Aneurysm of the sinus of Valsalva is an uncommon congenital lesion rarely reported in children. Unruptured aneurysms commonly go undiagnosed until a rupture has occurred. Usually, ruptured sinus of Valsalva presents with cardiac failure. There may be a history of trauma or infective endocarditis preceding the rupture. Asymptomatic paediatric presentation of ruptured sinus of Valsalva is rare. We discuss the cases of two children who presented with a murmur and were diagnosed with ruptured sinus of Valsalva. This unusual presentation in children highlights the importance of careful routine physical examinations and the evaluation of new murmurs. The Okham's razor principle states that “when you hear hoofbeats – think horses not zebras”. Sometimes, it is important to think beyond the usual in medicine, to avoid missing lesions that, if left untreated, could lead to adverse outcomes.

Aorta catheterisation can serve both diagnostic and therapeutic purposes in patients with aortic coarctation, especially when non-invasive evaluation cannot define severity of the abnormality. We report a case of bilateral brachial plexus injury due to standard positioning of the patient’s arms during non-complicated aorta catheterisation and aortic stent implantation under general anaesthesia. In discussion, we consider our patient’s predisposing factors, the understanding of which may be helpful in avoiding this complication in the future.

Left ventricular aneurysms in the foetus are a rare abnormality that can occur in isolation or associated with pentalogy of Cantrell. Here, we report a case of a foetus with a left ventricular aneurysm in association with interruption of the aortic arch, and no features of pentalogy of Cantrell. To our knowledge, this is the first report of such an association.

The Holt–Oram syndrome was confirmed in an asymptomatic 36-year-old man by a novel TBX5-gene mutation (exon 8 acceptor splicing site, c.663-1G greater than A). Computed tomography showed an atrial septal defect and an anomalous right coronary artery crossing between the aorta and pulmonary arteries. Surgery corrected the septal defect and the initial segment of the anomalous vessel was unroofed and enlarged. Anomalous coronary arteries were not previously described in the Holt–Oram syndrome patients and should be added to the list of possible associated cardiac defects.

The following document provides a summary of the guidelines and recommendations for paediatric cardiac intensive care training as a requirement for recognition as a European paediatric cardiologist. It is therefore primarily targeting paediatric cardiology trainees in Europe, including those doctors who might wish to become experts in cardiac intensive care. These recommendations represent a frame for consistency, will evolve, and may be adapted to specific institutional requirements. They will be complemented by a learning module to be provided by our Association in the near future.

Precise lung perfusion quantification is essential for evaluation of patients with hemi-Fontan surgery. It is possible for two-dimensional cardiac magnetic resonance phase contrast flow (two-dimensional flow) to evaluate non-invasively the systemic-to-pulmonary collateral blood flow. This case report intends to illustrate the benefit of four-dimensional flow over the current two-dimensional flow in the comprehensive assessment of hemi-Fontan patients.

In 2006, a process was initiated to develop evidence-based paediatric guidelines directed towards physicians for reduction of cardiovascular risk. In contrast to prior consensus-based guidelines from the National Heart, Lung, and Blood Institute, this process was to be evidenced based. This manuscript describes the process undertaken to write the evidence-based guidelines from the National Heart, Lung, and Blood Institute, beginning with the search for evidence, then the process of review of the evidence, and finally the writing of the final document. This manuscript also provides some thoughts on how this process might be adapted in developing guidelines for caring for patients with congenital cardiac disease.

A 32-year-old female patient presented with cardiac failure because of systolic dysfunction. Five years before, a DDD pacemaker had been implanted because of complete atrioventricular block. Echocardiographic examination disclosed left ventricular hypertrabeculation/non-compaction. Because of sinus tachycardia, ivabradine was started and the patient’s left ventricular function returned to normal within 4 months. Recurrent creatine-kinase elevation and reduced nicotinamide adenine dinucleotide staining on muscle biopsy suggested metabolic myopathy.

We report the case of a child with cor triatriatum dexter masquerading as Ebstein's anomaly on transthoracic echocardiography. This was attributed to a floppy membrane arising from the right atrium, protruding into the tricuspid valve and pushing the leaflets downwards, giving an impression of Ebstein's anomaly. The importance of recognising this pitfall will prevent misdiagnosis of Ebstein's anomaly.