Explain a total anomalous pulmonary venous connection diagnosis to parents and plan the surgery — OSCE

Communication framework

Acknowledge the fear and the suddenness first. Before any explanation, acknowledge how frightening it is to see a baby who was well at birth become so sick so quickly, and that they will naturally be looking for a cause. Do not rush past their fear; name it and sit with it for a moment. The parents will only hear your explanation once they feel heard. [7]

Explain the diagnosis in plain language. The heart has four chambers, and after blood picks up oxygen in the lungs it is supposed to come back to the top left chamber to be pumped to the body. Your baby was born with the pipes from the lungs plumbed into the wrong side, so all the oxygen-rich blood comes back to the right side instead of the left. On top of that, one of those pipes is narrowed, so the blood cannot get out of the lungs easily, which is why the lungs have become wet and your baby is working hard to breathe and is blue. [7]

Reassure on cause and guilt. Nothing you did or did not do caused this. It is a problem with the way the heart formed very early in pregnancy, before most women even know they are pregnant, and it is nobody's fault. You could not have prevented it, and the fact that your baby was well at birth is normal for this condition, because the changes that happen in the first day of life are what bring it to light. [7]

The urgency and the surgery

Be honest about the urgency without terrifying them. Because the pipe is narrowed, your baby's lungs are filling with fluid and the heart cannot deliver enough oxygen. This will not get better on its own, and the only treatment is an operation to reconnect the pipes to the correct side of the heart. The surgeons will do this operation today, because waiting would let the problem get worse. I know that is alarming, but the team here does this operation, and doing it now gives your baby the best chance. [2]

Explain the operation simply. The surgeon will make an opening in the breastbone to reach the heart. While a heart-lung machine does the heart's work, the surgeon will join the collecting pipe from the lungs onto the correct top left chamber, close the small hole between the top chambers, and tie off the narrowed pipe that is causing the problem. After the operation the blood will flow the right way, the lungs will start to clear, and your baby should become pink. [7]

Honest prognosis and lifelong follow-up

Give an honest but hopeful prognosis. Most babies do well after this operation and grow up like other children, but it is a big operation on a tiny baby, so there are risks, and I will not hide those from you. The team will talk you through the specific risks. The thing I want you to watch for in the months ahead is a narrowing where the pipes are joined, which can happen in some children and would show up as fast breathing or difficulty feeding; we will scan for it regularly, and if it happens we can treat it. [2] [3]

Explain the lifelong follow-up. Even after a good operation, your child's heart will be checked regularly for the rest of their life, because a small number of children develop narrowing of the lung veins or a rise in the lung pressures, and we want to catch that early. When your child reaches their late teens they will move to an adult heart doctor who looks after people who had heart conditions as babies, and that handover is planned. [1] [3]

Close with a clear plan and safety-netting

Agree the next steps and stay available. The surgeon will come and speak with you and ask for your consent. I will answer any questions you have now, and I will come back after the operation to tell you how it went. Here is the number for the ward, and the nurse looking after your baby will update you. Ask us anything, at any time; there is no such thing as a silly question. [7]