Acute type A aortic dissection is a catastrophic situation, often accompanied by aortic regurgitation. A rarely described cause of aortic regurgitation, in this clinical scenario, is the prolapse of an intimal flap into the left ventricular outflow tract. We present here a case of acute type A aortic dissection with a circumferential intimal flap, prolapsing into the left ventricular outflow tract and causing massive aortic regurgitation.

A 9-year-old boy, with a history of repair of severe coarctation of the aorta through balloon angioplasty 2 weeks ago, presented in the emergency paediatric department with symptoms consistent with transient cerebral ischaemia. MRI revealed an area of cerebral infarction in the right frontal lobe. Causes of cerebral ischaemia after aortic coarctation repair are briefly discussed.

Persistent primitive hepatic venous plexus is an anomaly of the systemic venous return characterised by postnatal persistence of the foetal intrahepatic venous drainage. Scimitar syndrome is a condition that consists of partial anomalous pulmonary venous return of the right pulmonary venous drainage into the systemic veins, associated with pulmonary artery hypoplasia with the underdeveloped right lung, pulmonary sequestration, and cardiac malposition. Both conditions are rare and together have been rarely described in the literature. We report the first case of this combination of lesions imaged by cardiac magnetic resonance imaging with a three-dimensional reconstruction and reviewed the literature to characterise this uncommon combination.

Ascending aortic aneurysm is uncommon in the paediatric population, and because of the rarity the aetiology, natural progression, and prognosis of the disease remain unknown. A 7-year-old boy with ascending thoracic aortic aneurysm (60×67 mm) underwent graft anastomosis from the sinotubular junction to the undersurface of the arch. Analytical determinations including karyotyping and genetic mapping were all normal. To our knowledge, idiopathic aneurysm of the ascending aorta in children is very rare.

Mesalamine-containing products are considered first-line treatment for inflammatory bowel disease. Myocarditis is recognised as a very rare possible side effect of these medications, but has not often been described in the paediatric population. We present a case of an adolescent with Crohn’s disease who presented with myopericarditis after recent initiation of Pentasa. Once identified as the causative agent, the drug was discontinued, with subsequent normalisation of troponin and improvement of function. This case identifies the importance of prompt evaluation, diagnosis, and treatment of paediatric patients receiving mesalamine-containing medications that present with significant cardiovascular symptoms.

Drug rash with eosinophilia and systemic symptoms is a drug hypersensitivity reaction. Hepatitis and nephritis are the most common visceral manifestations. Myocarditis is important to recognise, given the high mortality rate. We describe a child with drug rash with eosinophilia and systemic symptoms and discuss the role of N-terminal pro-hormone of basic natriuretic peptide in early recognition of associated myocarditis.

We present a case of a 2-year-old girl who presented with respiratory distress and umbilical abscess, and was found to have an inferior sinus venosus defect, malposition of the atrial septum primum, absent septum secundum, and anomalous drainage of the right upper and lower pulmonary veins to the right atrium.

Performing open heart surgery involves learning challenging techniques and a need for realistic training models to achieve and maintain a high level of surgical skills. The MAVID heart holder is an organ holder primarily designed to hold the heart in its anatomic position for the purpose of surgical simulation and education, thereby closing the gap between surgical performance in the laboratory and in the operating room. The device is simple to use, can be adjusted to organ size, and has the necessary instrumentation to be used with any solid organ. The MAVID heart holder also provides a platform for presentation and assists in advancing the research sphere. The advantage over other existing models is that the MAVID heart holder uses real tissue and does not distort the organ at the attachment sites. Further, it offers superior stability as well as the ability to manipulate the organ during presentation and dissection. Training with the MAVID heart holder has the potential to shorten training time to acquire surgical skills and proficiency before performing these techniques in the operating room and in so doing enhance patient safety.

Inverted left atrial appendage is an unusual complication associated with congenital cardiac surgery. Inversion of the left atrial appendage may occur during the surgical procedure or afterwards. The left atrial appendage may invert iatrogenically or as a result of the negative pressure during placement or removal of the left atrial vent or during deairing manoeuvres. This event can be life-threatening because of the mass effect of the atrial appendage within the left atrial cavity.

A 2-year-old boy developed fibromyxoid excrescence of the aortic valve 2 years after balloon dilatation for simple coarctation. Transthoracic echocardiography showed a mobile mass on the non-coronary cusp of the aortic valve. Definitive diagnosis was achieved after operative resection. This pathology was attributed to injury during catheter manipulation. Catheterised patients should be followed up carefully to avoid missing morphological changes.

We describe two cases of anomalous origin of the left lower-lobe pulmonary artery from the right pulmonary artery. The primary diagnosis was mitral atresia, hypoplastic left ventricle, aortic arch hypoplasia in the first child, and tetralogy of Fallot in the second. In both cases, the pulmonary trunk gave rise to a left pulmonary artery in the normal position. In addition, a second branch of the left pulmonary artery arose from the right pulmonary artery, and passed posterior and inferior to the left main or upper-lobe bronchus to supply the left lower lobe. In this review, we compare our findings with previously reported examples of this extremely rare cardiac malformation, and discuss possible embryological explanations for the lesion.

Iatrogenic Horner’s syndrome is a rare complication that can occur after trauma, cervical central line insertion, chest tube insertion, and rarely following adult thoracic and neck surgery, especially in high risk patients with hypertension and diabetes. The majority of cases reported in the literature describe non-iatrogenic Horner’s syndrome in adults as an unusual presentation for cervical tumours or apical lung carcinoma. In children, there are some reports describing acquired Horner’s syndrome following trauma or invasive intervention near the cervical–thoracic area. Less has been written about the incidence of Horner’s syndrome following paediatric cardiac surgery.

Myxoma in neonatal life are extremely rare. We report a case of a neonate with a pedunculated cardiac tumour arising from the anterolateral left ventricular wall protruding across the left ventricular outflow tract and continuously extending into the distal aortic arch. Surgical removal at 14 days of age via combined transaortic approach and apical ventriculotomy was indicated because of the risk of further compromise of aortic valve function and aortic arch obstruction. Histopathologic examination was consistent with a myxoma.

Using phase-contrast MRI in a foetus with borderline left ventricular hypoplasia at 37 weeks’ gestation we showed an increase in pulmonary blood flow during maternal hyperoxygenation. The associated increase in venous return to the left atrium, however, resulted in reversal of the atrial shunt, with no improvement in left ventricular output. The child initially underwent single ventricle palliation with a neonatal hybrid procedure, but following postnatal growth of the left ventricle tolerated conversion to a biventricular circulation at 5 months of age. We conclude that when there is significant restriction of filling or outflow obstruction across the left heart, neither prenatal nor postnatal acute pulmonary vasodilation can augment left ventricular output enough to support a biventricular circulation. Chronic pulmonary vasodilation may stimulate the growth of the left-sided structures allowing biventricular repair, raising the intriguing question of whether chronic maternal oxygen therapy might obviate the need for neonatal single ventricle pallation in the setting of borderline left ventricular hypoplasia.

In the domain of paediatric and congenital cardiac care, the stakes are huge. Likewise, the care of these children assembles a group of “A+ personality” individuals from the domains of cardiac surgery, cardiology, anaesthesiology, critical care, and nursing. This results in an environment that has opportunity for both powerful collaboration and powerful conflict. Providers of healthcare should avoid conflict when it has no bearing on outcome, as it is clearly a squandering of individual and collective political capital.

Outcomes after cardiac surgery are now being reported transparently and publicly. In the present era of transparency, one may wonder how to balance the following potentially competing demands: quality healthcare, transparency and accountability, and teamwork and shared decision-making.

An understanding of transparency and public reporting in the domain of paediatric cardiac surgery facilitates the implementation of a strategy for teamwork and shared decision-making. In January, 2015, the Society of Thoracic Surgeons (STS) began to publicly report outcomes of paediatric and congenital cardiac surgery using the 2014 Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) Mortality Risk Model. The 2014 STS-CHSD Mortality Risk Model facilitates description of Operative Mortality adjusted for procedural and patient-level factors.

The need for transparency in reporting of outcomes can create pressure on healthcare providers to implement strategies of teamwork and shared decision-making to assure outstanding results. A simple strategy of shared decision-making was described by Tom Karl and was implemented in multiple domains by Jeff Jacobs and David Cooper. In a critical-care environment, it is not unusual for healthcare providers to disagree about strategies of management of patients. When two healthcare providers disagree, each provider can classify the disagreement into three levels:

• SDM Level 1 Decision: “We disagree but it really does not matter, so do whatever you desire!”

• SDM Level 2 Decision: “We disagree and I believe it matters, but I am OK if you do whatever you desire!!”

• SDM Level 3 Decision: “We disagree and I must insist (diplomatically and politely) that we follow the strategy that I am proposing!!!!!!”

SDM Level 1 Decisions and SDM Level 2 Decisions typically do not create stress on the team, especially when there is mutual purpose and respect among the members of the team. SDM Level 3 Decisions are the real challenge. Periodically, the healthcare team is faced with such Level 3 Decisions, and teamwork and shared decision-making may be challenged. Teamwork is a learned behaviour, and mentorship is critical to achieve a properly balanced approach. If we agree to leave our egos at the door, then, in the final analysis, the team will benefit and we will set the stage for optimal patient care. In the environment of strong disagreement, true teamwork and shared decision-making are critical to preserve the unity and strength of the multi-disciplinary team and simultaneously provide excellent healthcare.

A 21-year-old man underwent mitral valve replacement and tricuspid annuloplasty for severe mitral regurgitation and moderate tricuspid regurgitation. Until the operation, he had been treated for hypermobility type Ehlers–Danlos syndrome. Gene examination revealed a mutation in filamin A gene, which is the gene responsible for X-linked myxomatous valvular dystrophy.

An intrapericardial vacuolated mass compressing and displacing the heart was diagnosed by echocardiography in a foetus of 22 weeks gestation. The birth was induced for early signs of foetal distress at 29 weeks and, after two initial pericardial evacuation procedures, the tumour was resected radically 7 days after birth at a weight of 1.55 kg. Mass histology showed teratoma associated with yolk sac tumour. We comment on the overall approach adopted after foetal diagnosis and the histopathological features of the tumour, and try to draw conclusions on patient outcome data.

Transposition of the great arteries is a common congenital heart defect causing cyanosis in neonates, occurring in 0.2 per 1000 live births. It has been reported to occur with other associated congenital heart lesions. However, its association with a superior sinus venosus defect and partially anomalous pulmonary venous return has not been reported. We present a neonate with transposition of the great arteries, superior sinus venosus defect with partially anomalous pulmonary venous return that underwent successful complete neonatal repair, and discuss important physiological and anatomic considerations.

A two-year-old boy with a background history of Down syndrome and partial atrioventricular septal defect presented with acute respiratory distress requiring intubation and mechanical ventilation. He continued to deteriorate, despite ventilation; direct laryngoscopy, bronchoscopy, and computed tomography demonstrated severe long segment tracheal stenosis. He was placed on extracorporeal membrane oxygenation to stabilise his condition. A slide tracheoplasty and complete repair of the partial atrioventricular septal defect was successfully undertaken. His post-operative recovery was complicated by myocardial infarction and stroke but he made a full recovery. This represents the first report of slide tracheoplasty and partial atrioventricular septal defect repair in a child following extracorporeal membrane oxygenation support.