InfiltrativeCardiomyopathyGroup

Key Research Areas

Never in the clinical history of the disease have treatments been this available for cardiac amyloid. Dr Nikki Bart and her team are facilitating research and clinical advances to provide life-saving treatments for patients with heart failure.

• Amyloid cardiomyopathy
• Infiltrative cardiomyopathy
• Early disease identification in cardiomyopathy
• Cardiac Genetics
• Sarcoidosis

Research Overview

Amyloid cardiomyopathy is a devastating multi-system disorder that frequently involves the heart. Untreated, the prognosis is extremely poor. Recently, disease specific therapy has become available, opening up new opportunities to dramatically improve patient outcomes through early diagnosis and treatment.

The St Vincent's Hospital/Victor Chang Cardiac Research Institute multidisciplinary amyloid clinic is a state-of-the art clinic which offers input from experts in cardiology, hematology, neurology and genetics. Patients are comprehensively worked up, and then stream-lined into research and treatment opportunities.

The research goals are to better understand this rare condition, to identify early disease, and to offer patients effective therapy for their specific clinical situation.

There are five key projects underway in the Infiltrative Cardiomyopathy Laboratory, led by Dr Nikki Bart;

1. Cardiac genetic variants and cardiac amyloidosis in heart failure with preserved ejection fraction

Genetic testing is rapidly becoming mainstream for several cardiac conditions with the recent MBS approval of genetic testing in inherited cardiomyopathies.

This study is using 'genetics first' approach to uncover patients with cardiac amyloid in a heart failure with preserved ejection fraction cohort. This will allow for early identification of patients with a treatable cause of cardiomyopathy, as well as identification of family members.

2. Diagnostic gains of histopathology on left ventricular assist device (LVAD) core and heart transplant explants, with a focus on restrictive cardiomyopathies

This study is assessing how the clinical diagnosis pre-transplant correlates to the histopathological diagnosis in patients with advanced heart failure undergoing LVAD or heart transplant. There is very literature available on the histopathological diagnosis of restrictive cardiomyopathies, of which amyloid and sarcoid are the leading two causes. This will be important for changing clinical diagnosis paradigms in the future.

3. Establishment of a state-of-the-art multidisciplinary (MDT) amyloid clinic

Amyloid is a rare multi-system disease and needs a multi-disciplinary approach to address the level of complexity of each patient. Dr. Bart is the lead of the Amyloid MDT at St Vincent's Hospital/Victor Chang Cardiac Research Institute and supervisor of the inaugural cardiac amyloid fellow appointed in 2021.

4. NSW TTR database

Establishment of a hospital based, and expansion into NSW amyloid database. Thill enable an understanding of the longitudinal outcomes of patients with systemic amyloidosis including a comparison between the subtypes of systemic amyloidosis. It will allow for streamlining of patients into clinical trials.

5. Biobanking of cardiac amyloid tissue

This project will allow for longitudinal assessment of genomic and proteomic markers of multiorgan disease progression. Serum and plasma samples as well as pathology taken from endomyocardial biopsy, LVAD explant and explanted native hearts will be used.

Book Chapters

1. Hungerford SL, Bart NK, Adji A. Cardiac Magnetic Resonance Techniques to Determine Systemic Impedance in Healthy Individuals and Cardiovascular Disease States. Horizons in World Cardiovascular Research. Volume 22. 2019. ISBN: 978-1-53616-157-1

Journal Articles

1. Bart NK; Thomas L; Korczyk D; Atherton JJ; Stewart GJ; Fatkin D, 2020, 'Amyloid Cardiomyopathy', Heart Lung and Circulation, vol. 29, pp. 575 - 583, https://www.heartlungcirc.org/article/S1443...
2. Bart NK; Curtis MK; Cheng HY; Hungerford SL; McLaren R; Petousi N; Dorrington KL; Robbins PA, 2016, 'Elevation of iron storage in humans attenuates the pulmonary vascular response to hypoxia', Journal of Applied Physiology, vol. 121, pp. 537 - 544, https://journals.physiology.org/doi/full/10.1152...
3. Bart NK; Hungerford SL; Curtis MK; Cheng HY; Southern JL; Petousi N; Dorrington KL; Robbins PA, 2018, 'Effects of modest iron loading on iron indices in healthy individuals', Journal of Applied Physiology, vol. 125, pp. 1710 - 1719, https://journals.physiology.org/doi/full/10.1152...
4. Bart NK; Kearney K; Macdonald PS, 2020, '‘Put on your own oxygen mask before helping others’: mitigating health care worker risk from COVID-19', Internal Medicine Journal, vol. 50, pp. 891 - 892, https://onlinelibrary.wiley.com/doi/10.1111...
5. Hungerford SL; Adji AI; Bart NK; Lin L; Namasivayam MJ; Schnegg B; Jabbour A; O'Rourke MF; Hayward CS; Muller DWM, 2021, 'A novel method to assess valvulo-arterial load in patients with aortic valve stenosis', Journal of hypertension, vol. 39, pp. 437 - 446, https://journals.lww.com/jhypertension/Abstract/2021/03000...
6. Hungerford SL; Adji AI; Bart NK; Lin L; Song N; Jabbour A; O'Rourke MF; Hayward CS; Muller DWM, 2021, 'Ageing, hypertension and aortic valve stenosis – Understanding the series circuit using cardiac magnetic resonance and applanation tonometry', International Journal of Cardiology: Hypertension, vol. 9, pp. 100087 - 100087, sciencedirect.com/science/article/pii/S259008
7. Hungerford S; Bart N; Jansz P; Kay S; Emmanuel S; Namasivayam M; Dahle G; Duncan A; Hayward C; Muller DWM, 2021, 'Improved right ventricular function following transapical transcatheter mitral valve implantation for severe mitral regurgitation', IJC Heart and Vasculature, vol. 32, sciencedirect.com/science/article/pii/S235290...
8. Kearney K; Lau EM; Darley D; Romfh A; Bart N; Kotlyar E; Hayes D; Khush K; Keogh A, 2021, 'Waitlist and post-transplant outcomes for eisenmenger syndrome: A comparison of transplant strategies', Journal of Heart and Lung Transplantation, https://www.jhltonline.org/article/S1053-2498...
9. Hungerford SL; Bart NK, 2021, 'Editorial for “Diverse Right Ventricular Remodelling Evaluated by Cardiac Magnetic Resonance Imaging and Prognosis in Eisenmenger Syndrome with Different Shunt Locations”', Journal of Magnetic Resonance Imaging, https://onlinelibrary.wiley.com/doi/10.1002...
10. Lal S; Hayward CS; De Pasquale C; Kaye D; Javorsky G; Bergin P; Atherton JJ; Ilton MK; Weintraub RG; Nair P; Rudas M; Dembo L; Doughty RN; Kumarasinghe G; Juergens C; Bannon PG; Bart NK; Chow CK; Lattimore JD; Kritharides L; Totaro R; Macdonald PS, 2020, 'COVID-19 and Acute Heart Failure: Screening the Critically Ill – A Position Statement of the Cardiac Society of Australia and New Zealand (CSANZ)', Heart Lung and Circulation, vol. 29, pp. e94 - e98, https://www.heartlungcirc.org/article/S1443-9506...
11. Bruno S, Nicole B, Nila J D, Gail M, James N, Peter S M, Christopher S H. Heart Transplantation From Hepatitis C-Positive Donors in the Era of Direct Acting Antiviral Therapy: A Comprehensive Literature Review. Transplant Direct. 2019 Aug 23;5(9):e486. doi: 10.1097/TXD.0000000000000928. PMID: 31579814; PMCID: PMC6739042.
12. Kempton HR, Bemand T, Bart NK, Suttie JJ. Using Coronary Artery Calcium Scoring as Preventative Health Tool to Reduce the High Burden of Cardiovascular Disease in Indigenous Australians. Heart Lung Circ. 2020 Jun;29(6):835-839. doi: 10.1016/j.hlc.2019.07.003. Epub 2019 Jul 21. PMID: 31378566.
13. Iliff J; Bart NK; Ghaly S; Granger E; Holloway CJ, 2019, 'Pericardial Mesothelioma in a 35-Year-Old Male With Ulcerative Colitis', Circulation. Cardiovascular imaging, vol. 12, pp. e008659, https://www.ahajournals.org/doi/10.1161..
14. Cheng HY; Frise MC; Curtis MK; Bart NK; Petousi N; Talbot NP; Balanos GM; Robbins PA; Dorrington KL, 2019, 'Intravenous iron delivers a sustained (8-week) lowering of pulmonary artery pressure during exercise in healthy older humans', Physiological Reports, vol. 7, https://physoc.onlinelibrary.wiley.com/doi/10.14814...
15. Nickol AH; Frise MC; Cheng HY; McGahey A; McFadyen BM; Harris-Wright T; Bart NK; Curtis MK; Khandwala S; O'Neill DP; Pollard KA; Hardinge FM; Rahman NM; Armitage AE; Dorrington KL; Drakesmith H; Ratcliffe PJ; Robbins PA, 2015, 'A cross-sectional study of the prevalence and associations of iron deficiency in a cohort of patients with chronic obstructive pulmonary disease', BMJ Open, vol. 5, A cross-sectional study of the prevalence and associations of iron deficiency in a cohort of patients with chronic obstructive pulmonary disease

Selected Conference Abstracts

1. Bart NK; Hungerford SL; Curtis MK; Cheng HY; Southern JL; Petousi N; Dorrington KL; Robbins PA, 2018, 'Effects of modest iron loading on iron indices in healthy individuals', Journal of Applied Physiology, vol. 125, pp. 1710 - 1719, https://journals.physiology.org/doi/full/10.1152...
2. Bart N; Hungerford S; Jacobs N; Granger E, 2018, '‘Now You See It, Now You Don’t’: A Rare Case of Pulmonary Artery Occlusion and Circulatory Collapse Due to Intravascular Extension of a Renal Cell Carcinoma to the Right Heart With Tumour Embolisation', Heart, Lung and Circulation, vol. 27, pp. S373 - S373, https://www.heartlungcirc.org/article/S1443...
3. Bart N; Parkinson M; Hungerford S; Brennan X; Gunalingam B, 2018, 'Staphylococcus aureus Pericarditis Presenting as Cardiac Tamponade Caused by Diabetic Foot Osteomyelitis', Heart, Lung and Circulation, vol. 27, pp. S275 - S275, https://www.heartlungcirc.org/article/S1443-9..
4. Bart N; Kearney K; McGrath-Cadell L; Hungerford S; Bensted K; Baron D; Muller D; Roy D, 2018, 'Superior Short-Term Haemodynamic Outcomes When Comparing Transcatheter Aortic Valve Implantation to Sutureless Aortic Valve Replacement Using a Perceval Valve', Heart, Lung and Circulation, vol. 27, pp. S481 - S481, https://www.heartlungcirc.org/article/S1443-95...
5. Kempton H; Jones K; Carroll J; Kearney K; Meredith T; Arriagada A; Bart N; Baron D; Muller D; Roy D, 2020, '786 Antithrombotic Therapy for Management of Early Thrombotic Risk in Transcatheter Aortic Valve Implantation: An Australian Experience', Heart, Lung and Circulation, vol. 29, pp. S390 - S390, https://www.heartlungcirc.org/article/S1443-950...
6. Meredith T; Kearney K; Carroll J; Kempton H; Jones K; Bart N; Roy D; Muller D, 2020, '815 Demographics and Outcomes of Transcatheter Aortic Valve Implantation (TAVI) in Renal Dialysis Patients: A Retrospective Study', Heart, Lung and Circulation, vol. 29, pp. S404 - S404, https://www.heartlungcirc.org/article/S1443...
7. Kearney K; Kempton H; Jones K; Carroll J; Meredith T; Bart N; Arrigiada A; Baron D; Muller D; Roy D, 2020, '828 Factors Influencing Periprocedural Stroke at the Time of Transcatheter Aortic Valve Implantation in the Era of Cerebral Protection', Heart, Lung and Circulation, vol. 29, pp. S409 - S410, https://www.heartlungcirc.org/article/S1443...
8. Kearney K; Kempton H; Jones K; Carroll J; Meredith T; Bart N; Arrigiada A; Baron D; Muller D; Roy D, 2020, '852 Long Term Follow-Up and Durability of Transcatheter Aortic Valve Implantation Devices', Heart, Lung and Circulation, vol. 29, pp. S420 - S420, https://www.heartlungcirc.org/article/S1443...
9. Kempton H; Jones K; Carroll J; Meredith T; Kearney K; Arriagada A; Bart N; Baron D; Muller D; Roy D, 2020, '855 Methods of Vascular Access for Transcatheter Aortic Valve Implantation (TAVI) at an Australian Centre', Heart, Lung and Circulation, vol. 29, pp. S421 - S421, https://www.heartlungcirc.org/article/S144...
10. Kempton H; Carroll J; Jones K; Meredith T; Kearney K; Arriagada A; Bart N; Baron D; Muller D; Roy D, 2020, '896 The Impact of Obesity on Transcatheter Aortic Valve Implantation Outcomes', Heart, Lung and Circulation, vol. 29, pp. S439 - S440, https://www.heartlungcirc.org/article/S1443...
11. Jones K; Kearney K; Carroll J; Kempton H; Meredith T; Phan J; Arriagada A; Bart N; Baron D; Muller D; Roy D, 2020, '912 Valve-in-Valve Transcatheter Aortic Valve Implantation for Failing Aortic Valve Prostheses: an Australian Interventional Experience', Heart, Lung and Circulation, vol. 29, pp. S446 - S446, https://www.heartlungcirc.org/article/S1443...