Urgent Excision of Primary Intracardiac Burkitt Lymphoma in a Child.

A 9-year-old girl presented with a recent history of shortness of breath, fatigue, visual disturbances, and gastrointestinal symptoms. Echocardiography demonstrated three large intracardiac masses in the right and left atria protruding into the mitral and tricuspid valve orifices causing bilateral inflow obstruction. She underwent urgent surgical excision of the masses. Histology revealed rare intracardiac Burkitt's Lymphoma.

Device Occlusion of Native Pulmonary Blood Flow After Cavopulmonary Anastomosis With Persistent Pleural Effusions.

Native pulmonary tract flow after a cavopulmonary anastomosis may promote pulmonary artery growth but can lead to undesirable consequences. We report the case of a 17-month child with prolonged pleural effusions after cavopulmonary anastomosis in whom a ventricular septal defect occluder device was placed in the native right ventricular outflow tract. (Level of Difficulty: Advanced.).

Early superior cavopulmonary connection with preoperative computer tomography shows comparable outcomes for infants following Norwood palliation.

OBJECTIVES: There is increasing evidence that performing superior cavopulmonary connection (SCPC) at 3 months reduces mortality: reducing the risky 'interstage' period, volume off-loading the ventricle and reducing coronary steal, hopefully preserving ventricular function. Our objective was to describe our experience of early SCPC with preoperative computer tomography (CT) assessment compared to later connection at 6 months. METHODS: Patients undergoing Norwood procedure from 2005 to 2020 were divided into 2 eras were described and compared. Era 1 from 2005 to 2016 when SCPC was undertaken at 6 months: and era 2 (2017-2020) when an earlier operation was performed. Demographics, mortality (interstage, early and late following SCPC) and data on postoperative course and complications were recorded. RESULTS: In era 1, 191 patients underwent Norwood (120 survivors to SCPC) and 28 patients (23 survivors) in era 2. There were no significant differences in the demographics. Interstage mortality was 17.8% in era 1 and 8.0% in era 2 but not significantly significant (P = 0.22). The median (interquartile range) age at pre-imaging and SCPC was significantly lower: 99 (81-120) vs 77 (47-102) days and 175 (117-208) vs 106 (102-122) days in era 1 vs era 2 (P < 0.005). Weight was lower at SCPC in era 2 [mean (standard deviation) 6.2 kg (1.2) vs 5.1 kg (0.8), P < 0.05]. Intubation time and total length of stay were not statistically different. Median intensive care unit stay was statistically significantly longer, but not clinically significant: 2.5 (2-4) vs 3 (3-5) days, respectively (P < 0.05). There was no significant difference in early or late mortality, rates of diagnostic or interventional catheter, postoperative magnetic resonance imaging/CT or stroke. Logistic regression analysis demonstrates increasing age at SCPC was associated with increased chance of stroke or early death (P = 0.043). CONCLUSIONS: Early SCPC with CT assessment is feasible and although intensive care unit length of stay was slightly longer there was no change in the overall length of stay and no change in postoperative mortality or complications.

Paediatric mitral valve disease - from presentation to management.

Mitral valve diseases are relatively rare in the paediatric population; however, they can cause considerable mortality and morbidity worldwide. Acquired causes are a major contributor to cardiovascular disease burden in the paediatric population. Diseases can be detected before birth, at birth, or when the child is older and presents with symptoms of advanced heart failure. Definitive management consists of surgical intervention, with mitral valve replacement being the gold standard.Conclusion: Repair has been gaining popularity; however, its outcomes require further study. Percutaneous mitral balloon valvuloplasty is an emerging technique which holds promise as a bridge to surgical treatment. The effect of these interventions on quality of life must be emphasised and requires further study. What is Known: • The epidemiology of mitral valve disease in the paediatric population - predominant causes include rheumatic disease and congenital defects.  • Mitral valve repair and replacement are the standard treatment methods for paediatric mitral valve disease.  What is New: • Emergence of percutaneous mitral valve interventions and their potential as bridge-to-surgery or definitive treatment in high-risk surgical candidates.  • Recent evidence comparing mitral valve repair and replacement in the paediatric population demonstrates increasing popularity of repair techniques.

Contemporary outcomes of aortic arch hypoplasia and coarctation repair in a tertiary paediatric cardiac surgery centre.

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. METHODS: We performed retrospective data collection for all neonates and infants who underwent aortic arch reconstruction between 2015 and 2020 at our institute. Data are presented as median and inter-quartile range (IQR). RESULTS: The cohort included 76 patients: 49 were males (64.5%). Median age at operation was 16 days (IQR 9-43.25 days). Median weight was 3.5 kg (IQR 3.10-4 kg). There was no 30 days mortality. Three patients died in hospital after 30 days (3.95%), neurological adverse events occurred in only one patient (1.32%) and recurrent laryngeal nerve injury was noted in four patients (5.26%). Only three patients required the support of extracorporeal membrane oxygenation (ECMO) with a median ECMO run of 4 days. Median follow-up was 35 months (IQR 18.9-46.4 months); 5 years survival was 93.42% (n = 71). The rate of re-intervention on the aortic arch was 9.21% (n = 7). CONCLUSION: Our experience shows excellent outcomes in repairing aortic arch hypoplasia with homograft patch under moderate to deep hypothermia with low in-hospital and 5 years mortality rates.

Management of phrenic nerve injury post-cardiac surgery in the paediatric patient.

BACKGROUND: Phrenic nerve injury is a common complication of cardiac and thoracic surgical procedures, with potentially severe effects on the health of a child. This review aims to summarise the available literature on the diagnosis and management of PNI post-cardiac surgery in paediatric patients with CHD. MAIN BODY: The presence of injury post-surgery can be difficult to detect and may present with non-specific symptoms, emphasising the importance of an effective diagnostic strategy. Chest X-ray is usually the first investigation for a suspected diagnosis of PNI, which is usually confirmed using fluoroscopy, ultrasound scan, or phrenic nerve stimulation (gold standard). Management options include supportive ventilation and/or invasive diaphragmatic plication surgery. While the optimal timing of plication surgery remains controversial, it is now the most widely accepted treatment for PNI in children post-CHD surgery, especially for very young patients who cannot be weaned off supportive ventilation. Further research is needed to determine the optimal timing of surgical intervention for positive outcomes and to explore the benefits of using minimally invasive surgical techniques in children. CONCLUSION: PNI is a common and serious complication of CHD surgery, therefore, its diagnosis and management in the paediatric population are of major importance. Further research is needed to determine the optimal timing of surgical intervention for positive outcomes and to explore the benefits of using minimally invasive surgical techniques in children.

Aortic valve surgery: management and outcomes in the paediatric population.

Congenital anomalies of the aortic valve frequently necessitate intervention in childhood. The most common aortic valve pathologies present in childhood are aortic stenosis and insufficiency. Presentation of aortic valve disease depends on severity and presence of concomitant syndromes and valvular disorders. Treatment options are largely categorised as medical, percutaneous repair or surgical repair and replacement. Surgical techniques have been refined over the last few years making this the mainstay of treatment in paediatric cases. Whilst repair is considered in most instances before replacement, there are substantial limitations which are reflected in the frequency of reintervention and restenosis rate. Replacements are typically undertaken with tissue or mechanical prosthesis. The current gold-standard aortic valve replacement surgery is called the Ross procedure-where replacement is undertaken with a competent pulmonic valve and a simultaneous pulmonary homograft.Conclusion: In this review, we aim to outline the various surgical options and discuss efficacy and complications of various interventions. What is Known: • Congenital aortic valve defects repair options medically and surgically What is New: • Comparisons between surgical options for aortic valve repair including efficacy, risks and long-term outcomes.

Infective endocarditis in paediatric population.

Infective endocarditis is very uncommon in children; however, when it does arise, it can lead to severe consequences. The biggest risk factor for paediatric infective endocarditis today is underlying congenital heart defects. The most common causative organisms are Staphylococcus aureus and the viridans group of streptococci. The spectrum of symptoms varies widely in children and this produces difficulty in the diagnosis of infective endocarditis. Infective endocarditis in children is reliant on the modified Duke criteria. The use of blood cultures remains the most effective microbiological test for pathogen identification. However, in blood culture-negative infective endocarditis, serology testing and IgG titres are more effective for diagnosis. Imaging techniques used include echocardiograms, computed tomography and positron emission tomography. Biomarkers utilised in diagnosis are C-reactive protein, with recent literature reviewing the use of interleukin-15 and C-C motif chemokine ligand for reliable risk prediction. The American Heart Association (AHA) and European Society of Cardiology (ESC) guidelines have been compared to describe the differences in the approach to infective endocarditis in children. Medical intervention involves the use of antimicrobial treatment and surgical interventions include the repair and replacement of cardiac valves. Quality of life is highly likely to improve from surgical intervention.Conclusion: Over the past decades, there have been great advancements in clinical practice to improve outcomes in patients with infective endocarditis. Nonetheless, further work is required to better investigative and manage such high risk cohort. What is Known: • The current diagnostic techniques including 'Duke's criteria' for paediatric infective endocarditis diagnosis • The current management guidelines utilised for paediatric infective endocarditis What is New: • The long-term outcomes of patients that underwent medical and surgical intervention • The quality of life of paediatric patients that underwent medical and surgical intervention.

Tetralogy of Fallot: stent palliation or neonatal repair?

Surgical repair of Tetralogy of Fallot has excellent outcomes, with over 90% of patients alive at 30 years. The ideal time for surgical repair is between 3 and 11 months of age. However, the symptomatic neonate with Tetralogy of Fallot may require earlier intervention: either a palliative intervention (right ventricular outflow tract stent, ductal stent, balloon pulmonary valvuloplasty, or Blalock-Taussig shunt) followed by a surgical repair later on, or a complete surgical repair in the neonatal period. Indications for palliation include prematurity, complex anatomy, small pulmonary artery size, and comorbidities. Given that outcomes after right ventricular outflow tract stent palliation are particularly promising - there is low mortality and morbidity, and consistently increased oxygen saturations and increased pulmonary artery z-scores - it is now considered the first-line palliative option. Disadvantages of right ventricular outflow tract stenting include increased cardiopulmonary bypass time at later repair and the stent preventing pulmonary valve preservation. However, neonatal surgical repair is associated with increased short-term complications and hospital length of stay compared to staged repair. Both staged repair and primary repair appear to have similar long-term mortality and morbidity, but more evidence is needed assessing long-term outcomes for right ventricular outflow tract stent palliation patients.