Ground-breaking PEARS procedure saves teen’s life

Cardiac surgeons at The Prince Charles Hospital (TPCH) have given an Adelaide teenager a future without fear after performing a ground-breaking procedure to fix her ailing heart.

Monica Tolic, 17, was the Prince Charles Hospital’s 100th patient to have a Personalised External Aortic Root Support (PEARS) implant. The procedure is designed to reinforce the aortic wall in patients who have aortic dilatation or expansion. In Monica’s case this dilatation was a result of a rare genetic condition, Filam A, which affects the connective tissue as well as the heart and lungs. Monica’s aorta had become enlarged, putting it risk of rupture.

The PEARS procedure uses a soft, fabric, custom-fitted external aortic support (like a scaffold) which reinforces the aortic wall and stops it from expanding. This avoids the need for aortic wall replacement.

Dr Livia Williams said that PEARS procedure was an operation designed to help avoid an aortic dissection in patients with genetic conditions such as Filam A, Marfan’s syndrome or other connective tissue disorders which cause continual enlargement of the aorta.

“If a patient develops aortic dissection, it’s an emergency with an extremely high operative risk. Even without aortic dissection, continued expansion of the aortic root can result in a severely leaky aortic valve and heart failure,” Dr Williams said.

“Monica is very young to have had this procedure however it has been performed in patients as young as three years of age. Monica’s aortic wall was particularly thin in one area and was at the point where it needed intervention. It was very fortunate Monica’s mum approached us when she did.”

Dr Williams said that the psychological burden of having a dilated aorta is something very much underappreciated in the scientific literature.

“Our team’s lead surgeon, Dr Peter Tesar, is a strong advocate for discussing intervention with suitable patients once their lifestyle, or quality of life becomes restricted by the disease process,” she said.

Monica said she was very grateful for the surgery because she had a ‘ticking timebomb’ in her chest.

“I could have been dead if I’d waited another three years and it ruptured. I was surprised and a bit scared when they told me afterwards how thin my aorta was,” Monica said.

In 2018 Dr Williams invited the world’s first PEARS surgeon, Mr John Pepper, to present the data on PEARS at an Australian Cardiac Surgery conference. The notion of an aortic operation which could be performed without the use of cardiopulmonary bypass or aortic valve intervention was both inspiring and ground-breaking.

The first PEARS implants were then performed at TPCH in 2019 under the proctorship of Mr Conal Austin, a leading UK PEARS Surgeon. Following this, TPCH cemented a formal PEARS program under the leadership of renowned local aortic surgeon, Dr Peter Tesar and paediatric cardiologist Dr Chris Whight. Whilst the surgery has a much lower operative risk, the delicate and treacherous nature of the surgery means the operation requires a highly specialised team.

“PEARS is a gamechanger for people with enlarged aortas. It allows people to have their lives back and not live in fear,” Dr Williams said.

“It also provides a safer alternative to traditional cardiac surgery as we don’t need a heart lung machine for the operation. This is a huge advancement in the field.”

Since first starting this procedure, TPCH PEARS team has achieved outstanding results, with a 100 percent survival rate and the hospital has become a national and international referral centre for the unique operation.