Livia hool laboratory wa

Enlarged heart syndrome

World-class imaging helping to tackle enlarged heart syndrome

29 September 2022

Victor Chang Cardiac Research Institute scientists based at The University of Western Australia are utilising world class imaging techniques to develop heart medications to tackle a common genetic heart condition.

The team at UWA is tracking a molecule targeted to the heart using the National Imaging Facility’s in vivo imaging techniques. It’s allowing them to take a step closer to developing the first therapeutic for preventing – and even reversing – hypertrophic cardiomyopathy (HCM).

They have identified a mechanism by which the powerhouse of the cell, mitochondria, use large amounts of oxygen to enlarge the heart wall in people with HCM – the leading cause of sudden cardiac death in five-to-fifteen-year-olds.

Professor Livia Hool , the Wesfarmers UWA-Victor Chang Cardiac Research Institute Chair in Cardiovascular Research said because HCM was hereditary screening at-risk family members would identify people to target for prevention.

“Prevention is better than cure with HCM,” said Professor Hool, who leads the research.

“Genetic testing is now much cheaper so it will become more common and that will help to identify the people who will benefit from HCM prevention strategies.

“At the moment, people don’t know they have HCM until they develop symptoms such as chest pain, shortness of breath and fatigue or tragically suffer cardiac arrest.

“But there is presently no treatment that can reverse or prevent HCM.”

In vivo multispectral fluorescence imaging at NIF’s Centre for Microscopy, Characterisation and Analysis (CMCA) tracked a molecule called AID-TAT that decreases oxygen utilisation by the mitochondria and prevents the enlargement of the heart.

NIF Fellow Diana Patalwala from The University of Western Australia (UWA) said a fluorescent dye was added to the AID-TAT molecule, to assess its in vivo cardiac uptake and biodistribution. It’s hoped by modulating cardiac metabolic activity it will be beneficial in preventing HCM in ‘at risk’ patients who carry the genetic mutation.

Professor Hool said the imaging was assisting the team move towards clinical trials to demonstrate safety and efficacy.

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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.

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