Explain long-QT syndrome to a parent after a near-drowning — OSCE

Candidate instructions

You are the paediatric registrar. You have five minutes to speak with Mrs Patel, whose nine-year-old daughter Anika was admitted after collapsing during a school swimming carnival. Anika has been diagnosed with long-QT syndrome — her QTc is 520 milliseconds and her exercise-related collapse is consistent with an arrhythmic event. Mrs Patel saw the collapse and is frightened. She has two other children aged six and twelve, and she mentions she has "always fainted" herself. Explain what long-QT syndrome is in plain language, what happened during the collapse and whether it could have been fatal, what the treatment involves and whether it is safe, what the restrictions are, and what it means for the rest of the family. Answer her questions, check her understanding, and agree a plan. [1]

Actor brief (parent — Mrs Patel)

You are terrified. You watched your daughter collapse in the pool and go limp, and you thought she was drowning. You have been told it is a heart condition called long-QT syndrome and that she will need medication for life. You want to know: (1) What is long-QT syndrome, and could that collapse have killed her? (2) Is it genetic — could your other children have it, and do you have it yourself since you have always fainted? (3) What is the treatment, and is it safe — will she need medication forever? (4) Can she still swim, play sport, go to school normally? (5) Could this have been found earlier, and is it your fault she was born with it? Push back if the candidate uses jargon or dismisses your concern about the other children. You calm when you feel heard and given clear, honest information. [2]

Exemplar candidate approach

Open and acknowledge. "Mrs Patel, please sit down. I can see how frightened you are, and I am so sorry you had to watch that. Anika is stable now, she is safe, and we know exactly what caused the collapse. I want to explain clearly what we found, what it means for Anika and for your family, and what the plan is. I will answer every question you have." [1]

Explain the diagnosis in plain language. "Anika was born with a heart condition called long-QT syndrome. The heart's electrical system has a tiny delay in the way it resets between beats — it is like a pause button that stays on a fraction too long. Most of the time that does not matter, but when the heart is stressed — like during hard swimming — the delay can trigger a very fast, chaotic rhythm that stops the blood reaching the brain for a few seconds. That is what caused her to collapse and go limp. It looked like a fit because the brain does not tolerate even a few seconds without blood, but it was not epilepsy. It was the heart rhythm, and it passed on its own." [1] [2]

Address the danger honestly. "I want to be honest with you, because you asked. Yes, that kind of event can be dangerous if it does not stop on its own — that is why we take it so seriously. The good news is that it did stop, Anika is with us, and now that we know what it is, we can treat it very effectively. With the right medication and a few precautions, the risk of it happening again drops dramatically. This is a condition we know how to manage." [1]

Explain the genetics and the family. "You asked about your other children, and this is important. Long-QT syndrome runs in families — it is passed down from parent to child, so if one person has it, each of their children has a fifty-fifty chance. Because you have had fainting spells yourself, it is quite possible you carry it too — those faints may have been the same thing. What this means is that your other two children, and you, need to be checked with a simple heart tracing, an ECG, and possibly a genetic blood test. We will organise that through our cardiac genetics team. The important thing is that we can find out who carries it and who does not, and treat everyone who needs it." [2] [4]

Explain the treatment and restrictions. "The treatment is a medication called a beta-blocker — it is a very well-established medicine, it has been used for decades, and it works by quieting the adrenaline response that triggers the rhythm problem. She will take it every day, and it reduces the chance of another event by about two-thirds. She will need to avoid competitive sport and swimming for now, and there are certain medicines she must not take — we will give you a list and you should always check with us or a pharmacist before she takes anything new. She can go to school normally, she can play with her friends, she can live a full life. She just needs the medication and regular check-ups." [3]

Address the guilt question. "I understand why you ask whether it could have been found earlier, and whether it is your fault. This is a genetic condition — it was there from before she was born, and it is nobody's fault. You could not have known, because most people with it never have a collapse until they do. The fact that you brought her in today, and that we found it now, means we can protect her going forward. You did the right thing." [1]

Check understanding and agree a plan. "Can I check — what is your biggest worry right now, and have I explained anything unclearly? Here is what happens next: Anika stays with us overnight for monitoring, we start the beta-blocker, and our cardiac genetics team will meet you tomorrow to talk through the family testing. We will teach you what to do if she ever has another collapse. You can call me or the team at any time. Is there anything else you want to ask?" [2] [3]

Mark scheme (10 marks)

• Empathy and relationship (2): acknowledges terror and guilt, sits and listens, plain language, does not dismiss the concern about siblings. [2]
• Explains the diagnosis clearly (2): the delayed electrical reset, the chaotic rhythm triggered by exertion, without frightening unnecessarily. [1]
• Addresses the danger honestly (2): acknowledges the event could have been fatal, but that treatment reduces the risk dramatically. [1]
• Explains genetics and family implications (2): autosomal dominant, fifty-fifty chance for each child, need for ECG and genetic testing of siblings and parent. [4]
• Checks understanding and agrees a plan (2): invites questions, checks comprehension, sets out next steps and ongoing availability. [2] [3]