The St.Emlyn’s Podcast

This is the first in a two part podcast series discussing Adult Congenital Heart Disease (ACHD) and how these patients may present to the Emergency Department (ED). Dr Sam Fitzsimmons, our guest on the podcast, is a Consultant in Adult Congenital Heart Disease at University Hospital Southampton. There is more information in this blog post.

Look out for Part 2, which will be released next week, where we discuss Eisenmenger Syndrome, Transposition of the Great Arteries and Coarctation of the Aorta. 

With advances in paediatric cardiac surgery, more and more patients with complex congenital heart disease are surviving to adulthood: in the 1950s you might expect a survival rate of about 10%, whereas now this is more like 85%. In fact, there are more patients in the adult congenital heart disease population than there are in the paediatric one (with 2.3 million adults vs 1.9 million children in Europe).

Many patients with Adult Congenital Heart Disease are young and able to live a relatively normal life. This means that they can travel and take part in just the same sort of activities as those without ACHD. They may well turn up in your Emergency Department one day, regardless of whether you are a tertiary centre or a district general hospital (DGH).

They are experts, and know their disease well, but this does not abstain you from a responsibility to know about them too! When these patients become unwell, they can go downhill very fast and you may not have the chance to discuss with them their exact lesion and its management.

The anatomy and physiology of these patients is abnormal, so they may present in atypical ways, and may not respond to usual medical interventions: in fact, some of our usual treatments may even be harmful.

However, starting with our usual 'ABC' approach is by far the best way to go, whilst gathering more information and contacting their specialist centre. Many patients will have their last clinic letter and ECG with them (which will also have the direct dial number of their specialist). And if they, or their relative, say there is something wrong you must believe them and do all you can to make sure they are fully investigated.

The presence of scars may give you some clues as to the patient's underlying condition and previous surgical repairs. (BMJ 2016; 354: i3905)

• Do your usual ABC assessment.

This is not a condition in itself, but in fact the resulting circulation after a series of operations that could've been performed due to a number of different underlying conditions:

• Tricuspid Atresia

In essence these patients are born with a single functioning ventricle, that has to be utilised to supply the systemic side of the circulation, whilst the Fontan acts as a passive means of returning blood to the pulmonary circulation.

It was first devised in the early 1970s by Dr Francis Fontan, so the majority of patients with this are in their mid thirties and younger.

As the patient is entirely dependent on their systemic ventricle to work optimally, any disturbance of the delivery into it is very poorly tolerated. Thus, any arrhythmia is life threatening, even a mild atrial tachycardia.

These patients need to be returned to sinus rhythm as quickly as possible and the recommended method for this is DC cardioversion in expert hands.

Fontan patients have an incredibly fragile circulation and any change in their respiratory physiology can be life threatening, especially if it increases their pulmonary pressures (and thus prevents the passive flow within the Fontan circulation). These patients are not candidates for sedation in the ED and should have an experienced anaesthetist to manage them during the procedure.

Beware if the patient comes in and tells you they are fasted! This means they have been in this situation before and needed DC cardioversion.

Over time the patient develops venous hypertension within the Fontan connection. This causes the formation of collateral vessels, that may link into the bronchial arterial tree.

If the patient presents in shock treat them as you would any other patient with emergency blood transfusion.

Any haemoptysis, however small, may herald the beginning of a massive bleed. These patients need further investigation, probably a CT chest with contrast. These vessels may then be coiled by interventional radiology.

If the patient has a non fenestrated Fontan they should have normal oxygen saturations. However, if there is a fenestration there will be shunting and therefore a reduction in oxygenation.

For patients this is trade of between being pink or blue, each of which have complications.

Dr Sam Fitzsimmons is a Consultant Cardiologist in Adult Congenital Heart Disease (ACHD) at the University Hospital Southampton, UK. Sam also subspecialises in pulmonary hypertension and maternal cardiology. Working within a tertiary surgical ACHD centre, Sam delivers an ACHD on call service for emergency admissions, inpatient care, routine outpatient follow-up, intra-operative imaging and post-surgical care, as well as specialist clinics in Pulmonary Hypertension and Maternal Cardiology. Sam holds a Honorary Senior Clinical Lecturer post with the University of Southampton as she is passionate about teaching and in particular, she is enthusiastic about helping demystify congenital heart disease for many non-specialist to improve patient care. Sam is well published in peer review journals, cardiology textbooks and specialist guidelines.

A podcast with Iain and Simon summarising all the latest content from the St Emlyn's blog in April 2021. Topics discussed include Vaccine Induced Thrombocytopenic Thrombosis, how our own biases can effect our critical appraisal and whether we need to worry about grading the quality of FOAMed resources.

Thanks for listening. Please check out the blogs themselevs at www.stemlynsblog.org and consider subscribing and rating us on iTunes. 

If you'd like to see some more from Peter Brindley you can watch one of his SMACC talks here. 

Simon joins Ankur Verma from Delhi, to talk about the impact of COVID in the latest wave devastating India.

A discussion about all the latest from the St Emlyn's blog, including a hot off the press article about vaccine induced thrombocytopenic thrombosis and the new FRCEM revision guide. 

Simon and Iain also talk about the latest results from the RECOVERY trial, Major Trauma Triage tools, cricothyroidotomy, thromboprophylaxis in COVID19 and the new Medical Licensing Assessment for medical students and the new St Emlyn's Undergraduate Curriculum 

An audio review of a paper in the Journal of Intensive Care Medicine with two of the authors.

Assessing on-line medical education resources: A primer for acute care medical professionals and others

Peter G Brindley, Leon Byker, Simon Carley, Brent Thoma https://doi.org/10.1177/1751143721999949

Our regular podcast round up from February 2021. Iain and Simon highlight the key learning points from this month on the St Emlyn’s blog and podcast.

Topics discussed this month include tocilizumab in COVID19, TIA risk scores, new Emergency Care standards (targets) and TXA use in epistaxis. We also pay tribute to Dr Cliff Mann, former President of RCEM who sadly died this month. 

Please remember to subscribe to the podcast on iTunes/Google Play and please do leave us some reviews and ratings there.

Our regular round up of the best of the blog and podcast from January 2021 with Iain and Simon. The St Emlyn's blog posts from January 2021 are discussed, including plenty about Coronavirus as well as other topics relevant to anyone interested in Emergency Medicine and evidence based care. 

Going into hospital as an emergency during the COVID-19 pandemic must be extremely scary for patients and their relatives. With no relatives allowed to visit and staff dressed in full PPE, the experience must be so much more unnerving than usual. Add to that the incredible worry about catching COVID-19 for those who don’t already have it; or the worry about what might happen for those who do. Will they pull through? Could this be the end?

I’m privileged to be co-leading the COvid-19 National DiagnOstic Research and evaluation programme (CONDOR), which involves a collaboration between amazing teams in Manchester, Oxford, Leeds, Newcastle, London and Nottingham. The programme evaluates diagnostic tests for COVID-19.

We’re extremely lucky to have two very experienced and proactive patient and public representatives as members of our steering committee: Graham Prestwich from Leeds and Val Tate from Oxford.

I recently spoke with Graham and Val to get their thoughts about how we, as clinicians, might effectively communicate with patients during the COVID-19 pandemic. They provide their important insights from a lay perpsective about what they would want from their clinician.

We cover everything from the challenges of communicating while wearing PPE to the way to answer important questions like, “Am I going to die?”, which many of us have, I’m sure, had to answer on a number of occasions over the past 12 months.

I hope that you enjoy the podcast. We realise that 25 minutes wasn’t long enough to cover everything we’d have liked to.We’d really like to know what you think. Are there things that we haven’t covered that you’d like us to? What are your experiences? We’d love you to share your thoughts in the chat!

Rick

A vaccine update with Rick Body, Simon Carley, Pam Vallely, Paul Klapper and Charlie Reynard. Bringing RCEM, St Emlyn's and the University of Manchester together for the latest thoughts and wisdom on the vaccines that might get us out of this pandemic.

Moderna vaccine phase 3 trial - https://www.nejm.org/doi/full/10.1056/nejmoa2022483

Oxford vaccine phase 2/3 - https://www.thelancet.com/pdfs/journals/lancet/PIIS0140-6736(20)32466-1.pdf

Pfizer vaccine trial - https://www.nejm.org/doi/full/10.1056/NEJMoa2034577

#vaccines #COVID19 #coronavirus

A special festive edition of our round up podcast featuring six weeks of blog posts and plenty more besides.

From all at St Emlyn's we hope you have a very happy festive season and we cannot wait to talk to you again in 2021.

Take care,

Simon and all the team