We present the rare case of lipomatous atrial septal hypertrophy associated with adrenocorticotropin hormone therapy in an infant with West syndrome, highlighting their relatively benign nature and good prognosis in children, and the relevance of the differential diagnosis with more dangerous cardiac masses in order to avoid aggressive diagnostic and therapeutic interventions.

Nationwide registry data of patients with single-ventricle physiology have been rarely reported. The Medical Aid Program for Chronic Pediatric Diseases of Specified Categories (Japan) has contributed to the financial support of medical expense for patients younger than 20 years with chronic paediatric disease, and almost all children in Japan who require disease-specific treatment voluntarily apply to this programme. The epidemiology and medium- to long-term outcome of patients following a Fontan procedure were investigated using the database. The usefulness of this epidemiologic investigation in identifying real-world objectives and clinical applications was also examined. A total of 2862 patients who underwent a Fontan operation were identified from 18,589 patients with chronic heart disease registered to the medical aid programme. The details of symptoms, treatment, and somatic growth were evaluated, from which we were able to clarify the nationwide data regarding the current status of post-Fontan patients younger than 20 years. This study elucidated the current status of post-Fontan patients under 20 years of age in Japan. Data analysis of the Medical Aid Program for Chronic Pediatric Diseases of Specified Categories cohort provided useful information towards understanding the comprehensive status of patients with chronic heart disease and contributed to improved disease management.

Congenitally corrected transposition of the great arteries is a rare form of CHD. Situs inversus is a much less common variant of a congenitally corrected transposition of the great arteries. In rare cases, transposition events may be accompanied by various cardiac anomalies. However, situs inversus patients with congenitally corrected transposition, single coronary artery anomaly, and atrioventricular block together have not been reported previously. This combination of abnormalities is presented as a first in the literature.

Over the past decades, survival of patients with CHD improved significantly, making it a life-cycle disease. Hence, there is a need for a workforce that can take up the care for afflicted individuals in the different phases of the life spectrum. Each life phase is associated with specific challenges. Topics that should receive more attention in clinical care or in CHD research are parenting styles of parents of children, transfer and transition of adolescents, cumulative burden of injury in the brain in adults, and geriatric care for older persons with CHD. Nurses, along with other healthcare professionals, will play a pivotal role in building up expertise in these areas and taking up these challenges.

Aorto-left ventricular tunnel is a rare congenital heart lesion, with an incidence of <0.1% of all CHD. We present a unique case of a valvar aorto-left ventricular tunnel in a neonate, in belief that our findings may shed some light on the developmental origins of this lesion.

In this review, I will address the problem as to whether children with disease of the aortic valve should undergo repair, or be put forward for the Ross procedure. So as to put this debate in the correct framework, we need to evaluate all the options for aortic valvar disease. These include not only the Ross procedure, involving the insertion of a pulmonary autograft, and valvar repair, but also the option of replacing the valve with a biological or mechanical prosthesis.

Vasospastic angina is extremely uncommon for adolescents to experience chest discomfort, which is defined by transitory ST segment elevation or depression and angina symptoms that occur while at rest. It may result in potentially fatal conditions like myocardial infarction, ventricular fibrillation, or even sudden cardiac arrest. To aim of this article is to report a very rare case of a 17-year-old male Afghan refugee who was diagnosed with vasospastic angina after presenting with chest pain, and after receiving calcium channel blocker and nitrates for medical therapy, there were no angina attacks. Our case underlines the value of a thorough evaluation of adolescent’s chest pain, the need to diagnose based on the symptoms, and the necessity of performing coronary angiography to rule out coronary causes when there is a high suspicion to a cardiac cause.

I have previously reported the successful use of the 0.9 millimetre excimer laser catheter in 2 neonates with pulmonary valvar atresia and intact ventricular septum. In this report, I describe the expanded role of the excimer laser in a premature infant weighing 1.8 kilograms who presented with an occluded right pulmonary artery secondary to an organized thrombus. Successful reconstruction of the right pulmonary artery was performed by sequential use of lasers of 0.9, 1.4, and 2 millimetres diameter, followed by conventional balloon angioplasty. As is now the situation in adults, laser catheter intervention should now be an integral part of the armamentarium of the paediatric cardiac interventionalist.

We report a case of combined severe aortic stenosis and regurgitation in a pregnant patient with a history of congenital bicuspid aortic valve. The patient presented at 22 weeks of gestation with angina and pre-syncopal symptoms. During her admission, she experienced intermittent episodes of non-sustained ventricular tachycardia and hypotension. A multi-disciplinary healthcare team was assembled to decide on the appropriate medical and surgical treatment options. At 28 weeks of gestation, the patient underwent a caesarean delivery immediately followed by a mechanical aortic valve replacement.

Serelaxin has been studied in trials in adults with acute heart failure, but not in children. We report the first compassionate use of serelaxin in an infant. A 6-month-old girl with dilated cardiomyopathy was placed on extracorporeal membrane oxygenation following cardiac arrest unresponsive to medical treatment. Extracorporeal membrane oxygenation weaning failed despite maximal ino-dilator therapy. During the 48-hour infusion of serelaxin, we observed marked improvement in brain natriuretic peptide, left ventricular systolic function, and dilatation. The patient was successfully weaned from extracorporeal membrane oxygenation 24 hours later. The child died after a second extracorporeal membrane oxygenation run owing to sepsis.

Pseudoaneurysms are rare complications of Blalock–Taussig fistulas. We present the case of an abscessed right pulmonary aneurysm after a Blalock–Taussig fistula in the context of a Salmonella bacteremia.

Extra-corporeal membrane oxygenation is a life-saving modality to support the cardiac and/or pulmonary system as a form of life support in resuscitation, post-cardiotomy, as a bridge to cardiac transplantation and in respiratory failure. Its use in the paediatric and neonatal population has proven incredibly useful. However, extra-corporeal membrane oxygenation is also associated with a greater rate of mortality and complications, particularly in those with co-morbidities. As a result, interventions such as ventricular assist devices have been trialled in these patients. In this review, we provide a comprehensive analysis of the current literature on extra-corporeal membrane oxygenation for cardiac support in the paediatric and neonatal population. We evaluate its effectiveness in comparison to other forms of mechanical circulatory support and focus on areas for future development.

A rare case of a patient with a large type IV aortico–left ventricular tunnel who underwent successful repair at 1 day after birth was described. To the best of our knowledge, no such cases that aortic opening was over 10 mm have been reported in the English literature. Our case demonstrated no leak or significant aortic regurgitation by a two-patch repair.

We describe the case of an infant with DiGeorge syndrome born with a right aortic arch and left arterial duct. Despite the remote location of the right aortic arch from the left arterial duct, he developed coarctation of the aorta during treatment with indomethacin. This was relieved by prostaglandin treatment. This case highlights the fact that, even in the absence of an arterial duct, ductal tissue can still be present in the aorta, and cause coarctation when exposed to indomethacin. We also demonstrate the utility of prostaglandin for relief of this type of obstruction.