Limited evidence exists to guide chest tube management following cardiac surgery in children. We assessed chest tube practice variation by surveying paediatric heart centres to prepare for a multi-site quality improvement project. We summarised management strategies highlighting variability in criteria for chest tube removal between and within centres. This lack of standardisation provides an opportunity for quality improvement.

There are several options for the repair of interrupted aortic arch. Direct anastomosis may cause several problems including anastomotic site stenosis, left main bronchus compression, and acute-angled aortic arch. Interposition of a prosthetic graft has no growth potential. We present a case of 34-month-old child with interrupted aortic arch, which was repaired using a pulmonary autograft tube.

Kawasaki disease is an acute febrile multisystem vasculitis. The term Incomplete Kawasaki disease is used in the presence of a minimum of two diagnostic criteria of clinical Kawasaki syndrome accompanied by at least 5 days of fever, the absence of any other reasons characterising the disease, and the presence of severe systemic inflammation findings. Gastrointestinal symptoms, notably diarrhoea, abdominal pain, and vomiting, frequently occur, and elevated serum aminotransferases, gallbladder hydrops, and rarely other forms of gastrointestinal involvement such as ischaemic colitis, intussusception, hepatic necrosis, splenic infarct, intestinal pseudo-obstruction, colitis, and colon oedema are also reported. In this paper, we present an incomplete and atypical Kawasaki case that explicitly shows gastrointestinal involvement. Progressive bowel oedema was detected in the patient presenting with severe abdominal pain and distension. We determined an aneurysm in the right coronary artery and diffuse dilatation in the left main coronary artery despite administration of early intravenous immunoglobulin. In addition to the cardiac problem, hypoalbuminaemia, electrolyte imbalance, sterile pyuria, hepatosplenomegaly, and hydrops of the gallbladder were observed in the case. All findings, including progressive bowel oedema accompanying abdominal distension, improved markedly after the second dose of intravenous immunoglobulin.

A tunnel between the aorta and right atrium in a newborn was occluded with 5×6 Amplatzer Duct Occluder II-Additional Size. Our case is different because of enlarged right atrium and atypical location of tunnel orifice.

We report three-dimensional imaging of a rare finding of Ebstein’s anomaly of the tricuspid valve in a patient with hypoplastic left heart syndrome, which has been previously reported only by two-dimensional echocardiography. A fetal echocardiogram was performed at 19 weeks that showed a moderately hypoplastic left ventricle, severely hypoplastic mitral valve, a severely hypoplastic aortic valve, and a dysplastic tricuspid valve. Post Caesarean delivery at 40 weeks of gestation, a transthoracic echocardiogram confirmed the findings seen on the fetal echocardiogram. A three-dimensional echocardiogram was then performed, which demonstrated an Ebstein tricuspid valve with apical displacement of the septal leaflet, chordal attachments of the valve to the right ventricular outflow tract, and moderate tricuspid regurgitation. The patient underwent a successful heart transplantation.

Scimitar or pulmonary venolobar syndrome, a rare pulmonary anomaly, consists basically of anomalous pulmonary venous drainage of the right lung to the inferior caval vein, anomalous systemic arterial supply to the right lower lobe from the descending aorta, hypoplasia of the right lung, and dextroposed heart. We present a rare case with constellation of all these findings of scimitar syndrome, but with the aberrant pulmonary vein draining into the left atrium.

We report a rare aetiology of vocal cord paralysis secondary to undiagnosed severe pulmonary hypertension from a de novo ACVRL1 variant identified by whole-genome sequencing. The patient had a partial response to intravenous treprostinil in addition to inhaled nitric oxide, bosentan, and sildenafil.

Transcatheter closure of a multi-hole perimembranous ventricular septal defect with an aneurysm is challenging. Specific ventricular septal defect closure devices have been developed, but some occluders are reportedly used in an off-label manner. This report describes a child who had a multi-hole perimembranous ventricular septal defect with an aneurysm and underwent successful transcatheter closure using two different occluders: the Occlutech Duct Occluder (Occlutech, Helsingborg, Sweden) and the Amplatzer Duct Occluder II (St. Jude Medical, Saint Paul, Minnesota, United States of America). Transcatheter closure of a multi-hole perimembranous ventricular septal defect with an aneurysm using these two different devices can be performed safely by an experienced interventionist in selected patients.

Simulation is used in many aspects of medical training but less so for echocardiography instruction in paediatric cardiology. We report our experience with the introduction of simulator-based echocardiography training at Weill Cornell Medicine for paediatric cardiology fellows of the New York–Presbyterian Hospital of Columbia University and Weill Cornell Medicine. Knowledge of CHD and echocardiographic performance improved following simulation-based training. Simulator training in echocardiography can be an effective addition to standard training for paediatric cardiology trainees.

We present a case of asymptomatic residual coarctation following combined resection and isthmusplasty with implantation of the left internal thoracic artery distal to the repair 30 years ago.

In this study, we report a patient with pulmonary atresia with intact ventricular septum (PA/IVS), confluent pulmonary arteries supplied by an arterial duct, and chromosome 22q11.2 microdeletion. The 22q11.2 deletion syndrome has been associated with anomalies of the outflow tracts, such as tetralogy of Fallot with either pulmonary stenosis or atresia, but we are aware of a solitary case described with pulmonary atresia when the ventricular septum is intact. The presence of genetic malformations can have long-term co-morbidities. By describing our patient, we aim to create awareness of this rare association.

Mowat–Wilson syndrome is a genetic condition due to a mutation in the ZEB2 gene; it affects many systems including the cardiovascular system. The pulmonary arterial sling originates from a failure of development of the proximal portion of the left sixth aortic arch, resulting in an anomalous left pulmonary artery origin from the posterior wall of the right pulmonary artery and the left pulmonary artery crossing to the left lung between the trachea and the oesophagus. We present a 4-month-old infant with Mowat–Wilson syndrome and left pulmonary arterial sling, and discuss the association of these two rare conditions. Pulmonary arterial sling is significantly more frequent in patients with Mowat–Wilson syndrome than in the general population.

Pulmonary artery sling is a congenital cardiovascular disease and is usually accompanied by tracheobronchial stenosis. Generally, infants diagnosed with pulmonary artery sling should have surgery. However, the treatment of tracheobronchial stenosis is still controversial. Our team developed a customised, degradable, three-dimensional printed splint and successfully applied it in the treatment of pulmonary artery sling associated with severe bilateral bronchus stenosis. We suggested that three-dimensional printing may be a novel and effective way to treat tracheobronchial stenosis and other diseases in children.

We describe the successful use of recombinant factor VIIa (rFVIIa) in the control of massive haemoptysis in a 17-year-old patient with a Fontan circulation. The patient was intubated and ventilated in the ICU with deteriorating gas exchange. Conventional methods to control the haemoptysis were ineffective, and rFVIIa was successfully administered as a rescue therapy. rFVIIa is a powerful pro-thrombotic agent, which is only licensed in haemophiliacs with acquired inhibitors to anticoagulation. It has been used off-license in the treatment of massive haemorrhage, although a Cochrane review did not show any significant benefit; however, it may have a role as a rescue therapy where alternatives options have been exhausted after careful risk–benefit analysis.

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.

A term newborn, who underwent lotus birth, developed Staphylococcus lugdunensis sepsis and endocarditis on the 1st day of life. This case reports an uncommon pathogen known to cause destructive endocarditis in the adult and paediatric populations, causing endocarditis in a neonate. We speculate that lotus birth, an atypical birthing practice, may have contributed to the development of this infection.

The absence of a subpulmonary ventricle in the Fontan circulation results in non-pulsatile pulmonary blood flow. Lower limb exercise in this setting can generate pulsatile pulmonary blood flow.

Coronary vasospasm can result from silent myocardial ischaemia to sudden death. There are many precipitant factors including different pharmacological agents. Kounis syndrome is defined by acute coronary syndromes associated with anaphylactic or anaphylactoid reactions. We report, to the best of our knowledge, the first paediatric case of Kounis syndrome due to intravenous atropine.