Growth failure is prevalent among infants with CHD. A Standardized Clinical Assessment and Management Plan was introduced at Boston Children’s Hospital’s cardiac medical ward to identify patients with growth failure, evaluate relevant contributing conditions, and recommend a management plan including collaboration with nutrition physicians.

The objective of this study was to determine whether enrolled patients had improved growth compared with historical controls.

A total of 29 patients were enrolled in the period July, 2013–June, 2014. In all, 42 historical controls who met eligibility criteria for enrolment were selected for comparison from patients admitted to the same ward in the period June, 2010–June, 2011. Patients with CHD aged <1 year , with growth failure defined as weight-for-age z-score <−2, or failure to sustain adequate weight gain were eligible for participation. Primary outcome was change in weight-for-age z-score from enrolment to most recent weight measurement among patients with at least 6 months of follow-up.

Control patients were older at baseline admission weight (118 versus 95 days, p=0.33), and had a higher weight-for-age z-score, −2.9 (−3.1, −2.6) versus −3.7 (−4.3, −3.0) (p=0.02), compared with enrolled patients. Enrolled patients had greater gain in weight-for-age z-score, 2.7 (2.0, 3.4) versus 1.8 (1.5, 2.2) (p=0.03), from baseline to most recent follow-up.

Patients enrolled in a nutrition-focused protocol had greater weight improvement than historical controls. Identification of growth failure and collaboration with a nutrition support team was associated with improved weight gain among CHD patients experiencing growth failure. CHD programmes should consider a structural approach, including nutrition expertise to address growth failure.

Anecdotal reports suggest that children and young adults with CHD frequently experience pain in their legs. The purpose of this pilot study, performed by Little Hearts Matter patient organisation, was to assess the burden of leg pains in this group and begin to investigate associated factors and consequences for daily living.

An internet-based survey was distributed by Little Hearts Matter patient organisation. After anonymisation and collation, responses were analysed and compared with their healthy siblings.

Of the 220 patients who responded, 94% reported leg pains compared with 30% of siblings (n=107; p<0.001). In respondents, pain was typically reported to occur in the lower legs or around the knees or ankles, often associated with crying and screaming (49.0%) and most commonly occurring at night-time (82.0%). Individuals taking aspirin and those who were more active were more likely to report leg pains. Older age was associated with leg pain that occurred with stress (p=0.02) and at night (p=0.05). Analgesia (64.1%) or massage (53.9%) was the preferred option for alleviation. There was no gender bias, association with diagnosis, surgical history, and/ or relationship with diagnosed orthopaedic issues.

Leg pains are more frequent in those with CHD compared with their healthy siblings. Aetiology is uncertain, but pains share many common characteristics with benign “growing pains”.

Infantile haemangioma is one of the most commonly known benign vascular tumours of infancy and childhood, having an incidence of 3–10%. Most lesions regress spontaneously; however, some may require treatment owing to their clinical and cosmetic effects. Propranolol has become the treatment of choice for infantile haemangioma, but treatment protocols are largely institutional based without any specific consensus guidelines. Our aim was to evaluate the cost-effectiveness of propranolol use as inpatient versus outpatient therapy.

A decision tree model was created depicting alternate strategies for initiating propranolol treatment on an inpatient versus outpatient basis combined with the option of a pretreatment echocardiogram applied to both strategies. Cost analysis was assumed to be based on treatment of haemangioma in patients who were born at term, had no chronic illnesses, a non-life-threatening location of the haemangioma, and those who were not taking any other medications that could potentiate the side effects of propranolol. A sensitivity analysis was performed to evaluate the probability of side effects.

The average cost incurred for inpatient treatment of infantile haemangioma was approximately $2603 for a single hospital day and increased to $2843 with the addition of an echocardiogram. The expected cost of treatment in the outpatient setting was $138, which increased to $828 after the addition of an echocardiogram.

Treating infantile haemangioma with propranolol is more cost-effective when initiated on an outpatient basis.

The Single Ventricle Reconstruction trial randomised neonates with hypoplastic left heart syndrome to a systemic-to-pulmonary-artery shunt strategy. Patients received care according to usual institutional practice. We analysed practice variation at the Stage II surgery to attempt to identify areas for decreased variation and process control improvement.

Prospectively collected data were available in the Single Ventricle Reconstruction public-use database. Practice variation across 14 centres was described for 397 patients who underwent Stage II surgery. Data are centre-level specific and reported as interquartile ranges across all centres, unless otherwise specified.

Preoperative Stage II median age and weight across centres were 5.4 months (interquartile range 4.9–5.7) and 5.7 kg (5.5–6.1), with 70% performed electively. Most patients had pre-Stage-II cardiac catheterisation (98.5–100%). Digoxin was used by 11/14 centres in 25% of patients (23–31%), and 81% had some oral feeds (68–84%). The majority of the centres (86%) performed a bidirectional Glenn versus hemi-Fontan. Median cardiopulmonary bypass time was 96 minutes (75–113). In aggregate, 26% of patients had deep hypothermic circulatory arrest >10 minutes. In 13/14 centres using deep hypothermic circulatory arrest, 12.5% of patients exceeded 10 minutes (8–32%). Seven centres extubated 5% of patients (2–40) in the operating room. Postoperatively, ICU length of stay was 4.8 days (4.0–5.3) and total length of stay was 7.5 days (6–10).

In the Single Ventricle Reconstruction Trial, practice varied widely among centres for nearly all perioperative factors surrounding Stage II. Further analysis may facilitate establishing best practices by identifying the impact of practice variation.

Static balloon atrial septostomy is a widely accepted intervention for children with CHD. Successful surgical palliation is creating increasing numbers of adult CHD patients who need subsequent left heart intervention requiring transseptal access. In these patients, the interatrial septum is usually thick and fibrotic because of a previous open heart surgery or catheter intervention, and conventional transseptal puncture may be unsuccessful. Static balloon atrial septostomy to access the left atrium may facilitate intervention via the interatrial septum in such situations. The purpose of this study was to investigate the usefulness and the safety of static balloon atrial septostomy, and the evolution of an iatrogenic atrial septal defect post procedure in adult CHD.

We retrospectively reviewed six procedures in five adults with CHD and collected demographic characteristics, details of the procedures, clinical outcome, and size changes of the iatrogenic atrial septal defect.

The mean age at the time of the procedure was 35 years. The intended primary interventions were pulmonary vein isolation, stenting for pulmonary vein obstruction, and catheter ablation for focal atrial tachycardia. All static balloon atrial septostomies were effective, and the left heart interventions were successfully achieved via transseptal sheaths. There were no major complications associated with the static balloon atrial septostomy. There were no adverse clinical outcomes related to iatrogenic atrial septal defect, and the size of the defects regressed over time in all cases.

Static balloon atrial septostomy can be a safe and useful technique in adult CHD patients needing left heart procedures. The thick interatrial septum found in postoperative patients may reduce the risk of persistent iatrogenic atrial septal defect.

The outcome of transcatheter closure in ostium secundum atrial septal defects is determined by the morphology of the defects. Modified techniques such as balloon assistance, pulmonary vein deployment, left atrial roof technique, and so on are used for circumventing the anatomic complexities and increasing the success rates.

We planned a prospective study looking at the outcomes of transcatheter closure in secundum atrial septal defects with modified techniques in different anatomic complexities identified in transoesophageal echocardiography and their association with outcome of transcatheter closure.

Transcatheter closure was successful in 295 out of 346 (82%) patients with modified techniques. Balloon-assisted technique offered a success rate of 87%. The mean defect size was 34.7±2.78 mm (95% confidence interval (CI) 30.67–43.1 mm) with success and 40.16±4.5 mm (95% CI 32.16–44.7) with failure (p = 0.02). The mean total septal length was 38.11±0.63 (95% CI 35.21–40.56 mm) with success and 42.54±0.34 (95% CI 38.79–43.21 mm) with failure. The defect to septal ratios were 0.82 and 0.94 in success and failure groups, respectively (p=0.02). However, the absence of a retro-aortic margin, septal aneurysm, and multiple defects did not affect the success rate. Deficient inferior vena caval margin, deficient posterior margin, and size⩾40 mm had a high risk of failure with transcatheter closure. The odds ratio for procedural failure was 25.3 (4.3–143.8) in patients with malaligned septum, 8.3(1.4–48.5) with deficient inferior vena caval margin, and 4.1(2.5–19) for size⩾40 mm.

The modified techniques for device deployment offer substantial chances of success in transcatheter closure of secundum atrial septal defects with anatomical complexity (82%). Variants such as defect size of⩾40 mm and deficient inferior and posterior margins have high failure rates with a modified technique.

There is no standard dose or protocol for beta-blocker administration as preconditioning in children undergoing coronary CT angiography.

A total of 63 consecutive patients, with a mean age of 10.0±3.1 years, who underwent coronary CT angiography to assess possible coronary complications were enrolled in a single-centre, retrospective study. All patients were given an oral beta-blocker 1 hour before coronary CT angiography. Additional oral beta-blocker or intravenous beta-blocker was given to those with a high heart rate. We compared image quality, radiation exposure, and adverse events among the patients without additional beta-blocker, with additional oral beta-blocker, and with additional intravenous beta-blocker.

There were no significant differences in image quality or radiation exposure among the groups. The heart rate just before scanning was significantly correlated with image quality (p<0.001, r=−0.533) but was not correlated with radiation exposure (p=0.45, r=0.096). There were no adverse events related to any allergic reaction, thereby showing the effectiveness of the beta-blocker.

Initial oral beta-blocker administration (0.8 mg/kg/dose) should be administered to all children undergoing coronary CT angiography. Additional intravenous beta-blocker should be given to those with poor heart rate control to improve image quality without increasing radiation exposure or allowing adverse events.