We report a rare complication of central venous catheter placement in a 5-month-old baby.

The term right aortic arch is used for an aorta that arches over the right bronchus. Right aortic arch was classified into two types by Felson et al, based on branching patterns, with a proposed embryological explanation, and into three types by Shuford et al. Other anatomical variants of right aortic arch were described later, including isolated left brachiocephalic artery and aberrant left brachiocephalic artery. We have classified right aortic arch anatomy into 10 variants, supported by radiological evidence, and with reference to possible embryology. This classification will help in understanding the morphological basis for the formation of different types of right aortic arch and the course of the recurrent laryngeal nerve in such cases.

A 14-year-old boy was receiving subcutaneous infusions of treprostinil for pulmonary artery hypertension. Because he had severe infusion site pain in the lower abdomen, we changed his infusion site to the upper buttocks, adding some analgesics. His pain improved gradually. This change may be an effective method for reducing infusion site pain in subcutaneous treprostinil therapy, particularly in children.

Technological advances have led to better patient outcomes and the expansion of clinical services in paediatric cardiology. This expansion creates an ever-growing workload for clinicians, which has led to workflow and staffing issues that need to be addressed. The objective of this study was the development of a novel tool to measure the clinical workload of a paediatric cardiology service in Cape Town, South Africa: The patient encounter index is a tool designed to quantify clinical workload. It is defined as a ratio of the measured duration of clinical work to the total time available for such work. This index was implemented as part of a prospective cross-sectional study design. Clinical workload data were collected over a 10-day period using time-and-motion sampling. Clinicians were contractually expected to spend 50% of their daily workload on patient care. The median patient encounter index for the Western Cape Paediatric Cardiac Service was 0.81 (range 0.19–1.09), reflecting that 81% of total contractual working time was spent on clinical activities. This study describes the development and implementation of a novel tool for clinical workload quantification and describes its application to a busy paediatric cardiology service in Cape Town, South Africa. This tool prospectively quantifies clinical workload which may directly influence patient outcomes. Implementation of this novel tool in the described setting clearly demonstrated the excessive workload of the clinical service and facilitated effective motivation for improved allocation of resources.

Undiagnosed coronary sinus obstruction can be a source of paediatric exertional chest pain and death. We present a case series of three interesting children who developed only exertional angina from various degrees of coronary sinus stenosis or atresia. We discuss the diagnosis and management of each case and the potential pathophysiology involved with their symptoms.

A 29-month-old girl had idiopathic massive pericardial effusion for over 6 months. Lymphangiography was performed for chronic and recurrent pericardial effusion and pulmonary lymphangiectasia, suspected based on CT findings. Magnetic resonance lymphangiography revealed chylolymphatic reflux from a tortuously dilated thoracic duct in the mediastinum to the pericardial space, suggesting primary chylopericardium with lymphangiectasia. Pericardial effusion resolved immediately after thoracic duct embolisation at the lower thoracic level. However, pericardial effusion recurred after 5 months, which resolved after additional embolisation of the abnormal lymphatic collateral vessels from the remnant upper thoracic duct. Here, we report an unusual case with chylous massive pericardial effusion diagnosed by magnetic resonance lymphangiography and treated with percutaneous embolisation.

Myocarditis is an important cause of arrhythmogenic sudden cardiac arrest in the young. A strong index of suspicion is required as not only can arrhythmias be the only clinical manifestation but also because these patients can have normal cardiac biomarkers, electrocardiographic and echocardiographic findings, and inflammatory markers. Patients with ventricular arrhythmias in the setting of viral myocarditis, especially the ones in whom cardiac MRI findings normalise upon follow-up, tend to do well in the long run and an implantable cardioverter-defibrillator should be avoided in these patients; instead, a wearable defibrillator should be temporarily used as we did in this 7-year-old.

The incidence of novel coronavirus disease-19 (nCoV-19) and its associated complications is higher in high-risk groups. In this article, we explain the symptoms and course of the disease and the treatment for an adult patient with CHD who has been infected with novel nCoV-19.

Constriction of the fetal ductus arteriosus is rare and usually attributed to medications or CHD. We describe a 24-year-old multigravida at 33 weeks 5 days gestation with echocardiographic findings of severe ductal constriction, a dilated, hypertrophied and hypocontractile right ventricle, and severe tricuspid regurgitation following BC powder® use. Treatment with Digoxin and oxygen resulted in a progressive 71% reduction in peak systolic ductal gradient, improved right ventricular function, and decreased tricuspid regurgitation.

True absence of a branch pulmonary artery is rare. We identified a patient initially diagnosed with an absent left pulmonary artery at a previous hospital. Due to disagreement in the initial diagnosis, she had a diagnostic catheterisation, which revealed an isolated left pulmonary artery off the left innominate artery via a ductus. The ductus was recanalised with serial stenting and balloon dilatation followed by reanastomosis to the main pulmonary artery. In a patient who initially is diagnosed with an absent pulmonary artery, an alternative diagnosis, such as this case report, should be considered.

Intercoronary communications are a very rare congenital coronary artery anomalies. We report a case of a man who underwent elective coronary angiography that showed a bidirectional direct intercoronary communication between right coronary and left circumflex arteries.

A number of microorganisms were hypothesised as an aetiology of the Kawasaki disease. Unfortunately, no specific agent that provides reproducible evidence has yet been reported. We report two cases of extremely rare Kawasaki disease with tsutsugamushi disease. These case reports suggest that Kawasaki disease can rarely occur concurrently or immediately after a rickettsial illness such as tsutsugamushi disease.

Ebstein anomaly is a rare CHD known for its wide spectrum of presentation with the age of diagnosis dependent on the malformation’s severity. Here, the authors describe a case of delayed diagnosis of Ebstein anomaly, secondary to lack of medical attention, which resulted in severe tricuspid regurgitation and pulmonary hypertension. Furthermore, the case was complicated by a unique pulmonary venous abnormality.

Pompe disease is a type-II glycogen storage disease, and clinical manifestations include hypertrophic cardiomyopathy and generalised muscular hypotonia. Enzyme replacement therapy has proven to be effective in reversing the ventricular hypertrophy. The outcomes are variable depending on time to diagnosis and severity of the cardiac disease. We describe two contrasting cases of patients with infantile-onset Pompe disease. The first child was diagnosed late and had severe cardiac hypertrophy with respiratory decompensation and ventilator dependence and eventual death. The second case was diagnosed at birth with early initiation of therapy resulting in a good outcome. Our cases highlight the importance of early initiation of enzyme replacement therapy to improve clinical outcomes.

A 9-year-old African-American girl presented with sudden cardiac arrest a few hours after adenotonsillectomy. She received anaesthesia which included propofol during the procedure. Her electrocardiogram (EKG) showed type 1 Brugada pattern, and genetic testing revealed a variant of unknown significance in desmoplakin (DSP) gene. We discuss the association between propofol, Brugada EKG pattern, and malignant ventricular arrhythmias.

Seroma formation is a relatively rare complication seen after a modified Blalock–Taussig shunt. Herein, we report a rare case of seroma formation on the posterior aspect of the left atrium without it touching the graft, and presenting with shock, due to pulmonary vein compression.