In 2001, the Institute of Medicine identified healthcare transparency as a necessity for re-designing a quality healthcare system; however, despite widespread calls for publicly available transparent data, the goal remains elusive. The transparent reporting of outcome data and the results of congenital heart surgery is critical to inform patients and families who have both the wish and the ability to choose where care is provided. Indeed, in an era where data and means of communication of data have never been easier, the paucity of transparent data reporting is paradoxical. We describe the development of a quality dashboard used to inform staff, patients, and families about the outcomes of congenital heart surgery at the Stollery Children’s Hospital.

Objectives: This study sought to evaluate left ventricular, right ventricular, and left atrial mechanics and their interactions in patients with congenital scoliosis without clinical heart failure. Methods: A total of 23 patients with a median age of 14 years and a median Cobb’s angle of 61° were studied. Ventricular and atrial myocardial deformation was measured using speckle tracking echocardiography. The results of the patients were compared with 22 controls. Results: Compared with controls, the patients had a significantly greater annular a velocity (p=0.04) and lower e/a ratio (p=0.03); the left ventricular deformation significantly decreased in radial global (p=0.04) and segmental systolic strain and early diastolic strain rate (p=0.03); the left atrial deformation showed a significantly lower positive strain (p=0.02), greater negative strain (p=0.01), and active contractile strain rate (p=0.01). For the patients, the Cobb’s angle was negatively correlated with the left ventricular global radial systolic strain (r=−0.65, p=0.001), left atrial positive strain (r=−0.68, p<0.001), and the left atrial negative strain was positively correlated with the left ventricular circumferential late diastolic strain rate (r=0.46, p=0.01). The left atrial conduit strain rate was positively correlated with the left ventricular circumferential early diastolic strain rate (r=0.42, p=0.03). The left atrial active contractile strain rate was positively correlated with the left ventricular longitudinal late diastolic strain rate (r=−0.4, p=0.03). Conclusions: Impaired left ventricular and altered left atrial mechanics occur relatively early in patients with congenital scoliosis, and are correlated with the severity of their scoliosis. Our findings provide evidence of preclinical heart dysfunction in patients with this disorder.

An indexed offset distance of the tricuspid septal leaflet ⩾8 mm/m2 is a quantitative criterion for the diagnosis of Ebstein’s anomaly. The purpose of this study was to investigate the validity of this criterion for the discrimination of Ebstein’s anomaly from pulmonary atresia with intact ventricular septum in neonatal patients. A total of 122 neonatal patients, 56 with Ebstein’s anomaly and 66 with pulmonary atresia with intact ventricular septum, were enrolled. Diagnosis of each anomaly was based on typical morphologic features. Echocardiographic variables, including the offset distance of the tricuspid septal leaflet, were measured via an offline analysis of images recorded before 1 month of age. The offset distance of the tricuspid septal leaflet was indexed by the body surface area, and the indexed offset distances in the Ebstein’s anomaly and pulmonary atresia with intact ventricular septum groups were 34.2 mm/m2 (7.1–119.1 mm/m2) and 7.2 mm/m2 (0.0–25.6 mm/m2), respectively. The indexed offset distance was ⩾8 mm/m2 in 29 (43.9%) of the patients with pulmonary atresia with intact ventricular septum; clinical and echocardiographic characteristics were comparable between these 29 patients and the remaining 37 patients with pulmonary atresia with intact ventricular septum. When an indexed offset distance ⩾8 mm/m2 was applied as a cut-off for the diagnosis of Ebstein’s anomaly, the sensitivity was 0.963 and the specificity was 0.561. In conclusion, indexed offset distance ⩾8 mm/m2 cannot be used as a cut-off for the diagnosis of complicated Ebstein’s anomaly in neonatal patients with pulmonary atresia with intact ventricular septum.

Asymptomatic isolated congenital left ventricular diverticulum: a cardiac MRI report about a 19-year-old man.

Mitral valve stenosis caused by a discrete supravalvular membrane is a rare congenital malformation haemodynamically leading to significant mitral valve stenosis. When the supravalvular mitral stenosis consists of a discrete supravalvular membrane adherent to the mitral valve, it is usually not clearly detectable by routine echocardiography. We report about the typical echocardiographic finding in three young patients with this rare form of a discrete membranous supravalvular stenosis caused by a membrane adherent to the mitral valve. These cases present a typical echocardiographic feature in colour Doppler generated by the pathognomonic supramitral flow acceleration. Whereas typical supravalvular mitral stenosis caused by cor triatriatum or a clearly visible supravalvular ring is easily detectable by echocardiography, a discrete supravalvular membrane adjacent to the mitral valve leaflets resembling valvular mitral stenosis is difficult to differentiate by routine echocardiography. In our opinion, this colour phenomenon does resemble the visual impression of polar lights in the northern hemisphere; owing to its typical appearance, it may therefore be named as “Polar Light Sign”. This phenomenon may help to detect this anatomical entity by echocardiography in time and therefore improve the prognosis for repair.

It is an honour to present the Anthony Chang lecture at this 10th International Conference of the Pediatric Cardiac Intensive Care Society. I have had the privilege of knowing Dr Chang for over 20 years, and although we only worked for a short period of time together at the Children’s Hospital, Boston, in the Cardiac Intensive Care Unit, we have remained close colleagues and friends since that time. The contributions of Dr Chang to the development of paediatric cardiac intensive care are very clear, based on his clinical expertise, research and scholarship, and the development of the Pediatric Cardiac Intensive Care Society in its early days. More than this, Dr Chang is an individual with vision; in many respects, he has been ahead of the curve, anticipating and leading the direction of paediatric cardiac intensive care.

Adult survivors of aortic interruption is extremely rare. Diagnosis mostly depends on additional imaging with CT and MRI. We present a rare case of acquired complete interruption of the aorta that was found incidentally at the time of coronary angiography. This finding was confirmed by CT scan.

Social media is any type of communication utilising electronic technology that follows two guiding principles: free publishing or sharing of content and ideas and group collaboration and inter-connectedness. Over the last 10 years, social media technology has made tremendous inroads into all facets of communication. Modalities such as Facebook, YouTube, and Twitter are no longer viewed as new communication technologies. Owing to their tremendous usage, they are now common ways to conduct a dialogue with individuals and groups. Greater than 91% of teenagers and 89% of young adults routinely use social media. Further, 24% of teenagers reported being online “almost constantly”. These forms of communication are readily used by individuals cared for in the field of paediatric cardiology; thus, they should carry significant interest for cardiology care providers; however, social media’s influence on medicine extends beyond use by patients. It directly affects all medical providers, both users and non-users. Further, social media has the ability to improve care for patients with paediatric heart disease. This article details social media’s current influence on paediatric cardiology, including considerations for professional use of social media and potential opportunities to improve cardiac care.

Primary ciliary dyskinesia and heterotaxy are rare but not mutually exclusive disorders, which result from cilia dysfunction. Heterotaxy occurs in at least 12.1% of primary ciliary dyskinesia patients, but the prevalence of primary ciliary dyskinesia within the heterotaxy population is unknown. We designed and distributed a web-based survey to members of an international heterotaxy organisation to determine the prevalence of respiratory features that are common in primary ciliary dyskinesia and that might suggest the possibility of primary ciliary dyskinesia. A total of 49 members (25%) responded, and 37% of the respondents have features suggesting the possibility of primary ciliary dyskinesia, defined as (1) the presence of at least two chronic respiratory symptoms, or (2) bronchiectasis or history of respiratory pathogens suggesting primary ciliary dyskinesia. Of the respondents, four completed comprehensive, in-person evaluations, with definitive primary ciliary dyskinesia confirmed in one individual, and probable primary ciliary dyskinesia identified in two others. The high prevalence of respiratory features compatible with primary ciliary dyskinesia in this heterotaxy population suggests that a subset of heterotaxy patients have dysfunction of respiratory, as well as embryonic nodal cilia. To better assess the possibility of primary ciliary dyskinesia, heterotaxy patients with chronic oto-sino-respiratory symptoms should be referred for a primary ciliary dyskinesia evaluation.

Portable media devices are widely used by today’s youth. When used in hospitals, these devices can produce artefactual arrhythmias on telemetry.

We report a rare case of CHD and abdominal aorta interruption. Renal hypertension was the first sign of the abdominal aortic malformation. The aetiology of abdominal aortic interruption remains unclear, it could be congenital or acquired.

Surveillance of fetal arrhythmias in the outpatient setting remains limited by lack of monitoring modalities. Despite technological advances made in the field of obstetrics, existing devices are not currently suitable to monitor fetal arrhythmias. In this report, the author describes the current and developing fetal heart rate monitoring technologies including the recent introduction of hand-held Doppler monitors for outpatient surveillance of fetal arrhythmias.

We report a left atrial appendage diverticulum in close proximity to the left coronary artery, thereby mimicking a fistulous connection between the two structures.