Pulmonary arterial and intracranial calcifications are rarely found in children. A female infant, the recipient of a twin–twin transfusion syndrome was found, by ultrasound and computed tomography, to have both pulmonary arterial and intracerebral calcification. A rare condition, termed idiopathic arterial calcification of infancy, is the likely cause. This condition carries a poor prognosis and is usually fatal.

We present a young infant with rapidly progressive idiopathic dilation of the right atrium, complicated by symptomatic and dynamic obstruction of the airways secondary to proximal obstruction which was reversed subsequent to surgical reduction of the right atrium. We discuss the surgical implications of the distorted cardiac anatomy, along with post-operative complications, which included refractory arrhythmias.

We report a 12-year-old child with anomalous origin of the left coronary artery from the right coronary aortic sinus, the artery taking a proximal intramural course. The anomalous artery was reimplanted into the left coronary aortic sinus. Postoperative stenosis was successfully treated with percutaneous transluminal angioplasty and implantation of a stent.

Innocent murmurs in childhood are common, and often they do not reflect cardiac disease. We have performed a prospective review, by means of a questionnaire, to clarify the parental perception of the innocent murmur identified in their child. Whenever possible, depending on age, we also sought the input of the child.

Of a cohort of 63 infants and children with innocent murmurs, a response was obtained from 40 parents and 8 children to an initial questionnaire, with 26 parents replying to a second questionnaire a year later. No parent reported any symptoms in their children, or had imposed any restrictions. Of the parents, however three sets considered regular review was necessary. We conclude that the diagnosis of an innocent murmur by a paediatric cardiologist, aided by investigations including cross-sectional echocardiography, and an opportunity for explanation and reassurance, led to the child being treated normally.

Twisted atrioventricular connections usually occur in hearts with biventricular artioventricular connections. Here, we describe a case of twisted atrioventricular valves associated with double inlet left ventricle and discordant ventriculo-arterial connections. Color Doppler echocardiography, and cine magnetic resonance imaging, clearly demonstrated that the right atrioventricular valve was located anterior and superior to the left atrioventricular valve, and that the axes of the two atrioventricular valves crossed each other within the dominant left ventricle.

A child with pseudoxanthoma elasticum had mitral valvar prolapse with severe regurgitation necessitating replacement with a prosthetic valve. Extensive formation of pannus caused obstruction of two mechanical valves, one after twenty months and the second after three years. Histology of the pannus was similar to the endocardial lesions that are considered unique to pseudoxanthoma elasticum.

A non-dysmorphic 10 month old female was discovered at surgery to have severe vasculopathy of both the systemic and pulmonary arteries. These findings were confirmed by pathologic examination. Followup angiography has confirmed multiple sites of vascular obstruction which appear to be worsening. Angioplasty has only partially relieved these obstructions. The pathology and possible etiology are reviewed.

A 7-year-old boy developed increasing cyanosis after a total cavopulmonary connection with a 3mm fenestration in the baffle. Catheterisation performed 4 years and 7 months after the operation showed reopening of a left superior caval vein draining into the pulmonary venous atrium. Due to the large size of the left superior caval vein, and the absence of intrinsic stenosis, we chose to use an Amplatzer ductal device to occlude the reopened vein. The procedure was safe and successful.

Supraventricular tachycardia is a documented feature of Marfan syndrome. The safety and efficacy of radiofrequency ablation in this population of patients, however, has not been reported. We report on the successful use of radiofrequency ablation utilizing a trans-septal approach for the treatment of supraventricular tachycardia produced by an accessory muscular atrioventricular connection in a patient with Marfan syndrome.

We successfully performed percutaneous transluminal angioplasty to treat severe renovascular hypertension with left ventricular failure in a 5-month-old infant. Using the transcarotid approach, we dilated the stenotic left renal artery without any difficulties, using progressively larger balloons designed for dilation of coronary arteries.

We report a case of anomalous origin of the right pulmonary artery from the ascending aorta that was diagnosed by fetal ultrasound at 21 weeks of gestation. The clue to the diagnosis was present in the three-vessel view, this being one of the views that we use for fetal cardiac screening. The anomaly was corrected surgically at 11 days of age. We discuss the importance of prenatal diagnosis in the management of this rare anomaly.

A 3-year-old boy developed acute obstruction of a left modified Blalock-Taussig shunt following selective power injection of contrast in the shunt. Balloon dilation was ineffective due to rapid recoil of the narrowed segment, but implantation of a stent effectively abolished the obstruction. The obstruction itself may have been produced by an intimal flap caused by the power injection.

We performed an electrophysiological study, using non-contact mapping, in an 8-year-old girl weighing 39.9 kg who had suffered recurrent symptomatic episodes of exercise-induced non-sustained ventricular tachycardia. Color-coded isopotential maps of the ventricular tachycardia identified the area of earliest endocardial activation high and anterior in the right ventricular outflow tract. Although partial deflation of the balloon was required to position the ablation catheter at the earliest site of activation, this site was still identified accurately, as demonstrated by termination of the ventricular tachycardia and ectopy upon mechanical pressure, as well as application of radiofrequency current.

In this young patient, precise mapping of the earliest endocardial activation using the non-contact mapping system was safe and effective, allowing successful radiofrequency ablation of the tachycardia.

A new technique is presented to create percutaneously an unrestrictive and lasting atrial communication in small children. In 2 infants, a stent was deployed in a restrictive atrial communication and first inflated with an 8 mm balloon. The stent was then further expanded up to 10 or 12 mm. The gradient and turbulent flow between the two atriums disappeared completely. The stent retained a very stable position without embolisation. Other complications, such as formation of thrombus or arrhythmia, were not observed until elective explantation after 2 and 11 months. We conclude that, in infants, dilation of the atrial septum with a stent can provide a safe, lasting, and unrestrictive atrial communication.

The prevention of unexpected cardiac events in the young remains elusive. Among them, some are lethal. Early identification of life-threatening arrhythmogenic disorders which often manifest in infancy, childhood or even later, may allow initiation of effective preventive therapies. A large prospective study has indicated that some infants with prolongation of the QT interval in the first week of life died suddenly, and would have previously been labelled as victims of the sudden infant death syndrome. Furthermore, in infants with this diagnosis, post-mortem molecular screening has revealed the presence of the long QT syndrome. As an evolution of this background, some European countries have begun to consider the possibility of introducing in their National Health Services the performance of an electrocardiogram during the first month of life in all newborns, as part of a programme for cardiovascular screening. Most adult cardiologists, however, have no or minimal experience with electrocardiograms recorded in infants. Accordingly, the European Society of Cardiology has instituted a Task Force with the objective of creating guidelines for the interpretation of the neonatal electrocardiogram, focusing on the most clinically relevant abnormalities and on the ensuing options for management and referral. The main objective of the present document is to provide adult cardiologists with a practical approach to neonatal electrocardiography, and paediatricians and neonatologists with a tool that should facilitate medical interaction on cardiologic issues. There are important differences between neonatal and adult electrocardiograms. When a cardiologist examines the electrocardiogram of an apparently normal and healthy infant, the focus has to be on distinguishing between patterns that should cause no alarm, and those that require action or additional investigations. To provide clues for this distinction has been the main objective of the members of this Task Force. Whenever possible or appropriate, we have also suggested steps in management.