Welsh Paediatric Cardiovascular Network Autumn Meeting, Oct 2019

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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So in summary...

What was good about it?

• Great opportunity for networking, especially if cardiology interest in paediatrics or neonatology
• Excellent MDT representation 
• Great value for money (only £5 for trainees!!)

What was not so good?

• Poorly advertised amongst paediatric trainees – lots of my colleagues were unaware the day was even taking place

Would I recommend?

• Absolutely! Excellent learning opportunity in a friendly, relaxed environment 

Date for the diary….

• WPCN Spring Meeting 2020 (Date to be confirmed)