Highly sensitised children in need of cardiac transplantation have overall poor outcomes because of increased risk for dysfunction of the cardiac allograft, acute cellular and antibody-mediated rejection, and vasculopathy of the cardiac allograft. Cardiopulmonary bypass and the frequent use of blood products in the operating room and cardiac intensive care unit, as well as the frequent use of homografts, have predisposed potential recipients of transplants to allosensitisation. The expansion in the use of ventricular assist devices and extracorporeal membrane oxygenation has also contributed to increasing rates of allosensitisation in candidates for cardiac transplantation. Antibodies to Human Leukocyte Antigen can be detected before transplantation using several different techniques, the most common being the “complement-dependent lymphocytotoxicity assays”. “Solid-phase assays”, particularly the “Luminex® single antigen bead method”, offer improved specificity and more detailed information regarding specificities of antibodies, leading to improved matching of donors with recipients. Allosensitisation prolongs the time on the waiting list for potential recipients of transplantation and increases the risk of complications and death after transplantation. Aggressive reduction of antibodies to Human Leukocyte Antigen in these high-risk patients is therefore of vital importance for long-term survival of the patient and cardiac allograft. Strategies to decrease Panel Reactive Antibody or percent reactive antibody before transplantation include plasmapheresis, intravenous administration of immunoglobulin, and specific treatment to reduce B-cells, particularly Rituximab. These strategies have resulted in varying degrees of success. Antibody-mediated rejection and cardiac allograft vasculopathy are two of the most important complications of transplantation in patients with high Panel Reactive Antibody. The treatment of antibody-mediated rejection in recipients of cardiac transplants is largely empirical and includes the use of high-dose corticosteroids, plasmapheresis, intravenous administration of immunoglobulins, anti-thymocyte globulin, and Rituximab. Cardiac allograft vasculopathy is believed to be secondary to chronic complement-mediated endothelial injury and chronic vascular rejection. The use of proliferation signal inhibitors, such as sirolimus and everolimus, has been shown to delay the progression of cardiac allograft vasculopathy. In some non-sensitised recipients of cardiac transplants, the de novo formation of antibodies to Human Leukocyte Antigen after transplantation may increase the likelihood of adverse clinical outcomes. The use of serial testing for donor-specific antibodies after cardiac transplantation may be advisable in patients with frequent episodes of rejection and patients with history of sensitisation. Allosensitisation before transplantation can negatively influence outcomes after transplantation. A high incidence of antibody-mediated rejection and graft vasculopathy can result in graft failure and decreased survival. Current strategies to decrease allosensitisation have helped to expand the pool of donors, improve times on the waiting list, and decrease mortality. Centres of transplantation offering desensitisation are currently using plasmapheresis to remove circulating antibodies; intravenous immunoglobulin to inactivate antibodies; cyclophosphamide to suppress B-cell proliferation; and Rituximab to deplete B-lymphocytes. Similar approaches are also used to treat antibody-mediated rejection after transplantation with promising results.

Brugada syndrome is a channelopathy characterised electrocardiographically by distinctive coved ST-segment elevation in the right precordial leads and is associated with a predisposition for sudden death secondary to ventricular arrhythmias in otherwise healthy patients. Previously known as Brugada-like patterns, Brugada phenocopies include agents and conditions that mimic true Brugada syndrome, presenting with an acquired Brugada Type-1 ECG pattern. We describe the first reported case of a 17-month-old female with an asymptomatic rhabdomyoma of the interventricular septum that presented as a Brugada phenocopy.

The Rastelli operation has been the most common procedure for the repair of transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction. A relatively recent approach is the Nikaidoh procedure. Despite the fact that it seems promising, the operation lacks long-term follow-up data. It has been postulated that patients with anomalous coronary arteries are high-risk candidates for the Nikaidoh procedure and its modifications. In this report, we present the case of a patient with transposition of the great arteries with remote restrictive ventricular septal defect and left ventricular outflow tract obstruction with coronary anomaly – with the right coronary artery originating from the left anterior descending coronary artery and crossing the right ventricular outflow tract – who underwent successful modified Nikaidoh operation.

Danon disease is a rare entity associated with the clinical triad of mental retardation, skeletal myopathy, and severe hypertrophic cardiomyopathy. We report two cases of Danon disease and describe the results of the cardiac magnetic resonance imaging studies that were conducted to assess the pattern of cardiac hypertrophy.

In utero diagnosis of complex progressive cardiac disease such as hypoplastic left heart syndrome presents a novel opportunity for antepartum, intrapartum, and neonatal management. The clinical possibilities and potential for differing outcomes challenge the mother–foetus dyad with regard to informed consent. Previous studies reveal that rates of termination of pregnancy for foetuses with hypoplastic left heart syndrome vary widely in the United States and Europe, leading us to surmise that informed consent may be practised differently. The purpose of this paper is to review the ethical considerations and physician responsibilities of informed consent as they relate to prenatal and postnatal patients with hypoplastic left heart syndrome. Special consideration is paid to the informed consent process as practised by the obstetrician, perinatologist, paediatric cardiologist, and paediatric cardiac surgeon as it relates to termination of pregnancy, comfort care, and surgical palliation. We will argue that informed consent as it relates to hypoplastic left heart syndrome is far from standardised and that there exists a state of bioethical equipoise concerning the extent and limits of its application in the current clinical setting.

Primary cardiac tumours in children are rare. Despite the majority of these tumours being benign, they may cause blood flow obstruction, ventricular dysfunction, and life-threatening arrhythmias. We present a case of a 4-year-old boy with repeated presyncope episodes in whom we found a large left ventricular fibroma. Our case emphasises that surgical resection is safely feasible even with large fibromas, avoiding cardiac transplantation in childhood.

We report a retrograde approach for the successful closure of large left coronary artery fistulas in the cases of two children using the Amplatzer vascular plug. This method simplified the procedure by eliminating the need for making an arteriovenous loop.

Cardiac strangulation from epicardial pacemaker leads is a rare event that can be difficult to recognise and can cause serious complications such as cardiac failure or death. We describe a 3-year-old girl who received an epicardial pacing system as a neonate for complete congenital cardiac block and developed cardiac strangulation from the leads. The clinical presentation modes are reviewed and technical aspects for lead and generator positioning are discussed.

An infant with cyanotic cardiac disease that was palliated with a bidirectional cavopulmonary shunt developed progressive and worsening cyanosis 5 years after the surgical procedure. A large venous collateral was found to be decompressing the bidirectional Glenn shunt from the superior caval vein to the inferior caval vein and was percutaneously closed with a vascular plug. The unusually large venous collateral, and the excellent outcome associated with percutaneous procedure are discussed.

We report an adolescent with a benign cardiac haemangioma with attachments exclusively to the anterior leaflet of the mitral valve. On the basis of our review of the literature, this study has not been reported previously.

Although recent advances have helped identify cases where foetal cardiac surgery might reverse the development of certain lesions, the indications and measurement of success in these procedures have yet to be established. Thus, both patients and physicians have a “burden of knowledge”, whereby a diagnosis is made without a clear course of action. The profound issues raised by foetal intervention, specifically the question of how concepts such as “patient” and “success” can be used, complicate this burden further and test the limits of language and logic. Similar issues raised in postmodern philosophy are discussed and can be incorporated into foetal cardiac surgery dialogues to produce a multi-disciplinary approach that will elucidate, not obfuscate, these issues in the future.

In a girl suffering from “Scimitar syndrome”, a rerouting of the scimitar vein was performed at the age of 6 years, but no embolisation of the aberrant systemic vessel was done. She presented with recurring respiratory problems 13 years later. An angiography revealed an invert flow from the aberrant systemic vessel via the right pulmonary artery into the left pulmonary artery. After pneumonectomy, she recovered well.

Almost half of the children with symptomatic dilated cardiomyopathy receive a transplant or die within 2 years; however, cardiac stem cell transplantation has become a promising therapeutic option. The present case demonstrates for the first time, to our knowledge, the intramyocardial administration of autologous bone marrow mononuclear cells in a critically ill 4-month-old child with severe dilated cardiomyopathy. Left ventricular ejection fraction increased from 20% before stem cell transplantation to 41% at 4 months of follow-up.

We describe a case of severe aortic stenosis in a 16-year-old male with Hurler's syndrome who had prior bone marrow transplantation. The excised aortic valve leaflets showed characteristic pathologic findings of Hurler's syndrome. This is the first case report of aortic valve replacement in a patient with Hurler's syndrome treated with bone marrow transplantation that demonstrates progression of the aortic valve disease despite treatment.