The recent significant reduction in operative mortality associated with surgical completion of the Fontan circulation is clearly multi-factorial. Better understanding of the fundamental physiological and anatomical issues have led to refinements in operative technique, early neutralization of potential risk factors, widespread use of a staged approach, and selective application of the concept of fenestration or incomplete partitioning. While further reduction of operative mortality is important, and may be anticipated in the future, major emphasis now must be placed on optimizing functional outcome, and understanding and managing the late complications associated with the unique physiology produced by the Fontan circulation.

We report an asymptomatic 18-month-old boy who underwent surgical resection of the discrete subaortic stenosis. Histopathologic examination of the muscle demonstrated diffuse lymphocytic infiltration of the myocardium. Polymerase chain reaction analysis of blood demonstrated parvovirus. This report highlights that asymptomatic myocarditis may be more prevalent than we realise, and that involvement of the myocardium by parvovirus may be entirely asymptomatic.

Background: Our purpose was to determine the outcome of pregnancy and delivery in patients with coronary arterial lesions caused by Kawasaki disease. Methods and Results: We surveyed by mail the Japanese national experience of pregnancy and delivery in patients known to have Kawasaki disease. The first questionnaire was returned by 154 of 207 (74%) institutions, and 16 of the 154 had knowledge of deliveries in their patients. Based on a second questionnaire, and previous Japanese case reports, we identified 46 deliveries in 30 patients from 16 institutions. The age at delivery ranged from 18 to 35 years, with a median of 27 years. Of the patients, 4 had undergone coronary arterial bypass grafting. Low-dose aspirin was given in 16 patients. The deliveries, 27 in all, had been vaginal in 20 patients, albeit that 7 required assistance by forceps or vacuum extraction under epidural anesthesia. Caesarean section had been performed in 11 patients, 3 for obstetric indications, and 1 for chest discomfort in the third trimester. Although there were no cardiac events, obstetric complications occurred in 2. Conclusion: The results of pregnancy and delivery were favourable. The mode of delivery should be primarily determined by obstetrical considerations, rather than the coronary arterial lesions caused by Kawasaki disease. Excessive anticoagulant therapy may not be needed for this population.

We report an unusual case in which the peripheral part of the otherwise normal left lung was supplied by major systemic arteries arising from the left subclavian, internal mammary and coeliac arteries. Unlike most cases with systemic arterial supply to a normal or sequestered lung, where the systemic arterial branches get access to the lung through the pulmonary hilum or inferior pulmonary ligament, in our patient the systemic arterial branches entered the lung through the transpleural routes.

A variety of surgical strategies have been utilized in attempts to accomplish long-term haemodynamic stability in patients with cardiac anomalies characterized by discordant atrioventricular connections, ventricular septal defect, and severe sub-pulmonary obstruction. The majority of these patients have what is commonly referred to as congenitally corrected transposition, together with a ventricular septal defect and pulmonary stenosis or atresia, in the setting of either usual or mirror imaged arrangement of the atrial chambers and the other organs of the body. A smaller sub-group, with discordant atrioventricular connections and double outlet right ventricle, with severe sub-pulmonary obstruction or pulmonary atresia, present similar physiology, and a comparable surgical challenge.

We report a rare variant of the Scimitar syndrome, in which the right lower pulmonary vein takes a meandering course before finally connecting in normal fashion to the left atrium. The pulmonary parenchymal segment drained by the tortuous vein is supplied by a systemic collateral artery, which was coiled via a catheter. We also closed the intracardiac defects by surgery. The elder brother of the patient had classical Scimitar syndrome.

Many patients with a functional univentricular heart were treated in the 1970s and 1980s, using an atriopulmonary connection to create the Fontan circulation.1–3 Although this procedure, in many patients, was initially successful, and provided arterial saturations of oxygen close to normal, as these patients were followed over the years, in some cases they developed significant complications. One complication of the atriopulmonary connection is progressive right atrial dilation, which leads to atrial arrhythmias, such as atrial flutter or fibrillation.4,5 The combination of these two problems leads to low cardiac output, diminished quality of life, and poor categorization within the classification of the New York Heart Association. This, and other issues, has led most centres to abandon the atriopulmonary connection as a means of creating the Fontan circulation in favour of the lateral tunnel with cavopulmonary connections,6 or the extracardiac conduit.7

We report the sudden cardiac death of a young male presenting with classic clinical features of LEOPARD syndrome, shown to be due to a mutation in the PTPN11 gene, and severe non obstructive hypertrophic cardiomyopathy. We also discuss briefly the usefulness of prophylactic risk stratification in patients with syndromic and non syndromic hypertrophic cardiomyopathy.

Common arterial trunk is frequently associated with anomalies of coronary arterial origin and distribution. We describe two infants in whom coronary intervention was performed during the repair of common arterial trunk, with reversal of ischaemic left ventricular dysfunction. We discuss the surgical implications of recognition of the coronary arterial anomalies during the repair of common arterial trunk.

We report a 3-month-old female infant, in whom pulmonary venous obstruction occurred after repair of totally anomalous pulmonary venous connection, and which was successfully released by a transcatheter implantation of a stent using the transseptal approach. Close follow-up is required, since the long-term outcome is still unclear. Nevertheless, transcatheter implantation of stents is a promising option for treating this challenging lesion.

We report an infant with hypoplastic left heart syndrome consisting of mitral valvar atresia, aortic valvar atresia, hypoplasia of the aortic arch and coarctation of the aorta, who demonstrated respiratory failure and global hypotonia, and who was eventually diagnosed with spinal muscular atrophy.

A 16-year-old girl with no previous cardiac problems presented to the casualty with septic shock. Investigations revealed endocarditis involving the mitral valve. At the time of surgery, she was noted to have a large left atrial appendage herniating into the left pleural cavity due to partial absence of the pericardium. Complete or partial absence of the pericardium is an uncommon congenital anomaly. Though various complications and presentations are reported, it has not been identified as a risk factor for endocarditis. We discuss the possible mechanism, and need for prophylaxis against endocarditis, in patients with absence of the pericardium.

Over a decade ago, I co-authored a review in Cardiology in the Young regarding neurological outcomes following surgery for congenital cardiac disease.1 In that review, I placed much emphasis on the conduct of cardiopulmonary bypass, and its role in neurodevelopmental disabilities. Much has been learned in the intervening years regarding the multifactorial causes of abnormal school-age development, in particular, the role of prenatal, perioperative, socioeconomic, and genetic influences. In this update, I will highlight some of the recent advances in our understanding of the protean causes of neurological, behavioral, and developmental abnormalities in children and young adults with complex forms of congenital cardiac disease. In addition, I will summarize the current data on patients at particular high-risk for adverse neurodevelopmental outcomes, specifically those with a functionally univentricular heart who have had staged reconstruction with ultimate conversion to the Fontan circulation.

In the United Kingdom, there has been a steady decline in the number of conventional autopsies performed in children. For cardiovascular disease, structural and not pathological considerations are often more important in defining the cause of death. Magnetic resonance imaging is now often used in the assessment of congenital cardiac malformations during life. In this case report, we demonstrate how post-mortem magnetic resonance imaging was able to establish the diagnosis of aortic rupture in a patient who had undergone surgical repair of aortic interruption. In this patient, the parents had declined conventional histopathological autopsy, but were happy to consent to magnetic resonance post-mortem assessment.

Innovation in surgical and medical management of cardiac disease has generated a dramatic improvement in operative survival. Along with these favourable results in terms of survival is the heightened awareness of neurologic complications, which often become evident beyond the early postoperative period. A large, multicentre prospective study found serious neurologic injury occurs in about one-twentieth of patients after myocardial revascularization in adults.1 More subtle evidence of persistent cognitive decline and functional impairment has been shown to occur in over two-fifths of such patients.2 Acute neurologic abnormalities are reported in up to one-fifth of infants and children who undergo cardiac surgery.3–6 Lasting impairments in cognitive, motor, and expressive functioning have been reported in up to three-fifths of children who have undergone complex cardiac surgery during infancy.7 Specifically, gross and fine motor delays, visual-spatial problems, language deficits and long-term emotional and behavioural problems have been found.8–13