Arrhythmias in patients with congenital heart disease present a challenge to the care of these patients and can result in significant morbidity and mortality. Transposition of the great arteries and common arterial trunk are no exceptions. It is important to identify risk factors for arrhythmia development in the peri-operative period. The peri-operative arrhythmia burden may relate to the underlying congenital heart disease, haemodynamic perturbations, operative events, and potential residual lesions. In addition, these patients are at risk for developing arrhythmias later in life, and non-invasive and potentially invasive arrhythmia surveillance should be a routine part of the care of these patients. This article highlights important strategies to manage arrhythmia development and prevention in this patient population.

We report the case of a patient with Donohue syndrome who died of heart failure due to obstructive hypertrophic cardiomyopathy. A literature survey revealed that hypertrophic cardiomyopathy was present in 30% of these patients and was often fatal. Therefore, every patient with Donohue syndrome should be screened for hypertrophic cardiomyopathy.

The term right aortic arch is used for an aorta that arches over the right bronchus. Right aortic arch was classified into two types by Felson et al, based on branching patterns, with a proposed embryological explanation, and into three types by Shuford et al. Other anatomical variants of right aortic arch were described later, including isolated left brachiocephalic artery and aberrant left brachiocephalic artery. We have classified right aortic arch anatomy into 10 variants, supported by radiological evidence, and with reference to possible embryology. This classification will help in understanding the morphological basis for the formation of different types of right aortic arch and the course of the recurrent laryngeal nerve in such cases.

The Maltese populartion constitutes an ideal location for epidemiological and historical studies dealing with congenital hearr malformations. Pulmonary stenosis was studied retrospectively from 1943 to 1994. A sharp, significant decline in age at diagnosis was found, which predates the introduction of echocardiography. All defects are now diagnosed by echocardiography, which has resulted in an increased prevalence at birth of this lesion, patticularly of pulmonary stenosis not requiring intervention. The prevalence at birth of pulmonary stenosis from 1990 to 1994 was 1.65/1000 live births (95% CL: 1.21–2.24), with 1.11/1000 mild lesions (95% CI: 0.76–1.62) and 0.54/1000 lesions requiring intervention (95% CI: 0.31–0.92). The prevalence at birth overal was significantly higher than that reported in recent studies with similar methodologies due to the higher pickup of milder variants of pulmonary stenosis by echocardiography.Significantly more pulmonary more pulmonary stenosis was found in females than in males.

Despite a good haemodynamic result, many children have amildly decreased arterial oxygen saturation following a total cavopulmonary connection. Our study was performed to determine possible mechanisms of right-to-left shunting in these patients. We performed elective cardiac catheterization in 19 children at a mean interval of 3.6 years following a total cavopulmonary connection. The intrapulmonary right-to-left shunt, the intracardiac right-to-left shunt and the total right-to-left shunt were calculated under mechanical ventilation with 100% oxygen. The intrapulmonary right-to-left shunt was 10.8±3.5% of the pulmonary blood flow, and the total right-to left shunt accounted for 18.9±5.2% of the systemic blood flow. The intracardiac right-to-left shunt in patients with no relevant venovenous collaterals or leaks in the atrial tunnel was calculated at 6.4±3.0% of the systemic blood flow, while the intracardiac right-to-left shunt in patients with relevant collaterials or leaks accounted for 13.0±5.9% of the systemic blood flow. Since intrapulmonary arteriovenous fistulas were not demonstrated angiographically in any of our patients, the intrapulmonary right-to-left shunt is probably due to low ratios of perfusion to ventilation in some pulmonary segments. The intracafdiac right-to-left shunt was due to leaks across the interatrial baffle, collaterals between stystemic and pulmonary veins, and to the coronary sinus draining to the pulmonary venous atrium.

We extracted L., the newborn of a diabetic mother, for antenatal diagnostic of myocardial hypertrophy and anomaly of foetal heart rate. Post-natal echocardiography showed severe septal myocardial hypertrophy with latero-basal myocardial akinesia. We did not observe a Doppler gradient through the left ventricular outflow. Selective coronary angiography showed an occlusion of the circumflex artery. Myocardial hypertrophy is a classic complication in newborns of diabetic mothers. The mother’s diabetes and neonatal infarction remain an exceptional association. We discuss a different hypothesis to explain coronary occlusion and how myocardial infarction avoided septal obstruction.

Aorto-left ventricular tunnels are anomalous communications between the ascending aorta and the left ventricle. Prenatal diagnosis, followed by successful surgical management, is rare. We describe a fetus diagnosed with aorto-left ventricular tunnel at 35 weeks who achieved a favourable outcome after immediate postnatal surgical repair.

Aims: Measurement of electrocardiographic intervals to assess dispersion in ventricular repolarization may be helpful in the assessment of the risk of ventricular arrhythmia. We measured QTc, QT dispersion, and T wave intervals in premature infants before and while on treatment with the IKr blocker cisapride as markers for dispersion in ventricular repolarization. Methods and Results: We enrolled 15 non-ventilated premature infants with a mean gestational age of 30.5 weeks, ranging from 26.5 to 33.5 weeks, and mean postnatal age of 24 days, with a range from 5 to 51 days. A digital 12 lead electrocardiogram was recorded prior to and 3 days after administering cisapride at a dose of 0.8mg/kg/day. Serum electrolytes were simultaneously measured. Electrocardiographic measurements before and after included: QT, QTc Bazett, QT dispersion, R–R, T wave interval peak to end, T wave interval peak to end/onset Q to T wave peak, T wave axis, T wave maximum voltage and QRS–T angle. A paired t test and analysis of variance was used to compare the variables before and during treatment. The QTc, T wave interval peak to end and the ratio T wave interval peak to end/onset Q to T peak increased significantly following treatment with cisapride. Results expressed as before and during treatment were for QTc: 429 (65) ms versus 454 (29) ms p < 0.02; for T wave interval peak to end: 65 (11) ms versus 103 (24) p < 0.01, for the ratio T wave interval peak to end/onset Q to T peak: 0.32 (0.06) versus 0.55 (0.16) p < 0.001. Treatment with the IKr blocker did not significantly alter the QT dispersion, T wave voltage, angle or QRS–T angle. Conclusion: The interval from the peak to the end of the T wave and the ratio of this value to the onset Q to T peak interval, represents regional dispersion of repolarization across the ventricular wall. This is a potentially useful clinical index in the assessment of arrhythmic risk in premature infants being treated by blockade of the IKr channels.

In all patients undergoing cardiac surgery, the effective delivery of oxygen to the tissues is of paramount importance. In the patient with relatively normal cardiac structures, the pulmonary and systemic circulations are relatively independent of each other. In the patient with a functional single ventricle, the pulmonary and systemic circulations are dependent on the same pump. As a consequence of this interdependency, the haemodynamic changes following complex palliative procedures, such as the Norwood operation, can be difficult to understand.

Comparison of the newly created surgical connections to a simple set of direct current electrical circuits may help the practitioner to successfully care for the patient. In patients undergoing complex palliations, the pulmonary and systemic circulations can be compared to two circuits in parallel. Manipulations of variables, such as resistance or flow, in one circuit, can profoundly affect the performance of the other circuit. A large pulmonary flow might result in a large increase in the saturation of haemoglobin with oxygen returning to the heart via the pulmonary veins at the expense of a decreased systemic flow. Accurate balancing of these parallel circulations requires an appreciation of all interventions that can affect individual components of both circulations.

Infective endocarditis is a serious complication occurring after percutaneous pulmonary valve replacement with a Melody valve; it is of uncertain incidence, and presents a challenging diagnosis. Vegetations may be difficult to visualise with transthoracic and transoesophageal echocardiography. We report the role of intracardiac echocardiography in this condition by confirming the prosthesis involvement.

The common respiratory syncytial virus has been associated with cardiac involvement, usually tachyarrhythmias and occasionally myocarditis. A child is described who probably developed a complete heart block following respiratory syncytial bronchiolitis. Such an association needs to be considered if the heart is structurally normal and there is no evidence of a congenital heart block in a setting of maternal anti-Ro and anti-La antibodies.

Video assisted thoracoscopic surgery for closure of the persistently patent arterial duct is an effective answer to the modern effort of seeking repair of congenital cardiac malformations with minimally invasive techniques.

Between June, 1994, and December, 2002, 150 consecutive patients with an echocardiographic diagnosis of isolated patency of the arterial duct were referred to our Institution for video assisted thoracoscopic closure. The median age at the time of operation was 45 months, with a range from 3 to 161 months, and mean weight was 18 kg, with a range from 4.2 to 73 kg. Video assisted closure was possible in 139 patients (93%). The mean operative time was 37 min, with a range from 14 to 89 min. In the remaining 11 patients (7%), seen early in our experience, we converted to a mini-thoracotomy to achieve closure. In no instance did we encounter major haemorrhage requiring blood transfusion, and there were no hospital deaths. The mean period of stay in hospital was less than 24 h in 61 patients, less than 48 h in 88 patients seen earlier in our experience, and 21 days in 1 patient because of a persistent chylothorax. Other complications included palsy of the left recurrent laryngeal nerve in 1 patient, with partial regression at 6 months follow-up.

We conclude that closure of the arterial duct by video-assisted thoroscopy combines the advantages of safe and complete extraluminal occlusion with an excellent clinical and aesthetic result, minimal postoperative pain, and short stay in hospital at low cost.

Down syndrome is the most common chromosomal abnormality, with an incidence of one case in every 650 live births. It is strongly associated with heart disease, which constitutes the main cause of mortality during the first 2 years of life in this population. Most of the cardiac abnormalities in patients with Down syndrome can be suspected by analysing the surface 12-lead ECG. The purpose of this systematic review was to analyse all available published material on surface ECG and cardiac rhythm and conduction abnormalities in patients with Down syndrome to facilitate the search to the clinical cardiologist and paediatrician.

Acute heart failure related to anaphylactic shock is often reversible and necessitates aggressive support to ensure full recovery. We report the case of a 15-year-old boy who developed severe ventricular dysfunction and haemodynamic instability after administration of amiodarone and required temporary mechanical circulatory support with a left ventricular assist device. He had full recovery of cardiac function and returned to baseline neurologic status. This is the first report of successful left ventricular assist device use for recovery from cardiovascular collapse due to anaphylaxis.

We report a case of thyroid storm precipitated by SARS-CoV-2 infection in an adolescent girl with a history of Graves disease and dilated cardiomyopathy. This case highlights that SARS-CoV-2 infection can potentially trigger a thyrotoxicosis crisis and acute decompensated heart failure in a patient with underlying thyroid disease and myocardial dysfunction even in the absence of multi-system inflammatory syndrome in children. We systematically reviewed the thyrotoxicosis cases with SARS-CoV-2 infection and described its impact on pre-existing dilated cardiomyopathy.