Hypoplastic left heart syndrome — antenatal planning structured clinical encounter

Station brief (candidate)

You are the paediatric registrar at the antenatal multidisciplinary meeting. Mei is 34 years old at 22 weeks gestation. The fetal echocardiogram confirms hypoplastic left heart syndrome with a normal karyotype and no other anomalies. She lives three hours from the nearest cardiac centre and asks what will happen when the baby is born and whether surgery will be needed. She has two young children and limited leave from work. You have 12 minutes with the patient and 5 minutes for examiner discussion. [1] [2]

Information available on request

• Third pregnancy, two children at home; conceived spontaneously; no relevant family history. [1]
• Fetal echocardiogram confirms hypoplastic left heart syndrome; karyotype normal; no other anomalies on detailed scan. [1] [19]
• Nearest cardiac centre is a three-hour drive; she has one car and limited leave from work. [2]
• Preferred language English; she is anxious and tearful. [2]

Tasks

1. Explain hypoplastic left heart syndrome in plain language, including why it is ductal-dependent and why that dictates the place of delivery. [1]
2. Outline the neonatal plan — cardiac-centre delivery, prostaglandin E1 readiness, and the staged surgical strategy — in terms she can teach back. [1] [19]
3. Address her anxiety and the practical reality of distance, childcare and leave, designing a capability-matched plan. [2]
4. Describe the long-term follow-up the medical home will coordinate after discharge. [20]

Marking anchors

Must-hit

• States that the circulation is ductal-dependent and that delivery must be planned at a cardiac-capable centre with prostaglandin ready from birth. [1] [19]
• Describes the staged surgical strategy (Norwood or hybrid, then Glenn, then Fontan) honestly, without over-promising. [1]
• Addresses the practical barriers (distance, childcare, leave) and names an owner, a contact path and a backup. [2]
• Uses teach-back and offers written information and a named contact. [2]

Merit

• Discusses the neurodevelopmental follow-up that survivors need and frames the medical-home role. [20]
• Explores the family's values and support honestly and non-directively, holding open residual uncertainty. [2]
• Considers the fetal-intervention discussion in appropriate specialist terms without offering it as a guarantee. [19]

Fail

• Tells the patient the baby will be fine after one operation. [1] [20]
• Plans a local delivery with cardiology follow-up later, missing the ductal-dependence rationale. [1]
• Pressures the family into decisions without information, time or practical support. [2]