500K to ‘Big Heart Syndrome'
Victor Chang Cardiac Research Institute Scientists in Western Australia to receive funding boost from CSL to advance hypertrophic cardiomyopathy trials
29 April 2021
The funding boost by global biotech giant CSL will be used to drive research into hypertrophic cardiomyopathy, the world’s leading cause of cardiac death for children aged between five and 15.
It is hoped the award will help Professor Livia Hool and her WA-based team deliver through on their breakthrough discovery, which could potentially both prevent and reverse the damage of the inherited heart disease which causes the heart to become dangerously large.
“This disease affects around one in 500 people and could be as common as one in every 200. It’s shocking that we have no way of preventing or reversing the damage to the heart. All we can do now is alleviate the symptoms,” says Professor Livia Hool.
‘This funding could make a huge difference to the lives of people not just here in Australia but around the world and provide hope to families who live with this condition every day.”
Using mouse models, Professor Livia Hool and her team at the University of Western Australia discovered that by targeting a calcium channel in the heart with medication they could not only prevent the disease from occurring altogether but may also be able to reverse it.
![Professor Livia Hool Portrait Professor Livia Hool Portrait](https://victorchang-cdn.imgix.net/assets/src/uploads/Livia-Hool-Horizontal.png?auto=compress%2Cformat&crop=focalpoint&fit=crop&fp-x=0.5&fp-y=0.5&h=80&q=60&w=120 120w, https://victorchang-cdn.imgix.net/assets/src/uploads/Livia-Hool-Horizontal.png?auto=compress%2Cformat&crop=focalpoint&fit=crop&fp-x=0.5&fp-y=0.5&h=160&q=60&w=240 240w, https://victorchang-cdn.imgix.net/assets/src/uploads/Livia-Hool-Horizontal.png?auto=compress%2Cformat&crop=focalpoint&fit=crop&fp-x=0.5&fp-y=0.5&h=240&q=60&w=360 360w, https://victorchang-cdn.imgix.net/assets/src/uploads/Livia-Hool-Horizontal.png?auto=compress%2Cformat&crop=focalpoint&fit=crop&fp-x=0.5&fp-y=0.5&h=319&q=60&w=480 480w, https://victorchang-cdn.imgix.net/assets/src/uploads/Livia-Hool-Horizontal.png?auto=compress%2Cformat&crop=focalpoint&fit=crop&fp-x=0.5&fp-y=0.5&h=399&q=60&w=600 600w, https://victorchang-cdn.imgix.net/assets/src/uploads/Livia-Hool-Horizontal.png?auto=compress%2Cformat&crop=focalpoint&fit=crop&fp-x=0.5&fp-y=0.5&h=479&q=60&w=720 720w, https://victorchang-cdn.imgix.net/assets/src/uploads/Livia-Hool-Horizontal.png?auto=compress%2Cformat&crop=focalpoint&fit=crop&fp-x=0.5&fp-y=0.5&h=559&q=60&w=840 840w, https://victorchang-cdn.imgix.net/assets/src/uploads/Livia-Hool-Horizontal.png?auto=compress%2Cformat&crop=focalpoint&fit=crop&fp-x=0.5&fp-y=0.5&h=639&q=60&w=960 960w, https://victorchang-cdn.imgix.net/assets/src/uploads/Livia-Hool-Horizontal.png?auto=compress%2Cformat&crop=focalpoint&fit=crop&fp-x=0.5&fp-y=0.5&h=719&q=60&w=1080 1080w, https://victorchang-cdn.imgix.net/assets/src/uploads/Livia-Hool-Horizontal.png?auto=compress%2Cformat&crop=focalpoint&fit=crop&fp-x=0.5&fp-y=0.5&h=798&q=60&w=1200 1200w, https://victorchang-cdn.imgix.net/assets/src/uploads/Livia-Hool-Horizontal.png?auto=compress%2Cformat&crop=focalpoint&fit=crop&fp-x=0.5&fp-y=0.5&h=878&q=60&w=1320 1320w, https://victorchang-cdn.imgix.net/assets/src/uploads/Livia-Hool-Horizontal.png?auto=compress%2Cformat&crop=focalpoint&fit=crop&fp-x=0.5&fp-y=0.5&h=958&q=60&w=1440 1440w, https://victorchang-cdn.imgix.net/assets/src/uploads/Livia-Hool-Horizontal.png?auto=compress%2Cformat&crop=focalpoint&fit=crop&fp-x=0.5&fp-y=0.5&h=1038&q=60&w=1560 1560w, https://victorchang-cdn.imgix.net/assets/src/uploads/Livia-Hool-Horizontal.png?auto=compress%2Cformat&crop=focalpoint&fit=crop&fp-x=0.5&fp-y=0.5&h=1118&q=60&w=1680 1680w, https://victorchang-cdn.imgix.net/assets/src/uploads/Livia-Hool-Horizontal.png?auto=compress%2Cformat&crop=focalpoint&fit=crop&fp-x=0.5&fp-y=0.5&h=1198&q=60&w=1800 1800w, https://victorchang-cdn.imgix.net/assets/src/uploads/Livia-Hool-Horizontal.png?auto=compress%2Cformat&crop=focalpoint&fit=crop&fp-x=0.5&fp-y=0.5&h=1278&q=60&w=1920 1920w, https://victorchang-cdn.imgix.net/assets/src/uploads/Livia-Hool-Horizontal.png?auto=compress%2Cformat&crop=focalpoint&fit=crop&fp-x=0.5&fp-y=0.5&h=1318&q=60&w=1980 1980w)
Hypertrophic cardiomyopathy is an inherited heart disease that can be incredibly aggressive in children but can also cause sudden cardiac arrest in otherwise healthy adults.
Professor Hool adds: “There are tens of thousands of Australians who have no idea they have this disease. If we could deliver a treatment and a way to reverse the damage to their hearts, this could save many lives and be life-changing.
“The funding from CSL will allow us to continue on with this vital work which is hugely exciting and incredibly welcome.”
It’s hoped the funding from CSL’s Research Acceleration Initiative will accelerate human trials which could start in the next three to five years.
The world-leading breakthrough builds upon more than a decade of work by Professor Hool and her WA team.
CSL’s Research Acceleration Initiative aims to fast-track discovery of innovative biotherapies through partnerships between CSL and global research organisations. These partnerships provide funding and access to industry resources for scientists working on novel biotherapeutic strategies in CSL’s therapeutic areas.
-- ENDS --
For all media enquiries and interview requests, please contact:
Julia Timms
Head, Media & Communications
j.timms@victorchang.edu.au
0457 517 355
“We are always grateful that we are not one of the families who receive the diagnosis at autopsy.“
- Nikki, Nate's mum
Acknowledgement of Country
The Victor Chang Cardiac Research Institute acknowledges Traditional Owners of Country throughout Australia and recognises the continuing connection to lands, waters and communities. We pay our respect to Aboriginal and Torres Strait Islander cultures; and to Elders past and present.