When Lucy Hijmans was born her heart was only able to deliver half the oxygen she needed. She could only just breathe for herself.

In the past this would have meant her paediatricians searching rapidly for a specialist cardiac centre to diagnose what transpired to be a congenital heart disorder of transposition of the greater arteries.

She would have been at serious risk from any journey and her shocked parents would have been separated from home, and probably each other, whilst mother recovered from the birth and father rushed to an unknown place.

Transposition of the greater arteries

But this did not happen to Lucy. Elizabeth Biggart, children’s specialist nurse manager, explains: “Lucy’s problems were first identified at an early pregnancy scan and her mother’s obstetricians referred her to Dr Julene Carvalho, consultant cardiac foetologist at Royal Brompton, who diagnosed her heart problems with a scan at St Georges Hospital Tooting.

“Lucy’s parents then had an opportunity to understand what this would mean for them and their unborn child, helped by the support of Dr Carvalho and Mary McLoughlin, the specialist foetal cardiac nurse. Both parents had the opportunity to visit the paediatric unit at Royal Brompton Hospital where the necessary surgery was to take place.”

To ensure the baby’s safety, Lucy’s mother was admitted to Chelsea and Westminster Hospital and the birth was induced. Lucy was admitted directly to the neonatal unit to stabilise her and then transferred along the road to Royal Brompton, where she was closely monitored and brought to the best possible state before her surgery.

Parents and baby together

Her parents were able to be with her and her mother was cared for by the community midwives. Surgery was scheduled and just a week after her birth Mr Darryl Shore and his team performed an arterial switch procedure and patent ductus ateriosis ligation.

For this procedure the two main blood vessels coming from the heart were corrected and switched, as they were performing the wrong functions; the vessel to transport blood round the body at high pressure was going to the lungs and the vessel that should have gone to the lungs was sending out blood to the body.  

Team monitoring

Elizabeth continues: “Lucy was cared for on the paediatric intensive care unit following her operation with the team of intensivists, cardiologists and nurses during the vulnerable days ahead, and was mechanically supported by intubation and ventilation and chest drains (her chest had to remain open for some days).

“Infusions of inotropes, drugs to support her heart function, pain control and sedation were necessary for many days. Every aspect of her physiology was monitored and she was able to start tolerating special milk the day following surgery.

“The surgeons were able to close her chest soon after and her steady progress allowed a slow weaning from the ventilator. Her parents were able to be at her side throughout this period and lived in the hospital.”

Specialist nurse support

Seven days after surgery Lucy was well enough to be transferred to Rose ward to continue her recovery. She continued in the care of the cardiologists and nurses, with support from her specialist nurse, Cath Taylor.

Finally she was able to be discharged home in the last week of April to start her life properly with her parents and older brother.

Mr Darryl Shore, director of paediatric and adult congenital cardiac surgery, commented: “Complete transposition occurs in approximately one in 4000 live births. Surgery involves switching back the coronary arteries to the appropriate ventricle which also involves transfer of the great arteries.

“It is generally considered one of the more technically demanding operations with little or no margin for error. Lucy had what is termed “intramural” coronary arteries where the coronary arteries actually run in the wall of the aorta for a short course. These are the most difficult to relocate. The operation has to be performed in the first two to three weeks of life.

“The most common problem during follow-up is some narrowing of the arteries to the lungs in a small percentage of patients. In most cases, this can be dealt with by balloon dilatation in the catheter laboratory. However, as long as she is monitored appropriately, Lucy should have a normal childhood with no further need for intervention.”   

Read more about our foetal cardiology expert Dr Julene Carvalho.