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Abstract Body

Background and aims: Only a century ago the heart and the great vessels were not surgically approachable and any pathology affecting these structures that required surgery inevitably led to a poor prognosis. Notions of biomedical engineering are now integral part of any modern surgeon's curriculum. A potential turning points in heart surgery is mainly related to imaging techniques and digital reconstruction.

Methods: Cardiologists and cardiac surgeons were among the first to take an interest in and adopt three-dimensional (3D) reconstruction techniques in order to improve clinical outcomes and patient care. At present many large centers have already engineers dedicated to the reconstruction and printing of complex clinical cases. This allows for a personalized approach leading to preoperative planning and counseling that has been shown to improve clinical outcomes and even mortality. Images are mainly derived from magnetic resonance imaging, computerized tomography and ultrasonography.

Results: 3D printed models have proven their usefulness in a wide range of diseases. Preoperative counseling has proved particularly useful in the pediatric world to explain and raise awareness among parents about the diseases affecting their children. A recent application in this regard is the use of 3D printed hearts derived from fetal imaging, a very promising application of 3D printing. More recently, virtual reality entered the medical field as a simulated experience that can be similar or completely different from the real world. Over the years, a huge number of applications have been made in the medical field such as surgical simulation, which has been shown to significantly improve surgical performance.

Conclusions: The future of pediatric and congenital heart surgery will increasingly depend on preoperative surgical planning and the development of prosthetic substitutes for a fully personalized approach. 3D reconstruction shows great potential in this field, leading to enormous changes in the prognosis and treatment of our patients.

Background and Aims

Patients with atrial septal defect (ASD) have increased mortality and morbidity. This can only be partly explained by hemodynamic changes caused by the ASD, suggesting additional underlying causes. Patients with an ASD have an increased burden of pathogenic gene variants in ASD related genes, indicating genetics as an important etiologic factor. This study aimed to investigate genetic associations in familial ASD and comorbidities.

Methods

In a cohort with familial ASD we identified a family with ASD present in 12 family members in three generations. We performed whole exome sequencing on five family members older than 18 years to detect the causative gene variant in this family and evaluated phenotype.

Results

We identified a novel pathogenic variant within the T-box domain of TBX5 (F232L) in all affected family members over 18 years of age. They presented with diverse cardiac phenotypes including heart failure and arrhythmias. Two carriers needed a pacemaker. Skeletal malformations where subtle with small hands as the only visible malformation. These findings propose Holt-Oram syndrome.

Conclusions

We report a novel variant in TBX5 in a family with Holt-Oram Syndrome, characterized by septal defects, severe cardiac arrhythmias and mild skeletal malformations. Clinical awareness of family history, arrhythmias, and heart failure in patients with familial ASD is important and may lead to timely treatment and uncover patients with Holt-Oram Syndrome.

Background and Aims

A huddle is a short, scheduled and structured meeting to ensure patient safety. It is a rare but devastating presentation when a toddler is brought into Accident & Emergency (A&E) peri-arrest during handover, leading to semi-organised chaos.

The Resuscitation Council UK guidelines state that “every hospital should have a resuscitation team” that "meets at the beginning of every shift”. Learning from our experiences and other service models during COVID-19 pandemic, we developed a Cardiac Arrest Huddle for A&E to enable us to prepare for the unexpected.

Methods

The Huddle is a one-page laminated document placed in Paediatric A&E. The Paediatric Registrar allocates themselves to "Leader" and team members to “Airway & Breathing”, “Circulation & Access”, “Chest Compressions” and “Scribe” roles. This promotes essential leadership skills. The Nurse-in-Charge allocates 1-2 nurses to “Monitor & Drugs”.

Results

Prior to implementation, a survey showed 69% had not always known their roles in a cardiac arrest. 92% and 100% felt their confidence and teamwork would improve with pre-allocated roles respectively.

After 6 months of using the Huddle, 100% either “always” or on “most occasions” knew their role during a resuscitation. 100% felt the Huddles improved teamwork, 80% felt it saved time before the “2222” call was made and 100% felt pre-allocation of roles increased efficiency.

Conclusions

The Cardiac Arrest Huddle is available in all acute departments and has proved successful across multiple levels from clinical and patient safety to education and training. It prompts us to recommend that other DGHs may benefit from adopting a similar model.

Background and Aims

Background: NT-proBNP has gained relevance as a biomarker in children with congenital heart disease (CHD) and is increasingly represented in paediatric guidelines. Obvious advantages of urinary sampling include the avoidance of traumatisation and the ability to transfer collection to homecare or facilities with no paediatric expertise.

Methods

Methods: the correlation between urinary and serum values in children and the potential age dependency were examined with samples from 33 children (newborns – 3.4 years). Due to the different scales of the results which were significantly higher in serum than in urine, values were log transformed to allow for direct linear correlation.

In the next step the utility of isolated urinary NT-proBNP in the ambulatory diagnosis of CHD was investigated. A total of 202 children were recruited (103 controls without CHD, 99 with CHD, of which 27 had already undergone biventricular repair).

Results

Results: the correlation coefficient for serum and urine values was 0,723 (blood/urine) and improved to R²=0.814 when NT-proBNP was corrected to creatinine concentration in the sample.

Healthy controls had significantly lower urinary NT-proBNP values than children with haemodynamically relevant CHD and those who underwent biventricular repair with no residual findings on echocardiography. A ROC analysis for the diagnosis of CHD using urinary NT-proBNP showed an area under the curve of 0.807. This could be improved to 0.831 when Ross-Class was factored in.

Conclusions

Conclusion: Urinary NT-proBNP values show great promise and could represent a new simple non-invasive biomarker for children with congenital heart disease.