We present a case of a previously healthy adolescent male for outpatient evaluation of prolonged QT interval. He was ultimately found to have acquired QT interval prolongation secondary to hypocalcaemia related to undiagnosed hypoparathyroidism. This case report highlights the importance of routine electrolyte analysis, even in the outpatient setting, during initial diagnostic workup for QT interval prolongation.

Acute myocarditis is a rare but potentially fatal disease. Endomyocardial biopsy and histologic examination are key to an accurate diagnosis. Despite being an uncommon cause, Influenza A and B viruses are a well-documented aetiology. Myocarditis may complicate about 0 to 10% of influenza virus infections (0.4 to 5% in paediatric cases). The clinical presentation varies widely, from ischemic-like chest pain to fulminant myocarditis with acute hemodynamic compromise, requiring mechanical circulatory support, with high mortality in the acute phase. We report a series of paediatric patients with myocarditis due to Influenza virus, to emphasize the importance of considering this uncommon aetiology.

Chronic physical training has been shown to produce multiple changes in the heart, resulting in the athlete’s heart phenotype. Some of the changes can make it difficult to discern athlete’s heart from true cardiac disease, most notably hypertrophic cardiomyopathy. Other diseases such as dilated cardiomyopathy and arrhythmogenic right ventricular cardiomyopathy may be difficult to rule in or out. In this article, the physiological cardiac changes of chronic athletic training are reviewed. A methodological approach using electrocardiography and echocardiography to differentiate between athlete’s heart and cardiac disease is proposed.

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.

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.

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.

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.

Congenital ductus arteriosus aneurysms develop in the third trimester of fetal life, possibly due to abnormal intimal cushion formation or elastin expression in the ductal wall. It is often diagnosed in infants before 2 months of age. Most have a benign course and resolve spontaneously. However, life-threatening complications have been reported. We report a case of large ductal aneurysm diagnosed incidentally in a neonate, in whom there was a novel mutation in the smooth muscle myosin protein gene—MYH11.

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.

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.

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.

The technique of modified ultrafiltration is a more efficient application of the concept of ultrafiltration during cardiopulmonary bypass. It has been shown to be superior to the conventional method of ultrafiltration.

The method can save considerable quantities of donor blood by returning not only the red cells, but also the white cells, platelets, and clotting factors back to the patient, components which otherwise would be discarded. The elevation of the hematocrit made possible by this method after bypass will permit an “acceptable” hematocrit to be achieved when temperatures become normal after ultrafiltration. The return of platelets and clotting factors along with a high hematocrit will contribute towards significantly minimizing the postoperative loss of blood, and, consequently, the amount of blood requiring to be transfused. The possibility of elevation of the hematocrit allows for a lower hematocrit during bypass. This can achieve a considerable saving of donor blood, particularly in small children who invariably need blood added to the prime, making bloodless pediatric surgery a real possibility. The technique also minimizes the rise in total water in the body after cardiopulmonary bypass in children and promises favorably to alter the course of leaking capillaries as seen frequently in neonates and infants. It has been shown to improve hemodynamics at a crucial time when the patient has been weaned from cardiopulmonary bypass and needs optimum conditions for recovery. From the point of view of research, ultrafiltration offers a window to look at the inflammatory process induced by cardiopulmonary bypass.

It promises to be a valuable technique to investigate the elimination and possibly quantification of toxic metabolites produced during bypass. It also allows evaluation and assessment of different protocols for bypass, involving varying rates of flow and temperature, for their response to the production of these toxic metabolites, thus offering the potential to achieve a model of cardiopulmonary bypass which is as physiological as possible. With advances in the systems of perfusion, aided by computers, it should be possible to control the rate of ultrafiltration and precisely regulate the transfusion of the venous reservoir fluid with the aid of feedback loops from the venous and arterial pressures in the heart. This could make ultrafiltration an integral part of routine cardiopulmonary bypass.

Fifteen patients requiring palliation for tetralogy of Fallot were treated by balloon dilation because of hypercyanotic spells. The mean age at dilation was 1.9 ± 0.7 years (range 0.5 – 3), and the mean weight 9.8 ± 2.1kg (range 6.0 –13.5). Dilation of the outflow tract was combined with dilation of the left and/or right pulmonary arteries in 5 patients. Successful dilation was achieved in 12 patients (80%), but failed in 3 patients with hypoplastic pulmonary arteries. In one patient, the stenosis of the right pulmonary artery could not be dilated because of a very sharp angle at the site of the stenosis. Two of the 3 patients in whom the procedure failed died of severe cyanotic spells within 24 hours of the unsuccessful procedure. No major complications occurred during or after the procedure in the cases undergoing successful dilation. The arterial oxygen saturation increased significantly, from 71 ± 5.7% to 89 ± 3.9%, immediately after the procedure (p < 0.005). During a period of follow up of 6 ± 3.7 months (range 1 – 13), the procedure was repeated on 3 occasions, and successfully accomplished in 2 of these. In conclusion, balloon dilation is a satisfactory palliative procedure for tetralogy of Fallot in those units in which total correction is not performed under 2 to 3 years of age.

We describe a 2-year-old girl with tetralogy of Fallot and pulmonary atresia, palliated as a neonate with a right modified Blalock Taussig shunt, who developed severe cyanosis following total correction in the absence of corresponding evidence of parenchymal lung disease on the chest X-ray. Selective pulmonary angiography showed new intrapulmonary shunting involving only the right middle and lower lobes only. The cyanosis resolved rapidly subsequent to inhalation of nitric oxide. To our knowledge, this is the first documented case of rapid onset of localised intrapulmonary right-to-left shunting, involving only two lung lobes, following biventricular repair for complex congenital heart disease.