Children with congenital heart disease are at high risk for malnutrition. Standardisation of feeding protocols has shown promise in decreasing some of this risk. With little standardisation between institutions’ feeding protocols and no understanding of protocol adherence, it is important to analyse the efficacy of individual aspects of the protocols.

Adherence to and deviation from a feeding protocol in high-risk congenital heart disease patients between December 2015 and March 2017 were analysed. Associations between adherence to and deviation from the protocol and clinical outcomes were also assessed. The primary outcome was change in weight-for-age z score between time intervals.

Increased adherence to and decreased deviation from individual instructions of a feeding protocol improves patients change in weight-for-age z score between birth and hospital discharge (p = 0.031). Secondary outcomes such as markers of clinical severity and nutritional delivery were not statistically different between groups with high or low adherence or deviation rates.

High-risk feeding protocol adherence and fewer deviations are associated with weight gain independent of their influence on nutritional delivery and caloric intake. Future studies assessing the efficacy of feeding protocols should include the measures of adherence and deviations that are not merely limited to caloric delivery and illness severity.

Pain control is an important element of care for patients after surgery, leading to better outcomes, quicker transitions to recovery, and improvement in quality of life. The purpose of this study was to evaluate the safety and efficacy of non-steroidal anti-inflammatory drugs in children after cardiac surgery

Patients between the ages of 1 month and 18 years of age, who received intravenous or oral non-steroidal anti-inflammataory drugs after cardiac surgery, from November 2015 until September 2017 were included in this study. The primary endpoints were non-steroidal anti-inflammataory drug-associated renal dysfunction and post-operative bleeding. Secondary endpoints examined the effect of non-steroidal anti-inflammataory drug use on total daily dose of narcotics, number of intravenous PRN narcotic doses received, and pain assessment score. Data were analysed using descriptive statistics for frequencies and ranges. Multivariate analysis was performed to measure the association of all predictors and outcomes. Wilcoxon singed-rank test was performed for secondary outcomes.

There was no association between the incidence of renal dysfunction and the use of or duration of non-steroidal anti-inflammataory drugs; in addition no association was found with increased chest tube output. There was a statistically significant reduction of patients’ median Face, Legs, Activity, Cry, Consolability (FLACC) scores (2–0; p = 0.003), seen within first 24 hours after initiation of ketorolac, and a significant reduction of morphine requirements seen from day 1 to day 2 (0.3 mg/kg versus 0.1 mg/kg; p < 0.001) and number of as-needed doses.

Non-steroidal anti-inflammataory drugs in paediatric cardiac surgery patients are safe and effective for post-operative pain management.

Patients with complex congenital cardiac disease are increasingly surviving to adulthood and many are keen to consider pregnancy. Haemodynamic status should be optimal prior to embarking on pregnancy and for some this may mean surgical intervention to alleviate haemodynamic residua.

We report the successful implantation of a percutaneous pulmonary stent valve into a right atrial to right ventricular conduit in a young woman with a Bjork modification of the Fontan palliation to improve haemodynamics prior pregnancy.

Catheter interventions offer a low-risk option for the treatment of haemodynamic residua and innovative use of new technologies such as the pulmonary stent valve presents a novel, safe, and effective treatment for such conduit problems.

To anglicise an American – that is, English language – disease-specific health-related quality of life measure, using the Paediatric Cardiac Quality of Life Inventory, for children in the age group of 8–12 years and adolescents in the age group of 13–18 years with cardiac disease, and to assess conceptual equivalence of the American and British versions.

A process of forward and backward translation of the measure was undertaken before focus groups and individual interviews with 40 participants – that is, 20 children/adolescents with cardiac disease and 20 parents of children/adolescents with cardiac disease – to determine their understanding of the meaning of the questions.

Interviews established that participants understood the meaning of the questions, although some found it difficult to explain the meaning of questions in which the language was explicit and wanted instead to answer the individual questions as they applied to them/their child. There was agreement that all versions of the questionnaire were relevant and comprehensive, and that the length of the questionnaires was acceptable and practical.

The anglicised version of the Paediatric Cardiac Quality of Life Inventory appears to be a linguistically valid measure of health-related quality of life for children and adolescents with cardiac disease. The psychometric properties of the anglicised Paediatric Cardiac Quality of Life Inventory are now being tested in a multi-centre study in the United Kingdom.

Paediatric cardiomyopathy is a progressive, often lethal disorder and the most common cause of heart failure in children. Despite its severe outcomes, the genetic aetiology is still poorly characterised. High-throughput sequencing offers a great opportunity for a better understanding of the genetic causes of cardiomyopathy.

The current study aimed to elucidate the genetic background of cardiomyopathy in Egyptian children.

This hospital-based study involved 68 patients; 58 idiopathic primary dilated cardiomyopathy and 10 left ventricular noncompaction cardiomyopathy. Cardiomyopathy-associated genes were investigated using targeted next-generation sequencing.

Consanguinity was positive in 53 and 70% of dilated cardiomyopathy and left ventricular noncompaction cardiomyopathy patients, respectively. Positive family history of cardiomyopathy was present in 28% of dilated cardiomyopathy and 10% of the left ventricular noncompaction cardiomyopathy patients. In 25 patients, 29 rare variants were detected; 2 likely pathogenic variants in TNNI3 and TTN and 27 variants of uncertain significance explaining 2.9% of patients.

The low genetic detection rate suggests that novel genes or variants might underlie paediatric cardiomyopathy in Egypt, especially with the high burden of consanguinity. Being the first national and regional report, our study could be a reference for future genetic testing in Egyptian cardiomyopathy children. Genome-wide tests (whole exome/genome sequencing) might be more suitable than the targeted sequencing to investigate the primary cardiomyopathy patients. Molecular characterisation of cardiomyopathies in different ethnicities will allow for global comparative studies that could result in understanding the pathophysiology and heterogeneity of cardiomyopathies.

To quantify myocardial blood flow in infants and children with mild or moderate aortic stenosis using adenosine-infusion cardiac magnetic resonance.

It is unclear whether asymptomatic children with mild/moderate aortic stenosis have myocardial abnormalities. In addition, cardiac magnetic resonance-determined normative myocardial blood flow data in children have not been reported.

We studied 31 infants and children with either haemodynamically normal hearts (n=20, controls) or mild/moderate aortic stenosis (n=11). The left ventricular myocardium was divided into six segments, and the change in average segmental signal intensity during contrast transit was used to quantify absolute flow (ml/g/minute) at rest and during adenosine infusion by deconvolution of the tissue curves with the arterial input of contrast.

In all the cases, adenosine was well tolerated without complications. The mean pressure gradient between the left ventricle and the ascending aorta was higher in the aortic stenosis group compared with controls (24 versus 3 mmHg, p<0.001). Left ventricular wall mass was slightly higher in the aortic stenosis group compared with controls (65 versus 50 g/m2, p<0.05). After adenosine treatment, both the absolute increase in myocardial blood flow (p<0.0001) and the hyperaemic flow significantly decreased (p<0.001) in children with mild/moderate aortic stenosis compared with controls.

Abnormal myocardial blood flow in children with mild/moderate aortic stenosis may be an important therapeutic target.

There are several published echo-derived scores to help predict successful biventricular versus univentricular palliation in neonates with critical aortic stenosis. This study aims to determine whether any published scoring system accurately predicted outcomes in these neonates.

Single centre, retrospective cohort study including neonates who underwent aortic valve intervention (surgical valvotomy or balloon valvuloplasty) with the intention of biventricular circulation. Primary outcome was survival with biventricular circulation at hospital discharge. Data from their initial neonatal echocardiogram were used to compute the following scores – Rhodes, CHSS 1, Discriminant, CHSS 2, and 2 V.

Between 01/1999 and 12/2017, 68 neonates underwent aortic valve intervention at a median age of 4 days (range 1–29 days); 35 surgical valvotomy and 33 balloon valvuloplasty. Survival with biventricular circulation was maintained in 60/68 patients at hospital discharge. Of the remaining eight patients, three were converted to univentricular palliation, four died, and one underwent heart transplant prior to discharge. None of the binary score predictions of biventricular versus univentricular (using that score’s proposed cut-offs) were significantly associated with the observed outcome in this cohort. A high percentage of those predicted to need univentricular palliation had successful biventricular repair: 89.4% by Rhodes, 79.3% by CHSS 1, 85.2% by Discriminant, and 66.7% by CHSS 2 score. The 2 V best predicted outcome and agreed with the local approach in most cases.

This study highlights the limitations of and need for alternative scoring systems/cut-offs for consistently accurate echocardiographic prediction of early outcome in neonates with critical aortic stenosis.

Certain congenital cardiac defects may go undetected for several years due to lack of symptoms and signs. Our purpose was to determine the prevalence of such defects among children greater than one year of age.

The study was performed on subjects diagnosed with systemic hypertension, aged from 1 to 19 years, with a mean of 12.4 years, in whom we performed echocardiography, using a standard protocol, to establish any end-organ damage or to reveal any congenital cardiac defects.

We found a congenital cardiac defect in 5 (3.5%) of the 143 children evaluated. Of these, 4 had not previously been detected, specifically Ebsteins malformation of the tricuspid valve, with moderate regurgitation, a coronary arterial anomaly, a bicuspid aortic valve, and prolapse of the mitral valve permitting regurgitation. In the other patient, we found a non-significant tiny muscular ventricular septal defect.

Our transthoracic echocardiographic investigation revealed previously unsuspected congenital cardiac defects in 4 of 143 older children, with 3 of these requiring further management by a paediatric cardiologist. A similar prevalence has also been reported in older children evaluated echocardiographically for other diseases such as insulin-resistance and leukemia. Hence, it is possible that the prevalence of congenitally malformed hearts is higher than previously reported. When clinically indicated, clinicians should more readily consider obtaining an echocardiogram to help in the identification of such malformations.