Hope is where the heart is

Lisa Paisley is no stranger to heart disease. Not only was she born with a heart defect, but so were all three of her children.

The impact of this has been great, but improvements in the treatments and outcomes for children with heart disease has meant the journey for Lisa and her family has not been without hope. 

In 1980, when Lisa was born with a heart murmur, echocardiographs (ECG) had only just been introduced as a way to monitor the heart and how it functions, stents had just been discovered as a way to help open up a narrowed artery and open heart surgery was the only option for kids needing their heart repaired.

Fast forward to 2016, the year Lisa’s first child, Xander, was born. Echocardiographs were considered routine, keyhole procedures had been introduced as a non-invasive alternative to some surgeries and those needing open heart surgery were having better outcomes than ever before.

Xander was one of those who needed open heart surgery.

The beginning of the journey

Initially detected at Lisa’s 12-week pregnancy scan, Xander was born with an extremely rare form of heart disease. His condition included congenitally corrected Transposition of the Great Artery (TGA), a defect where both ventricles (pumping chambers) of the heart are reversed, Pulmonary Atresia, where the valve that controls blood flow from the heart to the lungs doesn't form, and a Ventricular Septal Defect (VSD), causing a hole in the wall that separates the two lower chambers of the heart.

Forty years ago, a child like Xander wouldn’t have survived but thanks to state-of-the-art equipment and technology, and the expertise of teams at Sydney Children’s Hospitals Network, Xander’s complex condition could be faced head on with equally complex surgery.

In his first year of life, Xander underwent three different surgeries to correct his defect; one to place a shunt and create a pathway from his heart to his lungs, another to switch his arteries, close his VSD and remove shunt, and a third to place a permanent pacemaker.

During this tumultuous time, Lisa also found out she was expecting her second child, Scarlett.

The process starts again

Scarlett was diagnosed with a heart condition at her 19-week scan, right as Xander was preparing for his first big surgery.

“After Xander’s first major surgery, he spent five weeks in hospital recovering. We were home for 10 days and then I was back delivering Scarlett to do it all over again,” Lisa said.

Like her brother, Scarlett was born with TGA, as well as single indeterminate ventricle, meaning she only had one ventricle that could not be defined as left or right, and hyperplastic aortic arch with severe coarctation, where the aorta was too narrow to pump blood effectively around the body.

Scarlett was four days old when she had the first of three surgeries to correct her blood circulation. The surgery, known as the Norwood procedure, is highly complex and involves reconnecting the heart to allow the right ventricle to pump blood to the brain, where it passively flows to the body.

At five months old Scarlett underwent the second stage of surgery, the Glenn procedure, to allow the blood from the upper part of the body to flow directly to the pulmonary artery, which then takes blood to the lungs.

Both surgeries were successful, however Scarlett was still very unwell.

“Scarlett had only just been moved to the ward when she started having trouble breathing. A rapid response was called and she was rushed to intensive care. We came so close to losing her that day,” Lisa said.

“Thankfully she recovered but she was still always pale with purple lips, fingertips and toes and breathless.”

Lisa's third heart baby

After four and a half months, Scarlett was able to come home and with the next surgery not scheduled for another three and a half years, Lisa was able to start learning how to navigate life with two heart babies. While adjusting to this new normal, Lisa fell pregnant with Allegra.

“Allegra was our surprise baby and knowing what I’d been through with Xander and Scarlett, I requested early scans. At 17 weeks everything looked normal but a foetal scan at 20 weeks confirmed I was having my third heart baby,” Lisa said. 

Allegra was born with a range of complex heart defects including Dextro-Transposition of the Great Arteries, where the two main heart valves are switched, hypoplastic right heart syndrome with a cleft mitral valve, meaning the right side of her heart hadn’t developed properly, a VSD, like Xander, and hyperplastic aortic arch with severe coarctation, like Scarlett.

Allegra became the youngest in her family to have surgery, having her first operation to switch her arteries and repair her hyperplastic aortic arch and coarctation at just two days old.

Ten months later, Allegra had the Glenn procedure, the same surgery as her big sister, to help correct the blood flow throughout her body. This helped to manage her condition for the immediate future.

Surgery for Scarlett continues

After Allegra was able to come home, and a brief break from hospital visits, it was Scarlett’s turn again to enter the operating theatre for the third, and final, stage of her surgeries.

Scarlett underwent the Fontan procedure earlier this year to redirect the way deoxygenated blood is carried around the body, so it is circulated to the lungs without passing through the heart.

This highly complex surgery completed the reconstruction of Scarlett’s heart. The difference could be seen almost immediately.

“After Scarlett’s third operation, she was finally a ‘pink’ girl! She was able to come home, well, full of breath and full of energy. She was the best she’d ever been,” Lisa said.

Life after hospital

Today, all three of Lisa’s children are at home and doing well. They are finally having a chance to enjoy their childhood, and Lisa is finally having a chance to just be a mum.

“For almost two years, hospital was my safe space because they understood what I was going through. Now that hospital visits are less frequent, and my children are well, I have discovered what it means to be a mum, beyond being a heart mum,” Lisa said.

Xander and Allegra have more surgery on the horizon, but until then, Lisa intends to make the most of every moment.

“My experience has taught me to focus on what’s important, take nothing for granted and celebrate even the smallest things.”

“The bond I have with my children is indescribable. I am their biggest fan and advocate. I will do absolutely anything for them and want to make sure they are able to live every day to the fullest,” Lisa said.

The impact of heart disease has certainly taken a toll on Lisa and her family, but through her experience Lisa has been able to witness firsthand the difference made by advancements in equipment, technology and research. It has given her children a chance at a normal life - and for this, Lisa is beyond grateful

“My children received the best possible care and because of that, they are here today. I couldn’t ask for more than that.”

Lisa is a member of our Family Advisory Committee and has used her experience to help shape our new Cardiac Model of Care. Read more about it here.