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Paeds Casescardiology

Paeds Cases · cardiology

Explain tetralogy of Fallot and a tet spell to a frightened parent — OSCE

OSCE communication and shared-planning station: explaining the diagnosis of tetralogy of Fallot to a parent after an acute hypercyanotic spell, the planned surgery, the recovery and the lifelong follow-up, with empathy, honesty and without overpromising.

osce communication and shared planning
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Target exams

MRCPCH ClinicalRACP DCE

Target exams

MRCPCH ClinicalRACP DCE
Prompt
A mother whose four-month-old daughter was admitted after a frightening cyanotic spell describes wanting to know what tetralogy of Fallot is, whether the spell could have killed her baby, what the heart operation involves, and whether she will live a normal life.

Candidate instructions

You are the paediatric registrar. You have five minutes to speak with Mrs Chen, whose four-month-old daughter Mia was admitted an hour ago after going blue and limp during a feed. The team has stabilised her and her echocardiogram has confirmed tetralogy of Fallot. Mrs Chen saw the spell and is terrified. Explain what tetralogy of Fallot is in plain language, what happened during the spell and whether it will happen again, what the operation involves and when, and what the long-term outlook is — honestly and without overpromising. Answer her questions, check her understanding, and agree a plan with her. [1]

Actor brief (parent — Mrs Chen)

You are terrified and tearful. You watched your baby turn blue and go limp and you thought she was dying. You have been told it is a heart problem called tetralogy of Fallot and that she needs an operation. You want to know: (1) What is tetralogy of Fallot, and was that spell dangerous — could it have killed her? (2) Will it happen again before the operation? (3) What does the operation involve, and is it safe? (4) Will she live a normal life — sports, school, having children? (5) Could this have been found earlier, and is it your fault? Push back if the candidate uses jargon or overpromises. You calm when you feel heard and given honest, clear information. [2]

Exemplar candidate approach

Open and acknowledge. "Mrs Chen, please sit down. I can see how frightened you are, and I am so sorry you went through that. Mia is stable now, and she is in safe hands. I want to explain clearly what we found, what happened this morning, and what the plan is, and I will answer every question you have." [1]

Explain the diagnosis in plain language. "Mia was born with a heart condition called tetralogy of Fallot. It means the lower part of her heart, the right side that pumps blood to the lungs, has a narrowing, and there is a small gap in the wall between the two lower pumping chambers. Because of that, some of her blood skips the lungs and goes out to her body without picking up enough oxygen, which is why she has been looking bluer. It is something babies are born with — it is nobody's fault." [1] [2]

Explain the spell honestly. "The episode you saw this morning is called a tet spell. It happens when the narrowing suddenly gets tighter for a short time, usually with feeding or crying, so even less blood reaches her lungs and she goes very blue and sometimes floppy. Yes, it can be dangerous if it is not treated quickly, and that is why we acted fast. The good news is that we know exactly what it is, we know how to stop a spell, and once she has the operation the risk of spells goes away." [1]

Explain the operation and the outlook. "Mia will have an operation, usually around three to six months of age, to close the gap in the heart wall and open up the narrowing. The operation is very well established — the surgeons do it regularly, and most children do very well. After it, most children grow up, go to school, play, and live full lives. I want to be honest with you though: her heart will never be quite like everyone else's, so she will need check-ups for life, and a few people need another small procedure later on. She will be able to do most things; we will talk about sport and exercise as she grows." [4] [2]

Address the guilt question. "I understand why you ask whether it could have been found earlier, and whether it is your fault. This is nothing you did — tetralogy of Fallot happens as the baby's heart forms, and we do not always know why. Sometimes we find it on a scan before birth, but often it shows up over the first few months, exactly like this. You brought her in at exactly the right moment today, and that is what matters." [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: Mia stays with us for now, we watch her closely, and we teach you what to do if she has another spell before the operation — comfort her, bring her knees to her chest, and call us. The cardiology team will meet you today and talk through the operation and the timing. You can ask me anything, at any time." [2] [4]

Mark scheme (10 marks)

  • Empathy and relationship (2): acknowledges terror and guilt, sits and listens, plain language, does not dismiss. [2]
  • Explains the diagnosis clearly (2): tetralogy of Fallot as the narrowed outflow and the gap, the right-to-left shunt, without frightening unnecessarily. [1]
  • Explains the spell honestly (2): what a spell is, that it can be dangerous and is treated, and that the operation removes the risk. [1]
  • Explains the operation and outlook without overpromising (2): repair at three to six months, most do well, but lifelong follow-up and possible later procedures. [4]
  • Checks understanding and agrees a plan (2): invites questions, checks comprehension, sets out next steps and ongoing availability. [2]

References

  1. [1]Apitz C, Webb GD, Redington AN Tetralogy of Fallot. Lancet, 2009.PMID 19683809
  2. [2]Villafañe J, Feinstein JA, Jenkins KJ, et al. Hot topics in tetralogy of Fallot. J Am Coll Cardiol, 2013.PMID 24076489
  3. [3]McDonald-McGinn DM, Sullivan KE, Marino B, et al. 22q11.2 deletion syndrome. Nat Rev Dis Primers, 2015.PMID 27189754
  4. [4]Bacha EA, Scheule AM, Zurakowski D, et al. Long-term results after early primary repair of tetralogy of Fallot. J Thorac Cardiovasc Surg, 2001.PMID 11436049