Volume 54, Issue 5 pp. 1503-1513

Research Article

Right Ventricular Function and T1-Mapping in Boys With Duchenne Muscular Dystrophy

Seraina A. Dual PhD,

Corresponding Author

Seraina A. Dual PhD

[email protected]

orcid.org/0000-0001-6867-8270

Department of Radiology, Stanford University, Palo Alto, California, USA

Department of Cardiothoracic Surgery, Stanford University, Palo Alto, California, USA

Cardiovascular Institute, Stanford University, Palo Alto, California, USA

Address reprint requests to: S.A.D., 780 Welch Road, Palo Alto, CA 94304, USA. E-mail: [email protected]

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Nyasha G. Maforo,

Nyasha G. Maforo

orcid.org/0000-0002-5741-7021

Physics and Biology in Medicine Interdepartmental Program, University of California, Los Angeles, California, USA

Department of Radiological Sciences, University of California, Los Angeles, California, USA

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Doff B. McElhinney MD,

Doff B. McElhinney MD

orcid.org/0000-0001-7242-0934

Department of Cardiothoracic Surgery, Stanford University, Palo Alto, California, USA

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Ashley Prosper MD,

Ashley Prosper MD

orcid.org/0000-0001-8615-5246

Department of Radiological Sciences, University of California, Los Angeles, California, USA

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Holden H. Wu PhD,

Holden H. Wu PhD

orcid.org/0000-0002-2585-5916

Physics and Biology in Medicine Interdepartmental Program, University of California, Los Angeles, California, USA

Department of Radiological Sciences, University of California, Los Angeles, California, USA

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Shiraz Maskatia MD,

Shiraz Maskatia MD

orcid.org/0000-0002-8806-1398

Department of Pediatrics, Stanford University, Palo Alto, California, USA

Maternal & Child Health Research Institute, Stanford University, Palo Alto, California, USA

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Pierangelo Renella MD,

Pierangelo Renella MD

orcid.org/0000-0002-6444-2604

Department of Radiological Sciences, University of California, Los Angeles, California, USA

Children's hospital Orange County, University of California, Irvine, California, USA

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Nancy Halnon MD,

Nancy Halnon MD

orcid.org/0000-0002-8370-7831

Department of Medicine (Cardiology), University of California, Los Angeles, California, USA

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Daniel B. Ennis PhD,

Daniel B. Ennis PhD

orcid.org/0000-0001-7435-1311

Department of Radiology, Stanford University, Palo Alto, California, USA

Cardiovascular Institute, Stanford University, Palo Alto, California, USA

Maternal & Child Health Research Institute, Stanford University, Palo Alto, California, USA

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Seraina A. Dual PhD,

Corresponding Author

Seraina A. Dual PhD

[email protected]

orcid.org/0000-0001-6867-8270

Department of Radiology, Stanford University, Palo Alto, California, USA

Department of Cardiothoracic Surgery, Stanford University, Palo Alto, California, USA

Cardiovascular Institute, Stanford University, Palo Alto, California, USA

Address reprint requests to: S.A.D., 780 Welch Road, Palo Alto, CA 94304, USA. E-mail: [email protected]

Search for more papers by this author

Nyasha G. Maforo,

Nyasha G. Maforo

orcid.org/0000-0002-5741-7021

Physics and Biology in Medicine Interdepartmental Program, University of California, Los Angeles, California, USA

Department of Radiological Sciences, University of California, Los Angeles, California, USA

Search for more papers by this author

Doff B. McElhinney MD,

Doff B. McElhinney MD

orcid.org/0000-0001-7242-0934

Department of Cardiothoracic Surgery, Stanford University, Palo Alto, California, USA

Search for more papers by this author

Ashley Prosper MD,

Ashley Prosper MD

orcid.org/0000-0001-8615-5246

Department of Radiological Sciences, University of California, Los Angeles, California, USA

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Holden H. Wu PhD,

Holden H. Wu PhD

orcid.org/0000-0002-2585-5916

Physics and Biology in Medicine Interdepartmental Program, University of California, Los Angeles, California, USA

Department of Radiological Sciences, University of California, Los Angeles, California, USA

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Shiraz Maskatia MD,

Shiraz Maskatia MD

orcid.org/0000-0002-8806-1398

Department of Pediatrics, Stanford University, Palo Alto, California, USA

Maternal & Child Health Research Institute, Stanford University, Palo Alto, California, USA

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Pierangelo Renella MD,

Pierangelo Renella MD

orcid.org/0000-0002-6444-2604

Department of Radiological Sciences, University of California, Los Angeles, California, USA

Children's hospital Orange County, University of California, Irvine, California, USA

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Nancy Halnon MD,

Nancy Halnon MD

orcid.org/0000-0002-8370-7831

Department of Medicine (Cardiology), University of California, Los Angeles, California, USA

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Daniel B. Ennis PhD,

Daniel B. Ennis PhD

orcid.org/0000-0001-7435-1311

Department of Radiology, Stanford University, Palo Alto, California, USA

Cardiovascular Institute, Stanford University, Palo Alto, California, USA

Maternal & Child Health Research Institute, Stanford University, Palo Alto, California, USA

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First published: 26 May 2021

https://doi.org/10.1002/jmri.27729

Citations: 3

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Abstract

Background

Clinical management of boys with Duchenne muscular dystrophy (DMD) relies on in-depth understanding of cardiac involvement, but right ventricular (RV) structural and functional remodeling remains understudied.

Purpose

To evaluate several analysis methods and identify the most reliable one to measure RV pre- and postcontrast T1 (RV-T1) and to characterize myocardial remodeling in the RV of boys with DMD.

Study Type

Prospective.

Population

Boys with DMD (N = 27) and age-/sex-matched healthy controls (N = 17) from two sites.

Field Strength/Sequence

3.0 T using balanced steady state free precession, motion-corrected phase sensitive inversion recovery and modified Look-Locker inversion recovery sequences.

Assessment

Biventricular mass (Mi), end-diastolic volume (EDVi) and ejection fraction (EF) assessment, tricuspid annular excursion (TAE), late gadolinium enhancement (LGE), pre- and postcontrast myocardial T1 maps. The RV-T1 reliability was assessed by three observers in four different RV regions of interest (ROI) using intraclass correlation (ICC).

Statistical Tests

The Wilcoxon rank sum test was used to compare RV-T1 differences between DMD boys with negative LGE(−) or positive LGE(+) and healthy controls. Additionally, correlation of precontrast RV-T1 with functional measures was performed. A P-value <0.05 was considered statistically significant.

Results

A 1-pixel thick RV circumferential ROI proved most reliable (ICC > 0.91) for assessing RV-T1. Precontrast RV-T1 was significantly higher in boys with DMD compared to controls. Both LGE(−) and LGE(+) boys had significantly elevated precontrast RV-T1 compared to controls (1543 [1489–1597] msec and 1550 [1402–1699] msec vs. 1436 [1399–1473] msec, respectively). Compared to healthy controls, boys with DMD had preserved RVEF (51.8 [9.9]% vs. 54.2 [7.2]%, P = 0.31) and significantly reduced RVMi (29.8 [9.7] g vs. 48.0 [15.7] g), RVEDVi (69.8 [29.7] mL/m² vs. 89.1 [21.9] mL/m²), and TAE (22.0 [3.2] cm vs. 26.0 [4.7] cm). Significant correlations were found between precontrast RV-T1 and RVEF (β = −0.48%/msec) and between LV-T1 and LVEF (β = −0.51%/msec).

Data Conclusion

Precontrast RV-T1 is elevated in boys with DMD compared to healthy controls and is negatively correlated with RVEF.

Level of Evidence

Technical Efficacy

Stage 2

Supporting Information

References

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Citing Literature

Volume54, Issue5

November 2021

Pages 1503-1513

Right Ventricular Function and T1-Mapping in Boys With Duchenne Muscular Dystrophy

Abstract

Background

Purpose

Study Type

Population

Field Strength/Sequence

Assessment

Statistical Tests

Results

Data Conclusion

Level of Evidence

Technical Efficacy

Supporting Information

References

Citing Literature

References

Information

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Right Ventricular Function and T1-Mapping in Boys With Duchenne Muscular Dystrophy

Abstract

Background

Purpose

Study Type

Population

Field Strength/Sequence

Assessment

Statistical Tests

Results

Data Conclusion

Level of Evidence

Technical Efficacy

Supporting Information

References

Citing Literature

References

Related

Information