Volume 14, Issue 6 pp. 987-995

ORIGINAL ARTICLE

Prevalence and risk factors for low bone density in adults with a Fontan circulation

Paolo D’Ambrosio MBBS(Hons),

Paolo D’Ambrosio MBBS(Hons)

orcid.org/0000-0002-8459-2140

Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia

Faculty of Medicine and Health Sciences, University of Sydney, Sydney, New South Wales, Australia

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Derek Tran BAppSc(ExPhys),

Derek Tran BAppSc(ExPhys)

Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia

Faculty of Medicine and Health Sciences, University of Sydney, Sydney, New South Wales, Australia

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Charlotte E. Verrall BSc(Hons),

Charlotte E. Verrall BSc(Hons)

TheHeart Centre for Children, The Children’s Hospital at Westmead, Sydney, New South Wales, Australia

Discipline of Child and Adolescent Health, Sydney Medical School, Faculty of Health and Medicine, University of Sydney, Sydney, New South Wales, Australia

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Chantal Attard BSc(Hons),

Chantal Attard BSc(Hons)

orcid.org/0000-0003-1851-7836

Murdoch Children’s Research Institute, Royal Children’s Hospital, Melbourne, Victoria, Australia

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Maria Fiatarone Singh MD,

Maria Fiatarone Singh MD

Physical Activity, Lifestyle, Ageing and Wellbeing Faculty Research Group, Sydney Medical School, Faculty of Health Sciences, The University of Sydney, Sydney, New South Wales, Australia

Hebrew SeniorLife and Jean Mayer USDA Human Nutrition Research Center on Ageing, Tufts University, Boston, Massachusetts

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Julian Ayer PhD,

Julian Ayer PhD

TheHeart Centre for Children, The Children’s Hospital at Westmead, Sydney, New South Wales, Australia

Discipline of Child and Adolescent Health, Sydney Medical School, Faculty of Health and Medicine, University of Sydney, Sydney, New South Wales, Australia

Charles Perkins Centre, University of Sydney, Sydney, New South Wales, Australia

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Yves d’Udekem PhD,

Yves d’Udekem PhD

Murdoch Children’s Research Institute, Royal Children’s Hospital, Melbourne, Victoria, Australia

Department of Cardiothoracic Surgery, Royal Children’s Hospital, Melbourne, Victoria, Australia

Department of Pediatrics, University of Melbourne, Melbourne, Victoria, Australia

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Stephen Twigg PhD,

Stephen Twigg PhD

Faculty of Medicine and Health Sciences, University of Sydney, Sydney, New South Wales, Australia

Department of Endocrinology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia

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David S. Celermajer PhD,

David S. Celermajer PhD

orcid.org/0000-0001-7640-0439

Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia

Faculty of Medicine and Health Sciences, University of Sydney, Sydney, New South Wales, Australia

Heart Research Institute, Sydney, New South Wales, Australia

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Rachael Cordina PhD,

Corresponding Author

Rachael Cordina PhD

[email protected]

Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia

Faculty of Medicine and Health Sciences, University of Sydney, Sydney, New South Wales, Australia

Murdoch Children’s Research Institute, Royal Children’s Hospital, Melbourne, Victoria, Australia

Correspondence

Dr Rachael Cordina, Department of Cardiology, Royal Prince Alfred Hospital, 50 Missenden Road, Camperdown, NSW 2050.

Email: [email protected]

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Paolo D’Ambrosio MBBS(Hons),

Paolo D’Ambrosio MBBS(Hons)

orcid.org/0000-0002-8459-2140

Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia

Faculty of Medicine and Health Sciences, University of Sydney, Sydney, New South Wales, Australia

Search for more papers by this author

Derek Tran BAppSc(ExPhys),

Derek Tran BAppSc(ExPhys)

Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia

Faculty of Medicine and Health Sciences, University of Sydney, Sydney, New South Wales, Australia

Search for more papers by this author

Charlotte E. Verrall BSc(Hons),

Charlotte E. Verrall BSc(Hons)

TheHeart Centre for Children, The Children’s Hospital at Westmead, Sydney, New South Wales, Australia

Discipline of Child and Adolescent Health, Sydney Medical School, Faculty of Health and Medicine, University of Sydney, Sydney, New South Wales, Australia

Search for more papers by this author

Chantal Attard BSc(Hons),

Chantal Attard BSc(Hons)

orcid.org/0000-0003-1851-7836

Murdoch Children’s Research Institute, Royal Children’s Hospital, Melbourne, Victoria, Australia

Search for more papers by this author

Maria Fiatarone Singh MD,

Maria Fiatarone Singh MD

Physical Activity, Lifestyle, Ageing and Wellbeing Faculty Research Group, Sydney Medical School, Faculty of Health Sciences, The University of Sydney, Sydney, New South Wales, Australia

Hebrew SeniorLife and Jean Mayer USDA Human Nutrition Research Center on Ageing, Tufts University, Boston, Massachusetts

Search for more papers by this author

Julian Ayer PhD,

Julian Ayer PhD

TheHeart Centre for Children, The Children’s Hospital at Westmead, Sydney, New South Wales, Australia

Discipline of Child and Adolescent Health, Sydney Medical School, Faculty of Health and Medicine, University of Sydney, Sydney, New South Wales, Australia

Charles Perkins Centre, University of Sydney, Sydney, New South Wales, Australia

Search for more papers by this author

Yves d’Udekem PhD,

Yves d’Udekem PhD

Murdoch Children’s Research Institute, Royal Children’s Hospital, Melbourne, Victoria, Australia

Department of Cardiothoracic Surgery, Royal Children’s Hospital, Melbourne, Victoria, Australia

Department of Pediatrics, University of Melbourne, Melbourne, Victoria, Australia

Search for more papers by this author

Stephen Twigg PhD,

Stephen Twigg PhD

Faculty of Medicine and Health Sciences, University of Sydney, Sydney, New South Wales, Australia

Department of Endocrinology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia

Search for more papers by this author

David S. Celermajer PhD,

David S. Celermajer PhD

orcid.org/0000-0001-7640-0439

Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia

Faculty of Medicine and Health Sciences, University of Sydney, Sydney, New South Wales, Australia

Heart Research Institute, Sydney, New South Wales, Australia

Search for more papers by this author

Rachael Cordina PhD,

Corresponding Author

Rachael Cordina PhD

[email protected]

Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia

Faculty of Medicine and Health Sciences, University of Sydney, Sydney, New South Wales, Australia

Murdoch Children’s Research Institute, Royal Children’s Hospital, Melbourne, Victoria, Australia

Correspondence

Dr Rachael Cordina, Department of Cardiology, Royal Prince Alfred Hospital, 50 Missenden Road, Camperdown, NSW 2050.

Email: [email protected]

Search for more papers by this author

First published: 20 August 2019

https://doi.org/10.1111/chd.12836

Citations: 14

All authors takes responsibility for all aspects of the reliability and freedom from bias of the data presented and their discussed interpretation.

Funding information

NHMRC grant APP1065794: ‘Functional outcomes after Fontan Surgery’.

NHMRC partnership grant 1076849.

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Abstract

Objective and Patients

This study aimed to characterize bone mineral density abnormalities and pathophysiological associations in young adults living with a Fontan circulation.

Design

Participants underwent bone mineral density measurement using dual-energy X-ray absorptiometry and serum biochemical analysis, cardiopulmonary exercise and strength testing and transthoracic echocardiography.

Results

In our cohort (n = 28), 29% had osteopenic-range bone mineral density and one patient was osteoporotic (average hip t score: −0.6 ± 1.1; spine t score: −0.6 ± 0.9). Four patients (14%) had z scores < −2.0. Parathyroid hormone levels were increased compared with laboratory median (6.1 ± 3.5 vs 4 pmol/L, P = .01) and 27% had 25-hydroxy-vitamin D < 50 nmol/L. 25-hydroxy-vitamin D negatively correlated with parathyroid hormone (ρ = −0.53, P = .01) suggesting secondary hyperparathyroidism. Atrioventricular valve systolic to diastolic duration ratio, an echocardiographic measure of diastolic dysfunction, inversely correlated with hip t and z scores (P < .01). Hip t scores were positively associated with oxygen saturations (ρ = 0.45, P = .05) and tended to be inversely associated with parathyroid hormone levels (ρ = −0.44, P = .07) and N-Terminal pro b-type natriuretic peptide (ρ = −0.42, P = .08).

Conclusions

Many young adults with a Fontan circulation have abnormal bone mineral density. The underlying pathophysiology is likely multifactorial. Possible contributors include secondary hyperparathyroidism, hypoxemia, diastolic cardiac dysfunction and neurohormonal activation. As low bone mineral density is clinically relevant and potentially treatable, assessment of bone mineral density should be part of routine care in this cohort.

CONFLICTS OF INTEREST

Professor Yves d'Udekem is a Clinician Practitioner Fellow of the NHMRC (1082186). Murdoch Children's Research Institute is supported by the Victorian Government's Operational Infrastructure Support Program.

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Citing Literature

Volume14, Issue6

November/December 2019

Pages 987-995

Prevalence and risk factors for low bone density in adults with a Fontan circulation

Abstract

Objective and Patients

Design

Results

Conclusions

CONFLICTS OF INTEREST

REFERENCES

Citing Literature

References

Information

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Prevalence and risk factors for low bone density in adults with a Fontan circulation

Abstract

Objective and Patients

Design

Results

Conclusions

CONFLICTS OF INTEREST

REFERENCES

Citing Literature

References

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