar spent.

Overall, patient satisfaction with the Scholar Award was high and scholars met many academic markers of success. Despite this, programme challenges were identified and improvement strategies were developed.

Interrupted aortic arch is often associated with subaortic narrowing and hypoplasia of the aortic orifice. The best surgical strategy for the management of these additional lesions is a matter of current debate.

Between 1986 and 1996, 19 patients underwent repair of interrupted aortic arch with closure of ventricular septal defect in a single stage, with no attempt at subaortic resection, irrespective of the dimensions of the left ventricular outflow tract. There was no perioperative hospital mortality, and all patients were alive at 1 year. Follow-up ranges from 0.75 −10 years, with a mean 4.2 ± 3.0 years. Seven patients (37%) have required reintervention for relief of subaortic stenosis, 2 of whom have died.

Our results suggest that primary one-stage biventricular repair can be accomplished with low perioperative mortality without addressing the subaortic region. Further long-term follow-up will determine whether this is accomplished at the expense of later morbidity and mortality.

The most serious manifestation of rheumatic fever is carditis, as it can lead to chronic rheumatic heart disease. Neutrophil-to-lymphocyte ratio, platelet-to lymphocyte ratio and mean platelet volume have been accepted as novel indicators of the continuing inflammation that is correlated with the severity of valvular involvement in patients with rheumatic carditis.

As a diagnostic study to investigate the correlation of neutrophil-to-lymphocyte ratio, platelet-to-lymphocyte ratio and mean platelet volume with the severity of carditis in children with rheumatic fever.

This was a cross-sectional study at Sanglah Hospital, Bali. Data were collected retrospectively from medical records. Severity of carditis was characterised by a clinical, audible murmur during physical examination and demonstration of valvular involvement by echocardiography. Neutrophil-to-lymphocyte ratio, platelet-to-lymphocyte ratio and mean platelet volume were calculated from complete blood count. The correlation between parameters was assessed using Spearman’s correlation tests.

From January 2018 to December 2020, we found 45 cases admitted to Sanglah Hospital. Carditis was present in 40 (88.9%) subjects. Neutrophil-to-lymphocyte ratio had a strong positive correlation with severity of carditis (r = 0.66, p = 0.001), while platelet-to-lymphocyte ratio had a weak positive correlation with the severity of carditis (r = 0.23, p = 0.13) and mean platelet volume had a very weak negative correlation with the severity of carditis (r = −0.01, p = 0.95). Using a cut-off of 3.4, neutrophil-to-lymphocyte ratio predicted severe carditis with a sensitivity of 81.8% and specificity of 91.3%.

There was a positive correlation between neutrophil-to-lymphocyte ratio and severity of carditis in children with rheumatic fever. Neutrophil-to-lymphocyte ratio might be used as a diagnostic tool to predict the diagnosis of carditis severity.

Thrombocytopaenia is common in adults with cyanotic heart disease. Our aim was to explore potential mechanisms for thrombocytopaenia in these vulnerable patients.

Adults with cyanotic heart defects were identified from our clinical database. Haemoglobin levels, platelet counts, and resting oxygen saturations were determined at baseline and during follow-up. Associations between patient characteristics and cardiac physiology with these parameters at baseline and during follow-up were analysed using regression models. Survival estimates were determined by the Kaplan–Meier method.

We included 79 patients (mean age 32.2 ± 12.4, 48 (61%) Eisenmenger syndrome, 20 (25%) Down syndrome). Mean oxygen saturation was 84.1 ± 5.9%; 38 (48%) had thrombocytopaenia. There was a strong inverse correlation between platelet count and haemoglobin level (R = −0.655, R2 = 0.429, p < 0.0001) and a weaker but significant positive correlation between platelet count and oxygen saturation (R = 0.345, R2 = 0.119, p = 0.002). There was a significant inverse correlation between decrease in platelet count and increase in haemoglobin level during follow-up (R = −0.401, R2 = 0.161, p = 0.001) but not to changes in oxygen saturation (R = 0.043, R2 = 0.002, p = 0.726). Survival estimates were lower for patients with thrombocytopaenia at baseline (log-rank test p < 0.0001).

Our findings suggest a direct inverse correlation between platelet counts and haemoglobin levels in adults with cyanotic heart disease. Further studies are required to explore the mechanisms of thrombocytopaenia in cyanotic heart disease and its potential role as an independent marker of risk.

Cyanotic CHD is one of many disorders in paediatrics that influence the health of children in different clinical aspects. One of the fundamental aspects that may be affected is bone mineral density.

The aim of our study is to assess bone mineral density in children with congenital cyanotic heart disease of different anatomical diagnoses.

Cross-sectional, observational study included 39 patients (20 males) with congenital cyanotic heart disease of different anatomical diagnoses following with the cardiology clinic in Mansoura University children’s hospital. All patients were subjected to anthropometric measures, oxygen saturation assessment, and lumber bone mineral density using dual-energy X-ray absorptiometry.

Six patients (15.4%) out of the 39 included patients showed bone mineral density reduction, 13 patients (33.3%) showed bone mineral density with Z-score between −1 and −2, while 20 patients (51.3%) showed bone mineral density with Z-score more than −1.

Low bone mineral density can be found in children with cyanotic CHD, making it important to consider bone mineral density assessment and early treatment if needed to avoid further complications.

In our recent study, we first reported that mutation in vascular endothelial growth factor-A is associated with bicuspid aortic valve stenosis. However, to date no groups have explored the role of vascular endothelial growth factor-A variations in the aetiology of congenital tricuspid aortic valve stenosis.

We sequenced all eight coding exons and exon–intron boundaries of the vascular endothelial growth factor-A gene in deoxyribonucleic acid samples of a cohort of 32 sporadic patients with tricuspid aortic valve stenosis, 300 normal controls, and 103 disease controls – conotruncal defects – in order to identify sequence variants.

We identified a c.973C > T heterozygous nonsense variation in exon 6 of the vascular endothelial growth factor-A gene in a patient with an isolated tricuspid aortic valve stenosis. The c.973C > T variation, which was absent in all controls, changes a highly conserved arginine at amino acid position 325 to a stop codon (p.Arg325X) and is predicted to produce a truncated protein of 324 amino acid residues. The proband's parents had a normal cardiac phenotype; however, his father was a carrier of the p.Arg325X variation, which indicates that the p.Arg325X variation is inherited and incompletely penetrant.

We report for the first time that the p.Arg325X nonsense variation in the vascular endothelial growth factor-A gene may be associated with congenital tricuspid aortic valve stenosis.