Scimitar syndrome: Heart surgeons perform life-changing surgery

Leeds Children’s Hospital heart surgeons have performed two scimitar syndrome repairs using an innovative surgical procedure, never-before performed in the UK.

Scimitar Syndrome is a rare congenital condition in which a vein from the right lung is abnormally connected to the heart causing oxygenated blood from the lung to return to the wrong side of the heart. This causes an overload and enlargement of the right side of the heart and in time could lead to heart failure.

The new ‘Lugones’ procedure – pioneered in Argentina by Dr Ignacio Lugones – is an innovative way to redirect the blood to the correct (left) side of the heart, using the heart’s own lining. The traditional procedure involves creating a long artificial tunnel between the abnormal ‘scimitar’ vein and the correct side of the heart. This carries greater risks as the long tunnel can narrow or block over time. The Lugones procedure uses the body’s own tissue and creates a more direct route with less chance of narrowing or blocking.

Dr Lugones travelled to the UK to oversee the first use of his procedure in the country, working closely with heart surgeons at Leeds Children’s Hospital, led by Consultant Congenital Cardiac Surgeon Giuseppe Pelella. Children’s Heart Surgery Fund who support the Leeds Congenital Heart Unit provided four night’s accommodation for the visiting surgeon.

Other UK centres have since performed the procedure, following success at Leeds Children’s Hospital.

Adult patients Vicky Waite (43) from Tingley and Emma Clements (29) from Wetherby are the first patients in the UK to be treated using the Lugones procedure. Vicky and Emma are treated within the children’s hospital as congenital cardiac patients. The Congenital Heart Unit at Leeds Children’s Hospital looks after patients born with heart conditions from before birth, through childhood and throughout adulthood.

Full time mum of two, Vicky, only discovered she had been born with a heart condition after she’d had children. She found herself feeling breathless and tired even after climbing one flight of stairs or cleaning around the house.

Special Educational Needs teacher Emma was diagnosed with Scimitar Syndrome at the age of 19 but only required surgery recently when her condition deteriorated. Emma was easily fatigued and didn’t feel able to go out which affected her mentally as well as physically.

Both patients may not have been candidates for surgical interventions due to the complexity of their condition and risks associated with the traditional procedure. Surgical alternatives would have been temporary whereas the Lugones procedure has the potential to be a permanent fix that will reduce the risk of heart failure and enhance the patient’s quality of life.

After surgery that lasted between six and eight hours, both patients were discharged within a week of their surgery and are recovering well at home.

Only four weeks after the major heart surgery Vicky already felt better then pre-surgery, had a better appetite, is sleeping better and a post-operative scan four days later showed that the right side of heart (that was severely enlarged) had already dramatically reduced in size.

Dr Lugones is a Paediatric Congenital Cardiac Surgeon working at the Buenos Aires General Children’s Hospital and has pioneered this new procedure. Mr Pelella was introduced to Dr Lugones and his procedure at a surgical conference in Milan. Mr Pelella identified that this procedure could be beneficial for patients in the UK and invited Dr Lugones to deliver training and fly into the UK to supervise procedures on the first two UK patients.

Mr Pelella said:

‘The Lugones procedure has the potential to improve the quality of life of the patients, correcting an anatomical defect. With the defect corrected patients will have reduced risk of heart failure, feel less fatigued and be able to live full and active lives. The procedure can be used for patients who previously would not have been candidates for surgical intervention because the risk of failure was too high. I’m extremely pleased for our first two patients, and I hope that more patients will now benefit from this procedure in Leeds and across the UK’

Speaking four weeks after her surgery, Emma said:

‘I can’t thank Dr Pelella and his colleagues enough for being so committed and finding the best outcome for me. Despite the procedure being so new he made me feel so relaxed, he was there every day checking I was OK… Before surgery my heart might not have been strong enough to cope with a pregnancy. Our prospects for starting a family are now so much more positive – thanks to this procedure’

Emma and her fiancé have rescheduled their wedding for 2025 having postponed it pending Emma’s surgery.

Vicky reflected:

‘It was a bit unnerving and scary but I had confidence in Mr Pelella and it was really reassuring that Mr Lugones could be there to supervise the operation… It wasn’t like meeting two surgeons it was like making two new friends. I’m looking forward to being able to go to the beach, play football with my kids without getting out of breath… just do normal things without having to take a million breaks!’

Mr Colin Holton (Clinical Director at Leeds Children’s Hospital) said:

‘I’m extremely proud of the team who have brought this innovative procedure to Leeds in a UK first. Our Congenital Cardiac Team are well known for leading the field and pushing the boundaries when it comes to surgical interventions. It’s great to know that children and adults have a world class service on their doorstep here in Leeds’

Surgeons at Leeds Children’s Hospital hope that other centres will adopt the procedure, improving the outlook for hundreds of congenital cardiac patients across the UK.