Dr Annabel Greenwood ST5

​​When a flyer advertising the Welsh Paediatric Cardiovascular Network (WPCN) Autumn Meeting popped up in my inbox, I jumped at the chance to attend in the hope of broadening my (somewhat limited!) paediatric and neonatal cardiology knowledge.  I’m sure many fellow trainees would agree with me when I say that the subspecialty of paediatric cardiology can at times feel a little overwhelming and complex, so any additional learning opportunities in this field are certainly invaluable. 
 
This year’s Autumn meeting was held at the Radisson Blu Hotel in the centre of Wales’ capital city, and was attended by a range of representatives from the paediatric multidisciplinary team with an interest in cardiology.

The principal theme of the day was pulmonary artery hypertension (PAH), and the morning session was particularly neonatal-focussed, emphasising the relationship between prematurity and PAH.  To set the scene, the day began with a case presentation of an ex-premature baby with chronic lung disease and a patent ductus arteriosus who subsequently developed PAH.
 
Then followed a talk on cardiovascular outcomes in chronic lung disease (CLD) of prematurity. Professor Kotecha, Consultant Neonatologist at UHW, discussed the effects of CLD of prematurity on the left and right sides of the heart independently.  With regards to the left-side of the heart, he considered the theory that those born prematurely will have higher systolic blood pressures later in life.   This was based on the concept of arterial stiffness, and that prematurity and low birth weight are inversely related to arterial stiffness, and also that lung function is inversely related to development of arterial stiffness.  However, he then went on to discuss the results of an ongoing respiratory study, the ‘RHINO’ Study, (Respiratory Health Outcomes in Neonates), whereby children aged 7-12 years, born prematurely at <34/40 gestation, were assessed in terms of their lung function, including spirometry measurement pre and post-bronchodilator therapy.  The study found that there was no significant difference in peripheral systolic BP between those born prematurely with CLD, those born prematurely without CLD, and term controls.  There was, however, a significant difference in peripheral diastolic pressure (higher in preterm CLD patients).  This difference could perhaps be explained by measurement error or even steroid use.  
 
As for the right-side of the heart, pulmonary pressures increase in those born prematurely, but the key point to note is that by improving lung function and a child’s overall general health, then pulmonary pressures won’t be such a major issue. 
 
Dr Kevin Poon, Consultant Neonatologist at Royal Gwent Hospital then took us through the echocardiographic assessment of PA pressure.  He highlighted the echocardiographic parameters used to assess PA pressures, and also surrogate markers of raised PA pressure.  

The following measurements can be used routinely to assess PA pressure:

• TR jet – provided there is a good envelope, no RVOT obstruction or large VSD, and good RV function
• PR jet 
• PAAT:RVET ratio (PA Acceleration Time: RV Ejection Time) – in addition to TRJV, or if unable to obtain good TR envelope
• TAPSE (Tricuspid Annular Plane Systolic Excursion)
• LV eccentricity index - when septal flattening or bowing seen 
• Myocardial velocity – if you have a tissue doppler package

These are measurements that I am on the whole unfamiliar with, given my limited exposure to performing echocardiograms, however, it was very useful to learn more…and of course, the key message was to ‘leave the fancy stuff’ to the cardiologists!


After a brief refreshment break, Karina Parson-Simmonds, Children’s Cardiology Nurse Specialist at UHW, presented about the ‘6 Minute Walk Test.’  This is a test to determine the distance a person can walk at a constant, uninterrupted, unhurried pace in 6 minutes, and is widely used to assess exercise capacity of paediatric patients with CV disease.  It can also be used to monitor disease progression, to evaluate patients’ exercise tolerance pre/post-operatively or to measure response to an intervention, and can provide longitudinal data for a patient if repeated at intervals over a period of time.  It is not however ‘gold standard,’ as this remains the cardiopulmonary exercise test, and the BORG index score (rating of perceived exertion) is subjective, possibly causing discrepancy in results. 
 
 
 
Continuing with the multidisciplinary theme, Dr Lena Thia, Paediatric Respiratory Consultant at UHW, then discussed the respiratory management of the child with CLD and PAH. There are a number of respiratory causes of PAH, all associated with hypoxia;  

• CLD
• Pulmonary hypoplasia (congenital diaphragmatic hernia, single lung)
• Chronic interstitial lung disease
• Sleep disordered breathing 
• Severe airway malacia with hypoxia
• Severe CF with end stage lung disease
• Neuromuscular disease with night time hypoventilation

Co-existing cardiac disease predisposes to developing PAH in CLD, and infants with Trisomy 21 with or without structural lung or heart disease are at increased risk of developing PAH.
 
Key investigations to consider are;

• CXR

• Blood gas – how much compensation? How high is the CO2?
• Overnight sleep study
• Lung function tests (spirometry, transfer factor)
• Exercise testing with saturation – evidence of desaturation when exercising 
• CT chest, CTPA
• Bronchoscopy 
• VQ scan
• Lung biopsy

With regards to optimising respiratory management;

• O2 for any daytime or night time hypoxia
• Ambulatory O2 for exercise
• Supplementary O2 for air travel
• O2 for emergency use at home
• Treat infections promptly – annual influenza vaccine, palivizumab, prophylactic abx
• Safe feeding assessment, and management of GORD

​The final session of the morning was an impromptu session by Dr Dirk Wilson, Consultant Cardiologist at UHW, as the planned speaker was unable to attend.  This was a fantastic session on ECHO interpretation of common structural defects (ASD, VSD, AVSD), with the use of ECHO videos to enhance learning.
 
Unfortunately due to on-call commitments, I was unable to stay for the afternoon session, however, Dr Maria Mendoza (Neonatal Clinical Fellow at UHW), has kindly provided us with a great summary…
 
"The afternoon was a fun, interactive session amongst all participants of the meeting to culminate everything we learned during the day. Each table consisted of a multidisciplinary team of consultants, trainees, nurses or cardiac physiologists where a well-informed discussion was made possible. Different cases were presented and each team was able to analyse the case together, view the different imaging results available such as ECG, Chest X-ray and Echocardiogram findings and come up with a diagnosis and subsequent management. Some of the cases were Hypoplastic Left Heart Syndrome, Possible Noonan Syndrome with Pulmonary Stenosis, Atrial Septal Defect and Hypertrophic Cardiomyopathy. We also discussed the risk of acquiring RSV especially during the winter season and which babies are at risk and need RSV prophylaxis. Overall, it was a very high-yield discussion of different clinical cases with many learning points."

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Dr Tom Cromarty ST6

Earlier this month I travelled to Barcelona with the EU funded TALK Debrief research project. This is part of the Research and Innovation Staff Exchange (RISE) programme, where Medical staff from Cardiff (Wales), Barcelona (Spain) and Stavanger (Norway) visit each other’s hospitals to learn and help with the implementation with TALK debrief, as well as experiencing other healthcare systems.
 
TALK is designed to guide structured team self-debriefing after any learning event in clinical environments. The Structure can be seen in the figure below, with more information and FREE resources are available at TALKdebreif.org
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​Whilst on my latest placement in Barcelona, I was able to attend the only specific Children’s Hospital in Barcelona called Sant Joan de Déu (SJD). This large tertiary referral hospital is mostly public funded and is going through a series of renovations. I was fortunate enough to visit the brand new PICU which in Spanish is called the Pediatric UCI (Unidad de Cuidados Intensivos), and experience the innovative Simulation centre. I was particularly impressed with the Simulation team which was created and lead by José María Quintillà Martínez. Over the past 15 years he has been incredibly passionate about simulation in healthcare, starting off with a very low fidelity mannequin and no support, to now having an integrated simulation service.
 
José and his team were incredibly enthusiastic about Simulation, ensuring that the focus stays on the intended output and the methodology, rather than the expensive shiny technology. After developing a relationship with the world leading Boston Children’s Hospital Simulator programme, José and his team have developed a Simulation service that has the backing of the hospital board and is fully integrated with the hospital, rather than a stand-alone medical education tool.
 
Other than Simulation courses, which they run on >150 days of the year, the majority of the time for hospital staff, the team was recently involved with the architectural planning of the new PICU. Having created a mock bed space in the Simulation centre, they replicated the clinical scenarios which would need the most people and equipment, such as an unwell patient requiring ECMO (Extra Corporeal Membrane Oxygenation) or Dialysis. They modified the design and size of the room, from the location of sinks, equipment & monitors, to establish the optimum environment with the floorspace available.
 
Once the ward renovations had been finished, they again brought in the Simulation team to practice common procedures and daily routines. This meant that the teething issues which inevitably come up, were corrected without a patient in sight or put at risk. This forms part of the SIMTest function of their integrated Simulation Program which can be seen below.

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​SIMTrain is the traditional work of Simulation in Healthcare, however the team in SJD aim to develop personal and team skills, as well as training patients and families. Trainers from the Boston Simulation programme visit the hospital to train a multidisciplinary group (Doctors, Nurses and Physios), forming a core Simulation faculty who can practice and train others in their departments. The PICU had five nurses and five doctors trained up, who would run a two-hour scenario on three occasions every Thursday. The simulation philosophy is so embedded, that any new department being renovated (including PICU and NICU) has a separate ‘Simulation’ room (see below), specifically reserved and always ready for use. This also enables teams to put rented equipment and new protocols to the test before investing in them and practicing on patients.

Permanent Simulation room on the PICU unit

José’s team have used the Kerr-Patrick education model to validate learning from Simulation, currently working on levels 3 and 4 to demonstrate changes in behaviour and patient outcomes. The backing they receive from the organisations leaders has enabled them to hire an innovation team, subsequently creating the 3D4H (3D for health) unit. After a complicated oncological surgery was abandoned due to the challenging anatomy, the team used imaging to create a 3D version of the patient’s head. This meant the surgical team could make an accurate surgical plan and even practice the surgery as many times as possible. By saving money on theatre and anaesthetic time, this method saves time, money and makes the procedure safer for the patients.
 
As the specialist centre for MPS (Mucopolysaccharidosis) in Spain, they receive many referrals for management and perform up to fifteen maxilla reconstructive surgeries per year. These patients have complicated upper airway anatomy, making the intubation difficult. 3D4H printed a number of typical MPS heads with accurate upper airway anatomy and put this onto a traditional high-fidelity mannequin for simulations. This enabled anaesthetists to practice this complicated skill a number of times, demonstrating their proficiency before the patient had even arrived.
 
The 3D4H team have developed been used in a number of other ways.

• Tracheostomy’s: They have printed multiple versions of anterior necks with tracheostomies. They then run simulation scenarios for ‘blocked Trachy tubes’ for staff and parents to practice their skills and improve their confidence.
• Skin: Various printed skins help staff practice suturing, wound management, accessing tunnelled central lines and USS guided cannulation. Much cheaper than buying the material from external companies.

Simple 3D printer

Trachy and various skins printed from 3D4H team

​Integrated Simulation is a new way of designing and improving medical environments, teams and the care they provide, by exploring and learning from reality before it happens! I was completely inspired by the number of functions which their Simulation team performed as an integrated service with the hospital, and the incredible number of cost-effective uses for 3D printing. 3D printing is being used more and more in medicine, with the materials becoming cheaper and technical printing skills advancing every year.  
 
How could you use Simulation or 3D Printing where you work? Perhaps Barack can inspire you?

​As always feedback or questions are always welcome.
Twitter. @tomcromarty
Email. [email protected]

Davide Paccagnella, ST3 Paediatric Trainee

​When I attended the 4th Annual Paediatric Medication Error Prevention (PMEP) Study day (which took place in Morriston Hospital, on 8th October 2019), I already knew that it was going to be a great experience!
For the past four years, Morriston has achieved great results in the field of medication error reduction and prevention. They have done this by forming a revolutionary tripartite alliance between Paediatrics, Nursing and Pharmacology. It all makes sense – by nature, prescribing, administering, checking and supplying medication is a multi-disciplinary exercise, and improving the safety of these processes can only be achieved by working together across different specialties.

This annual study day has become increasingly popular, year after year, with almost 100 registered attenders this time around. Which meant that, on the day, the lecture theatre was nearly full, making me feel like I was attending a national conference as opposed to a local study day!

The morning was kick-started by Professor Philip Routledge, CBE, from the All Wales Therapeutics and Toxicology Centre. His keynote address provided us with a global perspective on drug manufacturing, testing and marketing. When it comes to prescribing, I did not know that the largest avoidable cost, worldwide, is non-adherence to prescribed medication!  Prof. Routledge also highlighted important steps (often taken as a result of tragedy) that helped make drugs safer over the course of time. Some of these stories were very moving and sobering. Overall, it was a brilliant lecture, and I’m certainly hoping that Prof. Routledge will consider returning to Swansea to inspire us all, again, next year.

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We were privileged to have Alana Adams, Principal Pharmacist from the Welsh Medicines Information Centre, and Dr Alison Thomas, Medical Director of YCC Wales, who came to give us an overview of the Yellow Card Scheme. Perhaps unsurprisingly, it turned out that not many audience members had filled a Yellow Card out before – this lecture was therefore a necessary reminder of just how important it is to provide this information, and what reactions need to be reported.