Cardiac catheterisation continues to play an important role in the long-term management of patients with common arterial trunk and transposition of the great arteries. Although non-invasive imaging has largely eliminated the need for diagnostic catheterisation in newborns with these congenital cardiac lesions, cardiac catheterisation is an important tool for the diagnosis of a variety of problems encountered after surgical intervention, and allows interventions to be performed when feasible. We review the indications for cardiac catheterisation and describe the specifics for various interventional procedures for these patients in this manuscript.

A young man had anomalous connection of the veins draining the upper lobe of the left lung to both a left-sided vertical vein and the left atrium. The ratio of pulmonary to systemic flows was 1.7 : 1, and he was symptomatic with evidence of volume overload of the right heart. He was successfully treated by percutaneous placement of an Amplatzer ductal occlusion device into the vertical vein.

Described is a rare association in a patient with the heart in the left chest, namely pulmonary atresia with intact ventricular septum, fistulous coronary arterial connections, a right-sided aortic arch and an aorto-pulmonary collateral artery feeding one lung. The pulmonary arteries were non-confluent, with the right lung supplied by the right arterial duct originating from the under surface of the right-sided aortic arch, and the left lung supplied through the aorto-pulmonary collateral artery arising from the descending aorta. The surgical management is different in the setting of non-confluent pulmonary arteries.

We describe our treatment of a premature baby born weighing 1400 g with severe aortic stenosis, with a gradient of 80 mmHg across the valve. Efforts to advance a 6 mm angioplasty catheter into the stenotic aortic valve via the left ventricle failed. Anterograde angioplasty, instead, was performed using two 4 mm coronary angioplasty catheters. Six months subsequent to the intervention, the pressure gradient measured 25 mmHg, and there was no hemodynamically significant aortic insufficiency.

The hybrid perventricular approach for the closure of trabecular ventricular septal defects is an attractive treatment modality for small children. Worldwide experience has shown that procedure success is influenced by the defect anatomical accessibility, operators’ expertise, and device technical features. In May 2018, a new promising device, the KONAR-Multi-functional™ ventricular septal defect occluder (Lifetech, Shenzhen, China), obtained CE-marking for septal defect transcatheter closure after the first-in-man implantation in 2013. Herein, this is the first report of successful perventricular closure of ventricular septal defect using this new device in a child with significant co-morbidities.

We report a case of endocarditis of a transcatheter pulmonary valve-in-valve in a 14-year-old boy with tetralogy of Fallot. He presented with recurrent low-grade fevers, lethargy, and anorexia. Multiple blood cultures grew a gram-positive rod, Corynebacterium pseudodiphtheriticum. He was taken to the operating room for removal of the vegetative endocarditis and pulmonary valve replacement.

Objective: To evaluate heart rate variability by Holter monitoring in type 1 diabetic children compared with a healthy control group and determine the factors modifying heart rate variability. Methods: This was designed as a prospective study comparing 28 patients, diagnosed with type 1 diabetes and under follow-up, with 27 healthy control group subjects. Results: The patients were aged 9.9 ± 4.2 years in the diabetic group, including 13 (46.5%) girls and 15 (53.5%) boys. The healthy control group comprised 20 (74%) girls and seven boys (26%) with an average age of 8.6 ± 3.7 years. The search for factors modifying heart rate variability yielded the following correlations: for the time-dependent variables, negative between age and both average and maximal heart rate (r = −0.263 and −0.460, respectively), negative between haemoglobin A1c and percentage of differences between adjacent RR intervals >50 ms, positive between diabetes duration and square root of the mean of the sum of squares of differences between adjacent NN intervals. The average heart rate and percentage of differences between adjacent RR intervals >50 ms was significantly higher in the girls than the boys in all groups. With regard to the frequency-dependent factors affecting heart rate variability, correlations were found between haemoglobin A1c level and both total power and very low frequency (r = −0.751 and −0.644) and between very low frequency and diabetes duration. Conclusion: A reduction in heart rate variability parameters was observed in type 1 diabetes mellitus patients who had a long disease duration or were poorly controlled, as compared with healthy controls.

Anomalous aortic origins of the coronary arteries comprise approximately one-third of all coronary artery anomalies and are characterised by coronary arteries with anomalies of aortic origin involving abnormal courses, stenoses, and compression that can lead to myocardial ischaemia and sudden death. Operative techniques to treat these anomalies have not been standardised yet. Moreover, the management of potential complications has not been addressed. Common and rare forms of anomalous aortic origins of the coronary arteries are reviewed and understood standard techniques for an uncomplicated unroofing procedure are illustrated. Also noted are techniques that can be applied to unexpected anatomical findings and unwanted complications that could prove to be life-threatening. Several technical recommendations are offered.

Hypoplastic left heart syndrome is the term introduced by Noonan and Nadas1 to describe a spectrum of cardiac anomalies characterized by varying degrees of significant underdevelopment of the left heart and aortic arch. These cardiac anomalies include mitral valvar disease, left ventricular hypoplasia, aortic stenosis at subvalvar, valvar, and supravalvar levels, hypoplasia of the ascending aorta and aortic arch, and aortic coarctation. Although descriptive, hypoplastic left heart syndrome suffers from being a very unspecific term, since it encompasses multiple degrees and combinations of abnormalities involving the left sided structures. In an attempt to provide gradings of severity, Kirklin and Barratt-Boyes2 categorized these patients falling into the syndrome into four classes, according to whether obstruction was found at one, two, or more levels, or whether there is aortic atresia. Then, in 1998, Tchervenkov and colleagues3 introduced the term hypoplastic left heart complex to describe a set of patients falling within the spectrum of hypoplasia of the left heart, but in the absence of intrinsic aortic or mitral valvar stenosis, this concept subsequently being endorsed by the International Committee established by the Society of Thoracic Surgeons, together with the European Association of Cardiothoracic Surgery, to rationalize the approach to nomenclature and databases.4 The analysis offered by Tchervenkov et al.3 showed that, in certain circumstances, there are potentially patients considered to have hypoplasia of the left heart who might be candidates for biventricular repair. If this is the case, then it is important to establish how this subset can be recognized, and how they are best treated.

Transcatheter pulmonary valve replacement is fast becoming an accepted alternative to repeat surgical pulmonary valve replacement for selected patients and therefore a complementary strategy in the long-term management of those requiring surgical pulmonary valve replacement. With a combined surgical and percutaneous approach, late morbidity for some of these patients may be diminished. This manuscript will review the current indications for this procedure, its limitations, and its benefits.

In a hemodynamically compromised neonate with complete transposition and intact ventricular septum (concordant atrioventricular and discordant ventriculo-arterial connections), it became necessary to control severe congestive heart failure. The arterial switch operation had been delayed because of intracranial bleeding and subsequent coagulopathy. The cardiac failure was reversed by surgical banding of the patent arterial duct. The arterial switch was performed successfully one week later.

Total anomalous systemic venous drainage is an extremely rare congenital heart defect. In this study we describe an 11-year-old girl who presented with a history of fatigue and central cyanosis that she had had since early childhood with unremarkable precordial examination results. Investigations revealed left heterotaxy with all systemic venous drainage to the left-sided atrium with non-compaction of the left ventricle.

Libman–Sacks endocarditis may be the first manifestation of systemic lupus erythematosus. The risk of its occurrence increases with the co-existence of the anti-phospholipid syndrome. Changes usually involve the mitral valve and the aortic valve. In this report, we present a case of Libman–Sacks endocarditis of the tricuspid valve in a teenage girl.

Aneurysms of the aortic sinus of Valsalva are rare and generally do not produce symptoms unless the aneurysm ruptures. When rupture occurs, the clinical findings depend to an extent on the site of the aneurysm and the cardiac chamber into which the aneurysm ruptures. We report a case of acute rupture of an aneurysm of the right aortic sinus, which was misdiagnosed as a ventricular septal defect. The clinical condition of the patient deteriorated rapidly, and transcatheter closure of the defect with an umbrella was associated with a fatal outcome.

Takotsubo cardiomyopathy is characterised by akinesis and ballooning of the left ventricular apex during contraction of the otherwise normal base of the heart. We describe the case of a 7-month-old previously healthy female who presented with an unwitnessed cardiac arrest. Workup raised suspicion for non-accidental trauma. Despite progression to brain death, the severely decreased ventricular function and apical akinesis of the left ventricle improved within 40 hours of admission. This report will familiarise paediatricians with this rare cardiomyopathy and emphasise the importance of considering non-accidental trauma as an inciting event for patients with unwitnessed cardiac arrest found to have decreased ventricular function.

With advances in surgical technique, more children with complex congenital heart disease are surviving.1 Increasingly, attention is focusing on late morbidities, such as right ventricular dysfunction.2 For many common forms of congenital cardiac malformations, the assessment of right ventricular size and function is now essential so as to make optimal decisions concerning management.3 Commonly encountered examples include the timing for operative replacement of the incompetent pulmonary valve following surgical repair of tetralogy of Fallot, and the assessment of suspected ventricular dysfunction in children with functionally single or systemic right ventricles.4 Echocardiography remains the most practical method for serially determining right ventricular function, despite its limitations.5 The easy accessibility of echocardiography has encouraged its continued development as a tool for determining right ventricular function in neonates, infants, and children. A comparison of values established for the right ventricle is shown in Table 1, along with the references from which they were culled. We will describe these values in sequence.