A 20-month-old girl presented with severe dilated cardiomyopathy and decompensated congestive cardiac failure. Despite escalating inotropic and mechanical ventilation support, she required placement on extracorporeal membrane oxygenation and transfer to the transplant centre in Newcastle, England. She was placed on biventricular assist device and then Berlin Heart but failed to show any recovery of ventricular function. She underwent orthotopic heart transplantation at 2 years of age. She developed bacterial endocarditis with Enterococcus faecalis resulting in severe aortic valve regurgitation requiring aortic valve replacement with a 19 mm On-X valve (Airtivion) 11 days after her transplant. Given the size of the donor heart, it was possible to implant a 19-mm valve in this 12 kg child with minimal risk of patient prosthesis mismatch. She was anticoagulated with warfarin (On-X valve INR 2-3 for first 3 months; INR 1.5-2.0 thereafter). Although she suffered several other post-operative complications, including malabsorption, nasojejunal feeding, liver dysfunction, vertebral fractures, renal impairment and renal calcification, and need for repeat opening of her tracheostomy site following her initial decannulation, her aortic valve function has remained stable.

Heart failure due to dilated cardiomyopathy is a major indication for paediatric cardiac transplantation. Endocardial fibroelastosis is a recognised pathological finding of unknown prognostic significance in paediatric dilated cardiomyopathy. To evaluate the nature of the association between left ventricular endocardial fibroelastosis and paediatric dilated cardiomyopathy, we reviewed surgical pathology reports of dilated cardiomyopathy explants (1986–2016) in order to characterise the pathological findings and to compare and contrast their frequency among four age groups: less than 1 year; 1–5 years; 6–10 years; and greater than 11 years. The 89 explants (47 males and 42 females) were all characterised by increased weight and left ventricular chamber dilatation without increased wall thickness. Ninety-five per cent of the specimens in the two youngest subsets had left ventricular endocardial fibroelastosis. Compared to the oldest age group, recipients aged 1–5 years had a 6-fold increase and those younger than 1 year a 19-fold increase in the odds of observing left ventricular endocardial fibroelastosis. Explants with and without endocardial fibroelastosis were otherwise phenotypically similar. In paediatric dilated cardiomyopathy endocardial fibroelastosis is a very common pathological finding, especially in infants and young children. We propose that the descriptive, clinico-pathological designation “Dilated Cardiomyopathy with Endocardial Fibroelastosis” should be adopted to facilitate future investigation into the potential prognostic/therapeutic significance of left ventricular endocardial fibroelastosis.

In view of the increasing complexity of both cardiovascular implantable electronic devices (CIEDs) and patients in the current era, practice guidelines, by necessity, have become increasingly specific. This document is an expert consensus statement that has been developed to update and further delineate indications and management of CIEDs in pediatric patients, defined as ≤21 years of age, and is intended to focus primarily on the indications for CIEDs in the setting of specific disease categories. The document also highlights variations between previously published adult and pediatric CIED recommendations and provides rationale for underlying important differences. The document addresses some of the deterrents to CIED access in low- and middle-income countries and strategies to circumvent them. The document sections were divided up and drafted by the writing committee members according to their expertise. The recommendations represent the consensus opinion of the entire writing committee, graded by class of recommendation and level of evidence. Several questions addressed in this document either do not lend themselves to clinical trials or are rare disease entities, and in these instances recommendations are based on consensus expert opinion. Furthermore, specific recommendations, even when supported by substantial data, do not replace the need for clinical judgment and patient-specific decision-making. The recommendations were opened for public comment to Pediatric and Congenital Electrophysiology Society (PACES) members and underwent external review by the scientific and clinical document committee of the Heart Rhythm Society (HRS), the science advisory and coordinating committee of the American Heart Association (AHA), the American College of Cardiology (ACC), and the Association for European Paediatric and Congenital Cardiology (AEPC). The document received endorsement by all the collaborators and the Asia Pacific Heart Rhythm Society (APHRS), the Indian Heart Rhythm Society (IHRS), and the Latin American Heart Rhythm Society (LAHRS). This document is expected to provide support for clinicians and patients to allow for appropriate CIED use, appropriate CIED management, and appropriate CIED follow-up in pediatric patients.

This series describes three adolescent females who presented with chest pain and ventricular dysfunction related to acute coronary ischemia secondary to Takayasu’s arteritis with varied courses of disease progression leading to a diverse range of therapies including cardiac transplantation. While Takayasu’s arteritis is rare in childhood, it should be strongly considered in any adolescent female presenting with systemic inflammation and chest pain consistent with myocardial infarction. A high index of suspicion can lead to early detection and aggressive management of the underlying vasculitis reducing associated morbidity and mortality. The purpose of this report is to describe the challenges in the clinical diagnosis and management of Takayasu’s arteritis with myocardial infarction. We also seek to enhance awareness about unique presentations of Takayasu’s arteritis within the paediatric community.

For most patients, adenoviruses cause few acute health concerns and are often self-limiting. Patients who are immunocompromised or immunosuppressed, however, are at risk for disseminated adenovirus and suffer high morbidity and mortality, without well-defined treatment options. We report the case of a 9-month-old boy who was successfully treated for disseminated adenovirus infection with intravenous immunoglobulin and cidofovir 3 months post heart transplant, tailored to serum adenoviral load and clinical response. We emphasise the importance of early identification, monitoring, and a potentially novel treatment in the paediatric cardiac transplant population with disseminated adenovirus infection.

Arterial switch operation has become the standard of care for d-transposition of great arteries and has excellent short- and long-term outcomes. We report the case of a newborn with a diagnosis of d-transposition of great arteries with intact ventricular septum and a low-risk coronary artery anatomy who developed coronary artery vasospasm while coming off bypass following arterial switch operation in the operating room. The coronary artery spasm led to severe biventricular dysfunction and need for extracorporeal membranous oxygenation support. Despite extracorporeal membranous oxygenation and inotropic support, there was no improvement in the left ventricular function, and cardiac transplantation was performed after 8 days. The explanted heart showed extensive infarction of both ventricles. Both the coronary ostei were patent with no evidence of thrombus, suggesting coronary artery vasospasm rather than embolus or thrombus formation. This is the first case of coronary artery vasospasm in a neonate with d-transposition of great arteries leading to cardiac transplantation. We speculate that early identification of patients who are at a high risk for coronary vasospasm and prophylactic or timely infusion of papaverine directly into the coronary arteries may be beneficial in this condition.

In the United States alone, ∼14,000 children are hospitalised annually with acute heart failure. The science and art of caring for these patients continues to evolve. The International Pediatric Heart Failure Summit of Johns Hopkins All Children’s Heart Institute was held on February 4 and 5, 2015. The 2015 International Pediatric Heart Failure Summit of Johns Hopkins All Children’s Heart Institute was funded through the Andrews/Daicoff Cardiovascular Program Endowment, a philanthropic collaboration between All Children’s Hospital and the Morsani College of Medicine at the University of South Florida (USF). Sponsored by All Children’s Hospital Andrews/Daicoff Cardiovascular Program, the International Pediatric Heart Failure Summit assembled leaders in clinical and scientific disciplines related to paediatric heart failure and created a multi-disciplinary “think-tank”. The purpose of this manuscript is to summarise the lessons from the 2015 International Pediatric Heart Failure Summit of Johns Hopkins All Children’s Heart Institute, to describe the “state of the art” of the treatment of paediatric cardiac failure, and to discuss future directions for research in the domain of paediatric cardiac failure.

In the United States of America alone, ~14,000 children are hospitalised annually with acute heart failure. The science and art of caring for these patients continues to evolve.

The International Pediatric Heart Failure Summit of Johns Hopkins All Children’s Heart Institute was held on 4 and 5 February, 2015. The 2015 International Pediatric Heart Failure Summit of Johns Hopkins All Children’s Heart Institute was funded through the Andrews/Daicoff Cardiovascular Program Endowment, a philanthropic collaboration between All Children’s Hospital and the Morsani College of Medicine at the University of South Florida (USF). Sponsored by All Children’s Hospital Andrews/Daicoff Cardiovascular Program, the Johns Hopkins All Children’s Heart Institute International Pediatric Heart Failure Summit assembled leaders in clinical and scientific disciplines related to paediatric heart failure and created a multi-disciplinary “think-tank”. Information about George R. Daicoff, MD, and Ed and Sarainne Andrews is provided in this introductory manuscript to the 2015 Supplement to Cardiology in the Young entitled: “Proceedings of the 2015 International Pediatric Heart Failure Summit of Johns Hopkins All Children’s Heart Institute”. Dr Daicoff founded the All Children’s Hospital Pediatric Heart Surgery programme and directed this programme for over two decades. Sarainne Andrews made her generous bequest to All Children’s Hospital in honour of her husband Ed and his friendship with Dr Daicoff in order to support cardiovascular surgery research efforts.

Donald Nixon Ross, FRCS (4 October 1922 to 7 July 2014) was a South African-born British cardiothoracic surgeon, who developed the pulmonary autograft, known as the Ross procedure, for the treatment of aortic valve disease, and also performed the first heart transplant in the United Kingdom in 1968. This paper, written by Jane Somerville, Professor of Cardiology [Retired], Imperial College London, London, United Kingdom, provides the personal recollections about Donald Ross from Jane Somerville, and thus provides a unique snapshot of cardiac surgical history.

A 7-year-old male patient who had abdominal swelling and eyelid oedema was diagnosed with restrictive cardiomyopathy. His serum albumin level was 2.3 g/dl. Protein-losing enteropathy due to restrictive cardiomyopathy was diagnosed and oral budesonide was started. His serum albumin level began to rise and ascites and peripheric oedema disappeared. The patient underwent a successful cardiac transplantation and budesonide was stopped. After the heart transplantation, the albumin level decreased to 2.3 g/dl, and therefore it was restarted. When the serum albumin level increased, the budesonide dose was tapered and stopped in 1 month. Budesonide may be an effective drug in patients with protein-losing enteropathy due to heart failure.

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.

We describe a recipient of an orthotopic cardiac transplant who developed severe ventricular dysfunction following an episode of sepsis. He had normal cardiac biopsies and responded to treatment with thiamine. Causes other than rejection should be considered in patients who have received a cardiac transplant and who present in cardiac failure.

Isolated noncompaction of the myocardium, also known as “spongy myocardium”, is a rare disease in children and adults. It is suggested that, during early development of the heart, the primary spongy structure persists due to an arrest of compaction. No other cardiac malformations are found, but there are familial occurences, relations to genetic disorders or syndromes such as Melnick-needles-syndrome or Xq28-linked cardiomyopathy, and reports of conduction disorders.

We have now diagnosed isolated noncompaction in seven children aged between five weeks and 5.5 years. Three are doing well with anticongestive therapy, while transplantation of the heart was performed in one. Three of the children have died, but in only one case due to cardiac failure.

Our experience emphasises the need rapidly to establish the diagnosis, to search for associated extracardiac abnormalities, and to consider transplantation at an early stage.

Endomyocardial biopsy in children remains important in the evaluation of cardiomyopathy, myocarditis, and rejection following cardiac transplantation. We sought to evaluate the morbidity associated with endomyocardial biopsy on the basis of a large experience from a single institution.

We reviewed records of all endomyocardial biopsies performed at our institution. Complications were defined as adverse events resulting from the biopsy requiring intervention or additional observation. We also reviewed the most recent echocardiogram from all the patients for the presence and severity of tricuspid valvar regurgitation.

Between November 1986 and April 2002, we performed 1051 endomyocardial biopsies in 135 patients ranging in age from 9 days to 18 years. The internal jugular vein was the site of vascular access in 68% of the procedures. There were 10 acute procedural complications requiring intervention or additional observation. Severe tricuspid regurgitation developed in two patients who had undergone multiple biopsies after cardiac transplantation, one of whom underwent subsequent replacement of the tricuspid valve. There were no deaths or cardiac perforations. The total incidence of morbidity was 1.1%. No demographic or procedural factors were identified to be predictive of complications.

In experienced hands, therefore, endomyocardial biopsy can be safely performed in children with very low morbidity.