An 8 year old girl presents with cyanosis, exertional dyspnoea, and palpitation only. X-ray displayed a typical coeur en sabot heart and auscultsation revealed a harsh ejection systolic murmur. Echocardiography confirmed Tetralogy of Fallot (McGoon ratio 1.3) and computed tomography was requested for further investigation of pulmonary stenosis. A vascular ring was detected, presenting asymptomatically and previously missed through echocardiography. The patient underwent a double-stage repair with ligation of an aortic arch. Recovery was unremarkable. This case highlights the limitations of echocardiography in detection of extra-cardiac anomalies.

The success of extracorporeal support in providing cardiopulmonary support for a variety of patients has led to use of Extracorporeal Life Support, also known as ECLS, as a rescue for patients failing conventional resuscitation. The use of Extracorporeal Life Support in circumstances of cardiac arrest has come to be termed “Extracorporeal Life Support during Cardiopulmonary Resuscitation” or “ECPR”. Although Extracorporeal Life Support during Cardiopulmonary Resuscitation was originally described in patients following repair of congenital cardiac defects who suffered a sudden arrest, it has now been used in a variety of circumstances for patients both with and without primary cardiac disease. Multiple centres have reported successful use of Extracorporeal Life Support during Cardiopulmonary Resuscitation in adults and children. However, because of the cost, the complexity of the technique, and the resources required, Extracorporeal Life Support during Cardiopulmonary Resuscitation is not offered in all centres for paediatric patients with refractory cardiac arrest. The increasing success and availability of Extracorporeal Life Support during Cardiopulmonary Resuscitation in post-operative cardiac patients, coupled with the fact that patients undergoing the Norwood (Stage 1) operation can have rapid, unpredictable cardiac deterioration and arrest, has led to a steady increase in the use of Extracorporeal Life Support during Cardiopulmonary Resuscitation in this population. For Extracorporeal Life Support during Cardiopulmonary Resuscitation to be most successful, it must be deployed rapidly while the patient is undergoing excellent cardiopulmonary resuscitation. Early activation of the team that will perform cannulation could possibly shorten the duration of cardiopulmonary resuscitation and might improve survival and outcome. More research needs to be done to refine the populations and circumstances that offer the best outcome with Extracorporeal Life Support during Cardiopulmonary Resuscitation, to evaluate the ratios of cost to benefit, and establish the long-term neurodevelopmental outcomes in survivors.

Sudden death in pulmonary atresia and intact ventricular septum with right ventricular dependent coronary circulation is a well-established complication, and is thought to be caused by myocardial ischaemia. We report a case of paroxysmal complete atrioventricular block that raises the possibility of an additional mechanism of sudden death.

Aneurysm of the left atrial appendage is a rare pathological condition. We describe the diagnostic work-up and surgical management of a child with giant congenital aneurysm of the left atrial appendage.

Extracorporeal cardiopulmonary resuscitation may be defined as the use of extracorporeal membrane oxygenation for the support of patients who do not respond to conventional cardiopulmonary resuscitation. Data from national and international paediatric databases indicate that the use of extracorporeal cardiopulmonary resuscitation is increasing. Guidelines from the American Heart Association suggest that any patient with refractory cardiopulmonary resuscitation and potentially reversible causes of cardiac arrest is a candidate for extracorporeal cardiopulmonary resuscitation. One possible framework for selection of patients for extracorporeal cardiopulmonary resuscitation includes dividing patients on the basis of favourable or unfavourable characteristics. Favourable characteristics include cardiac disease, witnessed event in the intensive care unit, ability to deliver effective cardiopulmonary resuscitation, active patient monitoring present, favourable arterial blood gases, and early institution of extracorporeal membrane oxygenation. Unfavourable characteristics potentially include non-cardiac disease, an unwitnessed cardiac arrest, ineffective cardiopulmonary resuscitation, and severely acidotic arterial blood gases. Considering the significant resources and cost involved in the use of extracorporeal cardiopulmonary resuscitation, its use needs to be critically examined to improve outcomes, assess neurological recovery and quality of life, and help identify populations and other factors that may help guide in the selection of patients for successful extracorporeal cardiopulmonary resuscitation.

We describe the case of a young man aged 19 years with a double inlet left ventricle associated with transposition of the great arteries, and a mechanical aortic valve requiring anticoagulation, who presented with massive haemoptysis. At cardiac catheterisation, there were multiple feeder vessels to a bleeding leash surrounding and communicating with his left main bronchus. Despite occlusion of the larger feeder vessels, he continued to have massive haemoptysis. We describe a novel surgical strategy of denuding the peribronchial vessels through a left lateral thoracotomy. This successfully stopped his bleeding.

Generalised lymphangiomatosis is a rare disease that is characterized by widespread bony and soft tissue involvement of lymphangioma. Radiological evaluation is crucial because the site and extent of the lymphangioma are important prognostic factors. The computed tomography, ultrasonography and magnetic resonance images showed sharply defined, non-enhanced cystic lesions involving the mediastinum, bones, spleen, lung, and lower neck. We report here a case that was referred to us for investigation of marked cardiomegaly.

The Shwachman–Diamond syndrome is an autosomal recessive bone marrow failure syndrome with exocrine pancreatic insufficiency. Additional organ systems, such as the liver, heart and bone, may also be affected. We report a patient with a long history of cardiac failure and diagnosis of dilated cardiomyopathy with intermittent neutropenia. Periodic follow-up revealed progressive cardiac failure and pulmonary hypertension. A diagnosis of Shwachman–Diamond syndrome was made at the autopsy.

Cervical aortic arch is a rare anomaly occasionally associated with other cardiovascular abnormalities. We present a case of tortuous left cervical aortic arch associated with hypoplastic transverse arch, coarctation of the aorta, and right brachiocephalic arteries arising below the coarctation and stenotic origin of the left subclavian artery. These multiple anatomic anomalies, which are associated in our case, have not been described in a single patient previously.

We describe a case of transcatheter occlusion of a large and tortuose coronary artery fistula in an 8-year-old girl. During 2.5 years’ of follow-up, she had no complaints and developed normally. This method of reserving the guide wire in cardiac catheterisation is extremely useful. Through the application of a patent ductus arteriosus occluder, the transcatheter closure of coronary artery fistulas proves to be a safe, feasible, and cost-effective option to surgery.

The use of extracorporeal membrane oxygenation in infants and children with cardiac disease who develop refractory cardiogenic shock, cyanosis, or cardiac arrest is increasing. Early mortality in children with cardiac disease who require extracorporeal membrane oxygenation remains an important issue, as only 40% of cannulated patients survive to discharge from the hospital. However, it is encouraging that 90% children who are discharged alive from the hospital after extracorporeal membrane oxygenation are still alive at intermediate-term follow-up. Surviving patients are at risk for long-term dysfunction of multiple organ systems related to their underlying cardiac disease, non-cardiac comorbidities, treatment-related complications, and exposure to extracorporeal membrane oxygenation. Among the most important acute complications related to support with extracorporeal membrane oxygenation is injury to the central nervous system, which may contribute to adverse neurodevelopmental outcomes. All of these factors, in turn, influence quality of life. Many survivors remain medically complex related to their underlying cardiac disease, comorbidities, and sequelae of complications acquired over their lifetime. Neurological morbidity clearly plays an important role in approximately one-third of survivors, with significant deficits in approximately 10%. The limited data about quality of life data that are available for survivors of cardiac extracorporeal membrane oxygenation suggests that approximately 15–30% of survivors have at least moderately decreased quality of life. Overall, published data support the ongoing use of support with extracorporeal membrane oxygenation in children with acute cardiac failure, most of whom would die without it. However, programmatic efforts to improve the selection of patients and the preservation of the function of end organs during extracorporeal membrane oxygenation are clearly needed in order to improve long-term outcomes.

Anomalous origin of the right coronary artery from the pulmonary artery is a rare congenital defect. We describe the case of an infant with anomalous origin of the right coronary artery from the pulmonary artery in association with tetralogy of Fallot. This patient had a pre-operative echocardiographic diagnosis, which was confirmed by angiography, and later underwent a successful surgical repair.

We present an unusual, biopsy-proven case of endomyocardial fibrosis in a 22-month-old male child, which progressed rapidly resulting in death. The patient was born to a father originating from Mozambique, where the disease is endemic but who had not himself travelled there, suggesting a genetic link. Other remarkable features were the presence of a right ventricular diverticulum, and a positive Mycoplasma pneumoniae immunoglobulin M enzyme-linked immunosorbent assay test.