A 20-day-old girl was referred to our clinic for systolic murmur, cyanosis, and dyspnoea with feeding. Echocardiography revealed an atretic aortic valve. CT angiography scan revealed that the left common carotid artery originated from the distal main pulmonary artery. The plan was patent ductus artriosus stenting and bilateral pulmonary artery banding and then follow-up for any possible future intervention.

We implanted two sinus Superflex DS© stents in a systemic non-stenotic arterial duct of a newborn with hypoplastic left heart syndrome. Forty-eight hours later an important collapse of the stent was detected and treated with a balloon expandable stent. Sinus Superflex DS© has been specifically designed for systemic ducts. This complication generates doubts about its radial force in this scenario.

Since the pioneering work of Fontan and Baudet, who suggested that a dysfunctional right ventricle could be bypassed by connecting the pulmonary arteries directly to the right atrium in the so-called atriopulmonary anastomosis, much experience has been gained in the field of the functionally univentricular circulation. In view of the continuing need to optimize the fluid dynamics of the connection, research on this topic remains very active. In particular, it is relevant to consider the power dissipated during flow across a connection of this type, due to the low level of the pressure head available for perfusion. The flow to the lungs in this setting is driven only by the low pressure in the caval veins, thus making it essential to minimize the losses of energy in the connection between the terminal part of the venous system and the pulmonary arteries.

A 15-day-old premature patient with ventricular septal defect and interrupted aortic arch type B underwent “hybrid” initial treatment consisting of bilateral pulmonary artery banding followed by stenting of the ductus arteriosus. A pre-registered CT scan was re-purposed with a new three-dimensional image fusion software (VesselNavigator) to create a roadmap for stent delivery.

We have conducted, in two stages, a descriptive and comparative analysis of the diagnostic and evolutionary clinical aspects of rheumatic fever. The descriptive analysis was based on a sample of 226 patients seen between 1976–1986. The forms and frequency of the manifestations of rheumatic fever were studied, as well as the difficulties encountered in diagnosing mild cases and the risk of making a hasty diagnosis due to the high prevalence of the disease. Subsequent to the establishment of an outpatient clinic for referral of patients with rheumatic fever, as part of a study and control program for the disease, 98 patients were seen in the period 1988–1991. In order to compare the profile of the manifestations in the acute phase with evolutionary aspects over the course of the disease, a subgroup of 61 children, treated in the period 1983–986 was selected to obtain equivalence in size of sample and time of follow-up (p = 0.08). The comparative analysis revealed that no modifications have occurred over the period of study in the profile of the manifestations during the acute phase, and no statistically significant differences have occurred in age at first attack, severity ofcarditis, or in the type ofvalvar and articular involvement. Evolutionary data after rigid control of primary and secondary prevention, however, showed a marked reduction in the incidence of recurrence (p = 0.036), the frequency of hospital admission (p = 0.001) and deaths (p = 0.024). The main factors modifying the evolution of the disease were analyzed. The action of the health team had a fundamental importance in the control of recurrence. This fact is especially important for developing countries, where a reduction of the prevalence of the disease can only be achieved through major structural socioeconomic changes in the community.

We report an infant with a large perimembranous ventricular septal defect, who had two separate orifices in the tricuspid valve, each supported by separate cordal apparatus, detected incidentally during surgery.

Objective: Modern surgical techniques now offer the potential for reconstructing rather than replacing the mitral valve in those patients who require an operation during childhood. The purpose of this study was to assess the outcome in our centre following mitral valvar surgery in children. Methods: Between 1992 and 2002, 51 consecutive children, with a mean age of 12.5 ± 3.5 years, underwent surgical treatment of mitral valvar stenosis and/or insufficiency in the Cardiovascular Surgical Department of Istanbul University. Of the children, 20 underwent repair, whilst we replaced the valve in 31 patients. In one, we inserted a pulmonary valvar autograft in mitral position. Results: There were neither early nor late deaths in those who underwent mitral valvar repair. Similarly, there were no early deaths in those in whom the valve was replaced, but two patients died late, while six outgrew their prosthetic valves and required insertion of a further valve within 10 years of their first operations. Conclusion: Our experience shows that procedures designed to repair the mitral valve are effective and reliable when performed in children, with low mortality, and a lower incidence of reoperation when compared with those having replacement of the mitral valve.

Limited evidence exists to guide chest tube management following cardiac surgery in children. We assessed chest tube practice variation by surveying paediatric heart centres to prepare for a multi-site quality improvement project. We summarised management strategies highlighting variability in criteria for chest tube removal between and within centres. This lack of standardisation provides an opportunity for quality improvement.

Kawasaki disease is the leading cause of acquired heart disease in infants and young children. Kawasaki disease that manifests as facial nerve palsy is extremely rare, and the diagnosis is challenging. We report a 4-month-old girl with Kawasaki disease who presented with fever, redness and cracking in the lips and oral cavity, and a right facial nerve palsy. The infant received intravenous immunoglobulin, acetyl salicylic acid, and warfarin. The patient’s fever subsided on the following day, and the right-sided facial nerve palsy was relieved a month later.

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.

Marfan syndrome, bicuspid aortic valve, and/or coarctation of the aorta are associated with medial abnormalities of the ascending aortic or para-coarctation aorta. Medial abnormalities in the ascending aorta are prevalent in other type of patients with a variety of CHDs such as single ventricle, persistent truncus arteriosus, transposition of the great arteries, hypoplastic left heart syndrome, and tetralogy of Fallot, encompassing a wide age range and may predispose to dilatation, aneurysm, and rapture necessitating aortic valve and root surgery. These CHDs exhibit ongoing dilatation of the aortic root and reduced aortic elasticity and increased aortic stiffness that may relate to intrinsic properties of the aortic root. These aortic dilatation and increased stiffness can induce aortic aneurysm, rapture of the aorta, and aortic regurgitation, but also provoke left ventricular hypertrophy, reduced coronary artery flow, and left ventricular failure. Therefore, a new clinical entity can be used to call this association of aortic pathophysiological abnormality, aortic dilation, and aorto-left ventricular interaction – “aortopathy”.

We describe a previously asymptomatic 7-year-old girl with a sudden cardiac arrest during elective pacemaker revision. Later imaging identified epicardial pacemaker lead strangulation of the left anterior descending and left circumflex coronary arteries. Anaesthetic induction led to a reduction in myocardial perfusion, precipitating the arrest. Extreme care should be taken during anaesthesia if cardiac strangulation is suspected.

This study evaluates clinical and epidemiological features of acute rheumatic fever using the data of last 25 years in our hospital in south-east of Turkey. The medical records of 377 patients with acute rheumatic fever admitted to Pediatric Cardiology Department of Çukurova University during 1993–2017 were retrospectively analysed. Two hundred and six patients were admitted between 1993 and 2000, 91 between 2001 and 2008, and 80 between 2009 and 2017. The largest age group (52%) were between 9 to 12 years of age and approximately two-thirds of the patients presented in the spring and winter seasons (62.8%). Among the major findings, the most common included carditis 83.6% (n = 315), arthritis at 74% (n = 279), Sydenham’s chorea at 13.5% (n = 51), and only two patients (0.5%) had erythema marginatum and two patients (0.5%) had subcutaneous nodule. Carditis was the most common manifestation observed in 315 patients (83.6%). The most commonly affected valve was the mitral valve alone (54.9%), followed by a combined mitral and aortic valves (34%) and aortic valve alone (5.7%). Of the patients with carditis, 48.6% (n = 153) had mild carditis, of which 45 had a subclinical. Sixty-two patients (19.7%) had moderate and 100 patients (31.7%) had severe carditis. At the follow-up, 2 patients died and 16 patients underwent valve surgery. Twenty-eight (7.4%) patients’ valve lesions were completely resolved. Conclusion: Although the incidence of acute rheumatic fever decreased, it still is an important disease that can cause serious increases in morbidity and mortality rates in our country.