Allergic reactions related to drug use is a common entity presenting often from minor urticaria to life-threatening anaphylactoid reactions. A common but easily overlooked diagnosis, Kounis syndrome, is an established hypersensitivity coronary disorder induced by drugs, foods, environmental factors, and coronary stents that can present in the same way as non-allergy-induced acute coronary syndrome

Neonatal Marfan syndrome is a rare condition with poor prognosis because of severe mitral and/or tricuspid valve insufficiency. Mitral valve replacement is sometimes required in early infancy, while tricuspid valve replacement is rarely done. We report the first infant neonatal Marfan syndrome case with a missense variant of c.3706T>C in the fibrillin-1 gene that was successfully managed by mitral

Objectives: There are several studies reporting the outcomes of hypoplastic aortic arch and aortic coarctation repair with combination of techniques. However, only few studies reported of aortic arch and coarctation repair using a homograft patch through sternotomy and circulatory arrest with retrograde cerebral perfusion. We report our experience and outcomes of this cohort of neonates and infants

Pulmonary atresia with ventricular septal defect is a complex congenital cardiac anomaly. The blood is supplied to the lungs through a patent ductus arteriosus, a major aortopulmonary collateral artery, or in very rare cases from a coronary artery–pulmonary artery fistula. We present two cases with coronary artery–pulmonary artery fistula which underwent surgical intervention. In our first patient

Despite acceptable survival for Fontan operation, there are concerns about late complications affecting the major organs. We herein present two cases of adults after Fontan operation who developed focal segmental glomerulosclerosis. These cases suggest that focal segmental glomerulosclerosis is owing to haemodynamic incompetence associated with Fontan operation, including congestion, hypoxia, and hyperviscosity

Background: Diagnosis of sinus venosus defects, not infrequently associated with complex anomalous pulmonary venous drainage, may be delayed requiring multimodality imaging. Methods: Retrospective review of all patients from February 2008 to January 2019. Results: Thirty-seven children were diagnosed at a median age of 4.2 years (range 0.5−15.5 years). In 32 of 37 (86%) patients, diagnosis was achieved

Nicolaides–Baraitser syndrome is a rare, neuro-developmental disorder caused by heterozygous pathogenic variants in the SMARCA2 gene, involved with chromatin regulation. Cardinal features include intellectual disability, short stature, microcephaly, triangular facies, sparse hair, brachydactyly, prominent interphalangeal joints and seizures. Genetic testing demonstrated a loss within SMARCA2 at 9p24

We report on a 7-month-old male with transient phrenic nerve palsy induced by diagnostic cardiac catheterisation. The phrenic nerve palsy, which is a rare complication, was due to extravascular bleeding from a branch of the internal mammary artery.

We report a 5-month-old infant who developed an unexpected acute ischaemia of the right lower limb following a surgical perimembranous interventricular communication closure. This rare case of ischaemia was due to an occlusive right common iliac artery dissection. It was early managed by angioplasty with two ABSORB® bioresorbable stents, resulted in complete revascularisation of the right leg.

We report a series of four patients with CHD who tested positive for COVID-19, got treated, and underwent cardiac surgery in the same sitting. All had an uneventful perioperative course similar to COVID-19-negative patients. We conclude that children with asymptomatic or mild COVID-19 disease may be subjected to surgery as early as within 1 week since COVID-19 negative.

Ebstein malformation of tricuspid valve is a congenital disease of tricuspid valve with associated right ventricular cardiomyopathy. Hypertrophic cardiomyopathy is a form of inherited left ventricular cardiomyopathy caused by sarcomeric protein gene mutations with inherent risks of sudden cardiac death. Here we report a rare case with co-occurrence of Ebstein malformation of tricuspid valve and hypertrophic

Background: In this era of public scrutiny, there is an ongoing need for innovative methods for patient follow-up. Objectives: As part of a quality initiative, we developed an automated post-operative follow-up system for patients following discharge after cardiac surgery at Boston Children’s Hospital. Methods: Discharge Communication (DisCo) is a web-based system developed at Boston Children’s Hospital

Combined tricuspid atresia and absent pulmonary valve with dysplasia of the right ventricular myocardium is a very rare congenital heart anomaly with a poor prognosis. We present three cases of this rare disease that reached the Fontan operation without prior surgical intervention of the right ventricle or pulmonary artery; no right ventriclar dilation was detected. All patients had uneventful post-operative

A boy diagnosed with persistent truncus arteriosus and severe truncal valve regurgitation had uncontrollable heart failure. Thus, truncal valve repair, ventricular septal defect closure, and right ventricular outflow tract reconstruction were performed on the second day of life. We report about a neonate with severe truncal valve regurgitation who was successfully treated with autologous pericardial

Objective: This study was conducted to evaluate the persisting Covid-19-related symptoms of the cases included in our study and to assess their cardiac findings to determine the impact of Covid-19 on children’s cardiovascular health. Methods: In this study, 121 children between the ages of 0– and 18 with Covid-19 were evaluated based on their history, blood pressure values, and electrocardiography

Children with cardiac diseases suffer from significant morbidity and mortality secondary to thromboembolic complications. Anticoagulant agents currently used for thromboprophylaxis have many limitations, including subcutaneous administration (low molecular weight heparins) and requirement for frequent monitoring via venipuncture (vitamin K antagonists). Edoxaban is an oral direct factor Xa inhibitor

Cardiac surgery for CHD was pioneered in Washington, DC by Charles Hufnagel and Edgar Davis working at Georgetown University and Children’s Hospital of the District of Columbia. Children’s Hospital, now Children’s National Hospital, had been established just 5 years after the end of the Civil War. In the 1950s, Davis and Hufnagel undertook many open-heart operations using the technique of surface cooling

Background: Increased risk donors in paediatric heart transplantation have characteristics that may increase the risk of infectious disease transmission despite negative serologic testing. However, the risk of disease transmission is low, and refusing an IRD offer may increase waitlist mortality. We sought to determine the risks of declining an initial IRD organ offer. Methods and results: We performed

The growing unmet demand for suitable organ donors increases each year. Despite relative contraindications for thoracic organ donation after previous cardiac surgery, experienced programmes and surgeons can successfully utilise the lungs from select donors who have undergone prior cardiac surgery. This is the first reported case of double lung en bloc procurement from a donor who had a previous arterial

Fontan palliation represents one of the most remarkable surgical advances in the management of individuals born with functionally univentricular physiology. The operation secures adult survival for all but a few with unfavourable anatomy and/or physiology. Inherent to the physiology is passive transpulmonary blood flow, which produces a vulnerability to adequate filling of the systemic ventricle at

NPC-QIC Registry data showed that angiotensin-converting enzyme inhibitors were described in almost 38% for patients with single ventricle physiology after stage-I Norwood palliation. However, mortality and ventricular dysfunction or atrioventricular valve insufficiency seems to be not improved by oral application of angiotensin-converting enzyme inhibitors. The final conclusion was that despite limited

Background: The usefulness of ultra-fast track cardiac anaesthesia may give great benefits to patients; however, its usefulness has not been completely evaluated in infants and toddlers, who are generally considered the most difficult group for ultra-fast track cardiac anaesthesia. Method: A total of 130 children were allocated randomly into to a ultra-fast track cardiac anaesthesia group (Group D)

Background: Coronary artery aneurysms are well-described in Kawasaki disease and the Multisystem Inflammatory Syndrome in Children and are graded using Z scores. Three Z score systems (Boston, Montreal, and DC) are widely used in North America. The recent Pediatric Heart Network Z score system is derived from the largest diverse sample to-date. The impact of Z score system on the rate of coronary dilation

Ductal dissection is rarely mentioned but intractable complication of ductal stenting. We demonstrate the dissection image under fluoroscopy and the use of a relatively large stent as an applicable treatment.

Chiari network is an infrequently visualized web-like structure in the right atrium that is usually thin and small. Rarely, it can be prominent and elongated with protrusion into the right ventricle during diastole and complications have been reported. Thirty-eight patients (median age 2.5 years) with prolapsing Chiari network were identified and associated cardiac abnormalities documented. Echocardiographic

A 4-hour-old infant with profound cyanosis on an alprostadil infusion was urgently transferred to Rady Children’s Hospital with suspected CHD. Upon arrival, urgent echocardiography was performed but could not confirm the presence of discrete pulmonary veins or pulmonary venous drainage. Given the difficulty in delineating the anatomy, a cardiac CT scan was performed and demonstrated a nearly atretic

Objectives: To compare patients treated for incomplete Kawasaki disease whose practitioners followed versus did not follow American Heart Association criteria and to evaluate the association of cardiology consultation with adherence to these guidelines. Study design: Single centre retrospective cohort study of patients <18 years old who received ≥1 dose of intravenous immunoglobulin for Kawasaki disease

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

Scimitar syndrome is a rare variant of anomalous right pulmonary vein connection to the inferior vena cava and it is associated with other cardiopulmonary anomalies. It generally requires surgery and sometimes it may go unrecognised into adulthood. We report a unique case of a scimitar syndrome variant in a young adult, who was successfully treated percutaneously, after the first misdiagnosis of arrhythmogenic

Background: Children with CHD are at risk for obesity and low levels of activity. These factors are associated with an increased risk of poor outcome. Participation in organised sports is an important avenue for children to maintain physical activity, though the relationship between sports participation and obesity has not been examined in the Fontan population. Methods: We performed a cross-sectional

Background and aim: Heterotaxy syndrome, being right atrial isomerism (RAI) or left atrial isomerism (LAI), often presents with Congenital Heart Disease (CHD). Intestinal abnormalities, including malrotation are common. We assessed the spectrum of gut abnormalities and their impact on medium-term outcome in a cohort of patients with fetal and postnatal diagnoses of heterotaxy syndrome. Methods: We

Isolated ventricular inversion with situs solitus is a severe and rare congenital cardiac malformation characterised by an atrioventricular discordance but with ventriculo-arterial concordance. Here, we present the rare case of an adolescent with isolated ventricular inversion and hypoplasia of the left-sided morphological right ventricle and pulmonary stenosis, a first of its kind to be reported in

Background: Loss of follow-up is a barrier to providing adequate care to paediatric cardiac patients. The purpose of this study was to determine variables associated with loss of appropriate paediatric cardiology follow-up, including potentially modifiable factors. We hypothesised having earlier recommend follow-up intervals was associated with less likelihood of loss of follow-up. Methods: We performed

Acute stent thrombosis may complicate neonatal arterial duct stenting for reduced pulmonary blood flow. Thrombolytic agents recanalise the clot but may cause bleeding around the vascular sheaths and other sites. Since early thrombus is platelet mediated, intravenous platelet glycoprotein inhibitor like eptifibatide is likely to be effective, but rarely utilised in neonates. Ductal stent thrombosis

Background: Acute rheumatic fever in childhood continues to cause serious morbidity despite all developments. The objective of this study was to evaluate the clinical and laboratory characteristics of patients with acute rheumatic fever and to determine the frequency of subclinical carditis and the side effects of the drugs used in the treatment. Methods: The data of patients hospitalised between 2008

Introduction: Fenestration in the total cavopulmonary connection system may improve the outcome of patients with significant risk factors for Fontan haemodynamics. Our study aims to analyse the difference in long-term survival between non-fenestrated and fenestrated patients. Methods: All consecutive patients (n = 351) who underwent total cavopulmonary connection between 1992 and 2016 were identified

Anomalous origin of the left coronary artery from pulmonary artery constitutes 0.5% of all CHD (Boutsikou M, Shore D, Li W, et al, Int J Cardiol 261: 49–53, 2018). Fifteen percent survive into adulthood undiagnosed and 90% present with sudden death (Yau JM, Singh R, Halpern EJ, Fischman D, Clin Cardiol 34: 204–210, 2011; Alexi-Meskishvili V, Berger F, Weng Y, Lange PE, Hetzer R, J Card Surg 10: 309–315

Objectives: Prenatal maternal stress is associated with adverse offspring outcomes, which may be mediated by maternal stress hormones. However, evidence supporting the association between maternal stress and cortisol levels in high-risk pregnancies is limited. This study aims to determine the relationship between self-reported maternal mental distress and maternal salivary cortisol levels in pregnancies

Introduction: Nucleated red blood cells (NRBCs) are immature red cells that under normal conditions are not present in the peripheral circulation. Several studies have suggested an association between elevated NRBC and poor outcome in critically ill adults and neonates. We sought to determine if elevations in NRBC value following cardiac surgery and following clinical events during the hospital stay

Background: We aimed to characterise the impact of Down syndrome on myocardial performance and loading conditions in infants with Down syndrome and CHD over the peri-operative period by comparing them with infants matched for cardiac lesion with a normal microarray. Methods: Left ventricular global longitudinal strain, right ventricular free wall longitudinal strain, left ventricular end-systolic wall

Cardiac fibromas are the second most common benign primary tumour of the heart in the children; the clinical features include chest pain, arrhythmia, low cardiac output due to outflow tract obstruction, and sudden cardiac death. Sports are associated with an increased risk for sudden death in athletes who are affected by cardiovascular conditions predisposing to life-threatening arrhythmias. We present

Percutaneous coronary intervention for stenosis of coronary artery after Kawasaki disease presents various challenges. The diameters of reference vessels and femoral artery in children are smaller, and the morphological changes are different from adults. Herein, we describe our successful experience with a severe coronary artery stenosis at the proximal portion of left anterior descending treated with

Objective: To investigate the risk factors associated with prolonged ventilation after Fontan surgery. Design: Retrospective case series. Setting: Tertiary childrens hospital. Patients: We included 123 children who underwent Fontan surgery without delayed sternal closure or extracorporeal membrane oxygenation between 2011 and 2017. Intervention: Fontan surgery. Measurements and main results: Prolonged

Pompe disease results from lysosomal acid α-glucosidase deficiency, which leads to cardiomyopathy in all infantile-onset and occasional late-onset patients. Cardiac assessment is important for its diagnosis and management. This article presents unpublished cardiac findings, concomitant medications, and cardiac efficacy and safety outcomes from the ADVANCE study; trajectories of patients with abnormal

Objective: The diagnosis of hypertension in adolescents aged ≥13 and <16 years is based on the percentile according to age, gender, and height in the European Society of Hypertension guidelines guideline; whereas, the American Academy of Pediatrics guideline uses blood pressure above 130/80 mmHg as a single criterion. Therefore, this study aimed to evaluate the compatibility of these two guidelines

Kawasaki disease is usually self-limited, but it can lead to aneurysm, stenosis, thrombosis, and myocardial infarction in the coronary arteries. The most important complication of Kawasaki disease is coronary artery aneurysm. Coronary artery aneurysm or ectasia may be seen in 15–25% of patients who do not receive treatment. It develops in 5% of children who receive intravenous immunoglobulin at the

Many interventionists are infatuated by the recent resurgence of the coilwire design with the Super Arrow-Flex® sheath (Teleflex, Inc., NC, United States of America). This exclusive sheath is a highly flexible, durable, conduit intended for use in diagnostic and interventional procedures with several advantages and maximum effectiveness in challenging cases. We report failure to easily advance memory

Pseudoaneurysm of the ascending aorta is rare (1–2%) and a potentially fatal complication following cardiac surgeries. Surgical repair is still the gold standard treatment of ascending aortic pseudoaneurysm. However, endovascular repair methods including stent grafts and Septal Occluder devices have been reported. We report a case of 38-year-old female patient who presented with giant ascending aortic

Objective: To determine the utility of screening electrocardiograms after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection among children in detecting myocarditis related to coronavirus disease 2019 (COVID-19). Study Design: A retrospective chart review was performed at a large paediatric academic institution to identify patients with prior SARS-CoV-2 infection who received a

Introduction: While the efficacy and guidelines for implementation of rapid response systems are well established, limited information exists about rapid response paradigms for paediatric cardiac patients despite their unique pathophysiology. Methods: With endorsement from the Paediatric Cardiac Intensive Care Society, we designed and implemented a web-based survey of paediatric cardiac and multidisciplinary

Background: Parents who receive a diagnosis of a severe, life-threatening CHD for their foetus or neonate face a complex and stressful decision between termination, palliative care, or surgery. Understanding how parents make this initial treatment decision is critical for developing interventions to improve counselling for these families. Methods: We conducted focus groups in four academic medical

Introduction: Adolescents with CHD require transition to specialised adult-centred care. Previous studies have shown that adolescents’ knowledge of their medical condition is correlated with transition readiness. Three-dimensional printed models of CHD have been used to educate medical trainees and patients, although no studies have focused on adolescents with CHD. This study investigates the feasibility

Balloon dilatation of coarctation of aorta is a standard of care for the patients presenting with severe left ventricular dysfunction. It can be performed through femoral, carotid, and axillary arterial access. Very few case series were available in the literature through axillary arterial access, despite being its advantage as non-end artery and easily palpable in coarctation of aorta. We present

Seckel syndrome is a very rare autosomal recessive disorder also known as bird headed dwarfism”. It is characterised by proportional short stature, low birth weight, dysmorphic facial appearance, and mental retardation. In addition to its dysmorphic features, skeletal, endocrine, gastrointestinal, haematologic, genitourinary, and nervous system has been involved. Cardiovascular features very rarely

Children with a family history of hypertension have higher blood pressure and hypertensive pathophysiological changes begin before clinical findings. Here, the presence of arterial stiffness was investigated using central blood pressure measurement and pulse wave analysis in normotensive children with at least one parent with essential hypertension. Twenty-four-hour ambulatory pulse wave analysis monitoring

The condition of partial anomalous origin of a branch pulmonary artery from the descending aorta could be found in several diseases and should be carefully differentiated. We report an unusual case of anomalous systemic arterial supply to normal basal segments of the left lower lung and another case of intralobar pulmonary sequestration. These two cases were treated successfully by transarterial embolisation

Introduction: To investigate quality of life and mental health after Fontan completion, we aimed to characterise outcomes in a representative group of adolescent patients. The study was part of the pre-transition clinical work-up in adolescents with Fontan-type palliation of univentricular CHD. The programme covers the entire paediatric Fontan patient population in Norway. Methods: Our cross-sectional

Noonan syndrome is a genetic disorder characteried by short stature, typical facial features, developmental delay, and CHD. In this single-centre retrospective study, we analysed typical Noonan syndrome-related electrocardiographic features in 95 patients with clinically and molecularly confirmed Noonan syndrome. Typical Noonan syndrome-related electrocardiographic features are left axis deviation

Pseudohypoaldosteronism type I is caused by a peripheral resistance to aldosterone and can present with electrolyte abnormalities, poor growth, or dehydration. Although a rare disease, several case reports have been published regarding Pseudohypoaldosteronism type I in neonates and infants. We report a case of failure to thrive and hyponatremia in an infant with hypoplastic left heart syndrome who