Patients with congenitally corrected transposition frequently benefit from re-synchronisation therapy or ablation procedures. This is likely to require catheterisation of the coronary sinus. Its anatomy, however, is not always appreciated, despite being well-described. With this caveat in mind, we have evaluated its location and structure in hearts with congenitally corrected transposition in order to reinforce the guidance needed by the cardiac interventionist. We dissected and inspected the coronary sinus, the oblique vein of the left atrium, and the left-sided-circumflex venous channel in eight heart specimens with corrected transposition and eight controls, measuring the orifice and length of the sinus and the atrioventricular valves. In two-thirds of the malformed hearts, the sinus deviated from its anticipated course in the atrioventricular groove, ascending obliquely on the left atrial inferior wall to meet the left oblique vein. The maximal deviation coincided in all hearts with the point where the left oblique vein joined the left-sided-circumflex vein to form the coronary sinus. We describe a circumflex vein, rather than the great cardiac vein, as the latter venous channel is right-sided in the setting of corrected transposition. The length of the sinus correlated positively with the diameter of the tricuspid valve (p=0.02). Compared with controls, the left oblique vein in the malformed hearts joined the circumflex venous channel significantly closer to the mouth of the sinus. The unexpected course of the coronary sinus in corrected transposition and the naming of the cardiac veins have important implications for venous cannulation and interpretation of images.

Research that is closely connected with the population it is studying and in which the translational value to healthcare is high is a laudable goal, but it is not often achieved. The Australian and New Zealand Fontan Registry has developed a model for involving patients and parents of children with a Fontan circulation in its research. The model involves consumer participation in the overarching Steering Committee, and has set in place multiple channels of communication allowing the early dissemination of research findings before peer-reviewing, and consumer feedback at all levels of the research. Our focus was not only to provide information but also to give a voice to this community and include them as researchers. These communication channels are a part of a larger network involving the practitioner community, support groups, funding agencies, and health authorities. This close connection with the target research population has multiple benefits: safeguarding the project; controlling and adjusting both the messages conveyed and the investigations; building a community; raising new ideas for research; increasing our research participation rate; increasing the weight of our endeavours; and, above all, increasing our own satisfaction in our research undertakings. In conclusion, the interactions with patients and their families within Australia and New Zealand provide one potential model for the involvement of patients and parents that may result in research that is more relevant, focussed, and practically applicable in a healthcare setting.

We present the case of a 29-year-old man with mutation-positive Duchenne muscular dystrophy and mutation-positive hypertrophic cardiomyopathy. His cardiac phenotype has characteristics of both disorders; he manifests sub-epicardial left ventricular free wall late gadolinium enhancement that is consistent with Duchenne cardiomyopathy, as well as asymmetric ventricular septal hypertrophy, hyperdynamic left ventricular systolic function, and septal mid-myocardial late gadolinium enhancement, which are characteristic of hypertrophic cardiomyopathy.

Left ventricular twist results from the movement of two orthogonally oriented muscular bands of the helical myocardial structure, with a consequent clockwise rotation of the left ventricular base and counterclockwise rotation of the left ventricular apex. To the best of the authors’ knowledge, this is the first time that left ventricular “rigid body rotation”, the near absence of left ventricular twist in hypoplastic right-heart syndrome, has been demonstrated.

Persistent fifth – double-lumen – aortic arch is a rarely seen entity, which is usually associated with other cardiac anomalies; it has been previously reported only in children. We report a new case of double-lumen aortic arch with a systemic-to-systemic connection diagnosed incidentally during cardiac multi-detector computer tomography evaluation of coronary artery bypass grafts in an adult.

In a prospective cohort of 181 individuals followed up since childhood – when aged 9, 12 and 15 years – patients with a family history of premature coronary heart disease (n=18) had higher left ventricular mass index in adulthood – at mean age of 31 years – compared with those without (mean±standard error 39.1±1.9 versus 34.6±0.7 g/m2.7, p=0.04). The correlation between adult left ventricular mass index and child triglycerides (r=0.66, p=0.04 versus r=−0.03, p=0.75; pdiff=0.02) and diastolic blood pressure (r=0.65, p=0.02 versus r=0.16, p=0.07; pdiff=0.05) was stronger among those with a family history of coronary heart disease than in those without. Although preliminary, these data suggest that the higher left ventricular mass index among adults with a family history might be explained by their increased susceptibility to child cardio-metabolic risk factors.

We report a newborn male who presented with severe central cyanosis on the third day of life. Partial thrombotic obstruction of the pulmonary trunk secondary to Antithrombin III (homozygous defect of heparin binding site) deficiency was subsequently diagnosed. Surgical thrombectomy, and infusions of Antithrombin III concentrate, led to a successful outcome. We postulate that intrauterine thrombosis occurred to give this unusual presentation.

An intrapericardial vacuolated mass compressing and displacing the heart was diagnosed by echocardiography in a foetus of 22 weeks gestation. The birth was induced for early signs of foetal distress at 29 weeks and, after two initial pericardial evacuation procedures, the tumour was resected radically 7 days after birth at a weight of 1.55 kg. Mass histology showed teratoma associated with yolk sac tumour. We comment on the overall approach adopted after foetal diagnosis and the histopathological features of the tumour, and try to draw conclusions on patient outcome data.

Traditional ambulatory rhythm monitoring in children can have limitations, including cumbersome leads and limited monitoring duration. The ZioTM patch ambulatory monitor is a small, adhesive, single-channel rhythm monitor that can be worn up to 2 weeks. In this study, we present a retrospective cross-sectional analysis of the ZioTM monitor’s impact in clinical practice. Patients aged 0–18 years were included in the study. A total of 373 studies were reviewed in 332 patients. In all, 28.4% had structural heart disease, and 16.9% had a prior surgical, catheterisation, or electrophysiology procedure. The most common indication for monitoring was tachypalpitations (41%); 93.5% of these patients had their symptoms captured during the study window. The median duration of monitoring was 5 days. Overall, 5.1% of ZioTM monitoring identified arrhythmias requiring new intervention or increased medical management; 4.0% identified arrhythmias requiring increased clinical surveillance. The remainder had either normal-variant rhythm or minor rhythm findings requiring no change in management. For patients with tachypalpitations and no structural heart disease, 13.2% had pathological arrhythmias, but 72.9% had normal-variant rhythm during symptoms, allowing discharge from cardiology care. Notably, for patients with findings requiring intervention or increased surveillance, 56% had findings first identified beyond 24 hours, and only 62% were patient-triggered findings. Seven studies (1.9%) were associated with complications or patient intolerance. The ZioTM is a well-tolerated device that may improve what traditional Holter and event monitoring would detect in paediatric cardiology patients. This study shows a positive clinical impact on the management of patients within a paediatric cardiology practice.

Cardiac rhabdomyomas in patients with tuberous sclerosis complex are usually detected antenatally or during infancy, with subsequent stabilisation or spontaneous regression. Development of a new cardiac rhabdomyoma beyond infancy is very rare. We report a male child who needed resection of a large rhabdomyoma in neonatal life, and then developed a new-onset rhabdomyoma at 2 years of age in a different location, needing another resection. Routine surveillance for cardiac rhabdomyomas in asymptomatic patients with tuberous sclerosis is essential.

A case of related individuals affected by sick sinus syndrome is presented in this study. The clinical and electrocardiographic signs of sinus node dysfunction and the most common causes of this disease are presented. Subsequently, the article includes descriptions of sinus node disease in three related children as well as details of the disease in their relatives. A literature review of the genetics of familial sinus node dysfunction concludes the study.

This case report documents the successful surgical repair of an aorto-bronchial fistula and a giant aortic pseudoaneurysm at the proximal anastomosis of a dacron interposition tube graft that was balloon dilated for recurrent coarctation. Balloon dilation for recoarctation of a dacron interposition tube graft may lead to serious complications.

Background: Disagreement currently exists regarding the definition of aortic dextroposition. It is suggested that the term be used interchangeably with aortic overriding, along with suggestions that the aortic valve overrides in the normal heart. The dextroposed aorta, however, does not always override the crest of the muscular ventricular septum. It is incorrect to argue that the normal aortic valve overrides. It is the cavity of the right aortic valvar sinus, rather than the valvar orifice, that sits above the muscular septum when the septum itself is intact. Therefore, to circumvent these difficulties, those using the term “dextroposition” find it necessary to distinguish “true” as opposed to “false” categories. The problems arise because “dextroposition” is remarkably ill-suited as an alternative term for aortic valvar overriding. Methods and Results: In this review, combining developmental, morphologic, and clinical data, we show how aortic overriding is best considered on the basis of biventricular connection of the aortic root in the setting of deficient ventricular septation. When analysed in this manner, it becomes an easy matter to distinguish between one-to-one and double outlet ventriculo-arterial connections. Appreciation of these features emphasises the different spatial alignments of interventricular communications as opposed to the plane of deficient ventricular septation. The concept of overriding is applicable not only to biventricular connection of the aortic root, but also the pulmonary and common arterial roots. Conclusions: The diagnostic techniques now available to the paediatric cardiologist illustrate the features of arterial valvar overriding with exquisite accuracy, informing the discussions now required for optimal decision making.

Introduction: The term cor triatriatum is used to describe a fibromuscular partition that divides an atrium into two compartments. It was first used by Borst in 1905, although the lesion had been described before by Church in 1868. Both described divided left atrial chamber (also referred to as cor triatriatum sinister). The incidence of the divided left atrial chamber among patients with congenital heart disease is reported at 0.1% and 0.4% in an autopsy study. Divided right atrial chamber – also referred to as cor triatriatum dexter – is even rarer to an extent that there is no reported incidence and only few hundred cases have been reported so far. A search through pubMed library revealed only 90 publications on humans, either under the term “dexter” or “dextrum”. We would like to report a new association of bilateral divided atrial chambers and pulmonary vein stenosis, with a review of the literature.

A Fontan circulation requires a series of three-staged operations aimed to palliate patients with single-ventricle CHD. Currently, the most frequent technique is the extracardiac total cavopulmonary connection, an external conduit connecting the IVC and right pulmonary artery, bypassing the right side of the heart. Fontan candidates must meet strict criteria; they are assessed utilising both cardiac catheterisation and cardiac magnetic resonance. Postoperatively, treatment protocols prioritise antibiotic prophylaxis, diuretics, angiotensin-converting enzyme inhibitors, anticoagulation, and oxygen therapy with fluid restriction and a low-fat diet. These measures aim to reduce length of stay in the ICU and hospital by preventing acute complications such as infection, venous thromboembolism, low cardiac output, pleural effusion, and acute kidney injury. Late complications of a Fontan procedure include circulation failure, protein-losing enteropathy, plastic bronchitis, and Fontan-associated liver disease. The definitive management is cardiac transplantation, with promising innovations in selective embolisation of lymphatic vessels and Fontan-specific ventricular assist devices. Further research assessing current protocols in the perioperative management of Fontan patients would be beneficial for standardising current practice and improving outcomes.