Longitudinal changes in cardiac function in Duchenne muscular dystrophy population as measured by magnetic resonance imaging
Abstract Background The lack of dystrophin in cardiomyocytes in Duchenne muscular dystrophy (DMD) is associated with progressive decline in cardiac function eventually leading to death by 20–40 years of age. The aim of this prospective study was to determine rate of progressive decline in left ventr...
| Main Authors: | , , , , , , , , , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
BMC
2022-06-01
|
| Series: | BMC Cardiovascular Disorders |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s12872-022-02688-5 |
| _version_ | 1818219001923239936 |
|---|---|
| author | Abhinandan Batra Alison M. Barnard Donovan J. Lott Rebecca J. Willcocks Sean C. Forbes Saptarshi Chakraborty Michael J. Daniels Jannik Arbogast William Triplett Erik K. Henricson Jonathan G. Dayan Carsten Schmalfuss Lee Sweeney Barry J. Byrne Craig M. McDonald Krista Vandenborne Glenn A. Walter |
| author_facet | Abhinandan Batra Alison M. Barnard Donovan J. Lott Rebecca J. Willcocks Sean C. Forbes Saptarshi Chakraborty Michael J. Daniels Jannik Arbogast William Triplett Erik K. Henricson Jonathan G. Dayan Carsten Schmalfuss Lee Sweeney Barry J. Byrne Craig M. McDonald Krista Vandenborne Glenn A. Walter |
| author_sort | Abhinandan Batra |
| collection | DOAJ |
| description | Abstract Background The lack of dystrophin in cardiomyocytes in Duchenne muscular dystrophy (DMD) is associated with progressive decline in cardiac function eventually leading to death by 20–40 years of age. The aim of this prospective study was to determine rate of progressive decline in left ventricular (LV) function in Duchenne muscular dystrophy (DMD) over 5 years. Methods Short axis cine and grid tagged images of the LV were acquired in individuals with DMD (n = 59; age = 5.3–18.0 years) yearly, and healthy controls at baseline (n = 16, age = 6.0–18.3 years) on a 3 T MRI scanner. Grid-tagged images were analyzed for composite circumferential strain (ℇcc%) and ℇcc% in six mid LV segments. Cine images were analyzed for left ventricular ejection fraction (LVEF), LV mass (LVM), end-diastolic volume (EDV), end-systolic volume (ESV), LV atrioventricular plane displacement (LVAPD), and circumferential uniformity ratio estimate (CURE). LVM, EDV, and ESV were normalized to body surface area for a normalized index of LVM (LVMI), EDV (EDVI) and ESV (ESVI). Results At baseline, LV ℇcc% was significantly worse in DMD compared to controls and five of the six mid LV segments demonstrated abnormal strain in DMD. Longitudinal measurements revealed that ℇcc% consistently declined in individuals with DMD with the inferior segments being more affected. LVEF progressively declined between 3 to 5 years post baseline visit. In a multivariate analysis, the use of cardioprotective drugs trended towards positively impacting cardiac measures while loss of ambulation and baseline age were associated with negative impact. Eight out of 17 cardiac parameters reached a minimal clinically important difference with a threshold of 1/3 standard deviation. Conclusion The study shows a worsening of circumferential strain in dystrophic myocardium. The findings emphasize the significance of early and longitudinal assessment of cardiac function in DMD and identify early biomarkers of cardiac dysfunction to help design clinical trials to mitigate cardiac pathology. This study provides valuable non-invasive and non-contrast based natural history data of cardiac changes which can be used to design clinical trials or interpret the results of current trials aimed at mitigating the effects of decreased cardiac function in DMD. |
| first_indexed | 2024-12-12T07:32:43Z |
| format | Article |
| id | doaj.art-4f3246949aea4b8fb84745347438167c |
| institution | Directory Open Access Journal |
| issn | 1471-2261 |
| language | English |
| last_indexed | 2024-12-12T07:32:43Z |
| publishDate | 2022-06-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Cardiovascular Disorders |
| spelling | doaj.art-4f3246949aea4b8fb84745347438167c2022-12-22T00:32:59ZengBMCBMC Cardiovascular Disorders1471-22612022-06-0122111210.1186/s12872-022-02688-5Longitudinal changes in cardiac function in Duchenne muscular dystrophy population as measured by magnetic resonance imagingAbhinandan Batra0Alison M. Barnard1Donovan J. Lott2Rebecca J. Willcocks3Sean C. Forbes4Saptarshi Chakraborty5Michael J. Daniels6Jannik Arbogast7William Triplett8Erik K. Henricson9Jonathan G. Dayan10Carsten Schmalfuss11Lee Sweeney12Barry J. Byrne13Craig M. McDonald14Krista Vandenborne15Glenn A. Walter16Department of Physical Therapy, University of FloridaDepartment of Physical Therapy, University of FloridaDepartment of Physical Therapy, University of FloridaDepartment of Physical Therapy, University of FloridaDepartment of Physical Therapy, University of FloridaDepartment of Statistics, University of FloridaDepartment of Statistics, University of FloridaDepartment of Physiology and Functional Genomics, University of FloridaDepartment of Physical Therapy, University of FloridaDepartment of Physical Medicine and Rehabilitation, University of California, DavisDepartment of Pediatrics, UC DavisDepartment of Medicine, Cardiology, University of FloridaDepartment of Pharmacology and Therapeutics, University of FloridaDepartment of Pediatrics, University of FloridaDepartment of Physical Medicine and Rehabilitation, University of California, DavisDepartment of Physical Therapy, University of FloridaDepartment of Physiology and Functional Genomics, University of FloridaAbstract Background The lack of dystrophin in cardiomyocytes in Duchenne muscular dystrophy (DMD) is associated with progressive decline in cardiac function eventually leading to death by 20–40 years of age. The aim of this prospective study was to determine rate of progressive decline in left ventricular (LV) function in Duchenne muscular dystrophy (DMD) over 5 years. Methods Short axis cine and grid tagged images of the LV were acquired in individuals with DMD (n = 59; age = 5.3–18.0 years) yearly, and healthy controls at baseline (n = 16, age = 6.0–18.3 years) on a 3 T MRI scanner. Grid-tagged images were analyzed for composite circumferential strain (ℇcc%) and ℇcc% in six mid LV segments. Cine images were analyzed for left ventricular ejection fraction (LVEF), LV mass (LVM), end-diastolic volume (EDV), end-systolic volume (ESV), LV atrioventricular plane displacement (LVAPD), and circumferential uniformity ratio estimate (CURE). LVM, EDV, and ESV were normalized to body surface area for a normalized index of LVM (LVMI), EDV (EDVI) and ESV (ESVI). Results At baseline, LV ℇcc% was significantly worse in DMD compared to controls and five of the six mid LV segments demonstrated abnormal strain in DMD. Longitudinal measurements revealed that ℇcc% consistently declined in individuals with DMD with the inferior segments being more affected. LVEF progressively declined between 3 to 5 years post baseline visit. In a multivariate analysis, the use of cardioprotective drugs trended towards positively impacting cardiac measures while loss of ambulation and baseline age were associated with negative impact. Eight out of 17 cardiac parameters reached a minimal clinically important difference with a threshold of 1/3 standard deviation. Conclusion The study shows a worsening of circumferential strain in dystrophic myocardium. The findings emphasize the significance of early and longitudinal assessment of cardiac function in DMD and identify early biomarkers of cardiac dysfunction to help design clinical trials to mitigate cardiac pathology. This study provides valuable non-invasive and non-contrast based natural history data of cardiac changes which can be used to design clinical trials or interpret the results of current trials aimed at mitigating the effects of decreased cardiac function in DMD.https://doi.org/10.1186/s12872-022-02688-5Cardiac magnetic resonance imagingDuchenne muscular dystrophyCardiac circumferential strain |
| spellingShingle | Abhinandan Batra Alison M. Barnard Donovan J. Lott Rebecca J. Willcocks Sean C. Forbes Saptarshi Chakraborty Michael J. Daniels Jannik Arbogast William Triplett Erik K. Henricson Jonathan G. Dayan Carsten Schmalfuss Lee Sweeney Barry J. Byrne Craig M. McDonald Krista Vandenborne Glenn A. Walter Longitudinal changes in cardiac function in Duchenne muscular dystrophy population as measured by magnetic resonance imaging BMC Cardiovascular Disorders Cardiac magnetic resonance imaging Duchenne muscular dystrophy Cardiac circumferential strain |
| title | Longitudinal changes in cardiac function in Duchenne muscular dystrophy population as measured by magnetic resonance imaging |
| title_full | Longitudinal changes in cardiac function in Duchenne muscular dystrophy population as measured by magnetic resonance imaging |
| title_fullStr | Longitudinal changes in cardiac function in Duchenne muscular dystrophy population as measured by magnetic resonance imaging |
| title_full_unstemmed | Longitudinal changes in cardiac function in Duchenne muscular dystrophy population as measured by magnetic resonance imaging |
| title_short | Longitudinal changes in cardiac function in Duchenne muscular dystrophy population as measured by magnetic resonance imaging |
| title_sort | longitudinal changes in cardiac function in duchenne muscular dystrophy population as measured by magnetic resonance imaging |
| topic | Cardiac magnetic resonance imaging Duchenne muscular dystrophy Cardiac circumferential strain |
| url | https://doi.org/10.1186/s12872-022-02688-5 |
| work_keys_str_mv | AT abhinandanbatra longitudinalchangesincardiacfunctioninduchennemusculardystrophypopulationasmeasuredbymagneticresonanceimaging AT alisonmbarnard longitudinalchangesincardiacfunctioninduchennemusculardystrophypopulationasmeasuredbymagneticresonanceimaging AT donovanjlott longitudinalchangesincardiacfunctioninduchennemusculardystrophypopulationasmeasuredbymagneticresonanceimaging AT rebeccajwillcocks longitudinalchangesincardiacfunctioninduchennemusculardystrophypopulationasmeasuredbymagneticresonanceimaging AT seancforbes longitudinalchangesincardiacfunctioninduchennemusculardystrophypopulationasmeasuredbymagneticresonanceimaging AT saptarshichakraborty longitudinalchangesincardiacfunctioninduchennemusculardystrophypopulationasmeasuredbymagneticresonanceimaging AT michaeljdaniels longitudinalchangesincardiacfunctioninduchennemusculardystrophypopulationasmeasuredbymagneticresonanceimaging AT jannikarbogast longitudinalchangesincardiacfunctioninduchennemusculardystrophypopulationasmeasuredbymagneticresonanceimaging AT williamtriplett longitudinalchangesincardiacfunctioninduchennemusculardystrophypopulationasmeasuredbymagneticresonanceimaging AT erikkhenricson longitudinalchangesincardiacfunctioninduchennemusculardystrophypopulationasmeasuredbymagneticresonanceimaging AT jonathangdayan longitudinalchangesincardiacfunctioninduchennemusculardystrophypopulationasmeasuredbymagneticresonanceimaging AT carstenschmalfuss longitudinalchangesincardiacfunctioninduchennemusculardystrophypopulationasmeasuredbymagneticresonanceimaging AT leesweeney longitudinalchangesincardiacfunctioninduchennemusculardystrophypopulationasmeasuredbymagneticresonanceimaging AT barryjbyrne longitudinalchangesincardiacfunctioninduchennemusculardystrophypopulationasmeasuredbymagneticresonanceimaging AT craigmmcdonald longitudinalchangesincardiacfunctioninduchennemusculardystrophypopulationasmeasuredbymagneticresonanceimaging AT kristavandenborne longitudinalchangesincardiacfunctioninduchennemusculardystrophypopulationasmeasuredbymagneticresonanceimaging AT glennawalter longitudinalchangesincardiacfunctioninduchennemusculardystrophypopulationasmeasuredbymagneticresonanceimaging |