Cardiac MRI in Duchenne and Becker Muscular Dystrophy
Background and Objectives: Cardiovascular magnetic resonance imaging (CMRI) is the noninvasive technique of choice for early detection of cardiac involvement in Duchenne and Becker muscular dystrophy (DMD and BMD, respectively), but is seldom used in routine clinical practice in the Indian context....
Saved in:
| Main Authors: | , , , , , , , , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wolters Kluwer Medknow Publications
2024-10-01
|
| Series: | Annals of Indian Academy of Neurology |
| Subjects: | |
| Online Access: | https://journals.lww.com/10.4103/aian.aian_988_23 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1850181039653126144 |
|---|---|
| author | Manu Santhappan Girija Deepak Menon Kiran Polavarapu Veeramani Preethish-Kumar Seena Vengalil Saraswati Nashi Madassu Keertipriya Mainak Bardhan Priya Treesa Thomas Valasani Ravi Kiran Vikas Nishadham Arun Sadasivan Akshata Huddar Gopi Krishnan Unnikrishnan Ashita Barthur Atchayaram Nalini |
| author_facet | Manu Santhappan Girija Deepak Menon Kiran Polavarapu Veeramani Preethish-Kumar Seena Vengalil Saraswati Nashi Madassu Keertipriya Mainak Bardhan Priya Treesa Thomas Valasani Ravi Kiran Vikas Nishadham Arun Sadasivan Akshata Huddar Gopi Krishnan Unnikrishnan Ashita Barthur Atchayaram Nalini |
| author_sort | Manu Santhappan Girija |
| collection | DOAJ |
| description | Background and Objectives:
Cardiovascular magnetic resonance imaging (CMRI) is the noninvasive technique of choice for early detection of cardiac involvement in Duchenne and Becker muscular dystrophy (DMD and BMD, respectively), but is seldom used in routine clinical practice in the Indian context. We sought to determine the prevalence of CMRI abnormalities in patients with DMD and BMD and to compare the CMRI parameters with the phenotypic and genotypic characteristics.
Methods:
A prospective, observational study was conducted on patients genetically diagnosed with DMD and BMD who could complete CMRI between March 2020 and March 2022. Abnormal CMRI was the presence of any late gadolinium enhancement (LGE) that signifies myocardial fibrosis (LGE positivity), regional wall motion abnormality, or reduced left ventricular ejection fraction (LVEF <55%).
Results:
A total of 46 patients were included: 38 patients with DMD and eight with BMD. Cardiac abnormality was seen in 23 (50%) patients. LGE was more common than impaired LVEF in DMD (16, 42.1%), while impaired LVEF was more common in BMD (5, 62.5%). LGE was most frequently found in lateral wall (18/19) followed by inferior (6/19), septal (5/19), anterior (2/19), and apex (1/19). Among the various clinicodemographic parameters, only age (r = 0.495, P = 0.002) and disease duration (r = 0.407, P = 0.011) were found to significantly correlate with LGE in patients with DMD. No association was found between the various CMRI parameters and the genotype.
Conclusions:
The current study highlights the differences in myocardial fibrosis and LV dysfunction between DMD and BMD, along with other CMRI parameters. Notably, a genotype–CMRI correlation was not found in the current cohort, which needs to be further explored. |
| format | Article |
| id | doaj-art-38c2098435744099ac7e752bf86f779b |
| institution | OA Journals |
| issn | 0972-2327 1998-3549 |
| language | English |
| publishDate | 2024-10-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Annals of Indian Academy of Neurology |
| spelling | doaj-art-38c2098435744099ac7e752bf86f779b2025-08-20T02:17:59ZengWolters Kluwer Medknow PublicationsAnnals of Indian Academy of Neurology0972-23271998-35492024-10-0127555255710.4103/aian.aian_988_23Cardiac MRI in Duchenne and Becker Muscular DystrophyManu Santhappan GirijaDeepak MenonKiran PolavarapuVeeramani Preethish-KumarSeena VengalilSaraswati NashiMadassu KeertipriyaMainak BardhanPriya Treesa ThomasValasani Ravi KiranVikas NishadhamArun SadasivanAkshata HuddarGopi Krishnan UnnikrishnanAshita BarthurAtchayaram NaliniBackground and Objectives: Cardiovascular magnetic resonance imaging (CMRI) is the noninvasive technique of choice for early detection of cardiac involvement in Duchenne and Becker muscular dystrophy (DMD and BMD, respectively), but is seldom used in routine clinical practice in the Indian context. We sought to determine the prevalence of CMRI abnormalities in patients with DMD and BMD and to compare the CMRI parameters with the phenotypic and genotypic characteristics. Methods: A prospective, observational study was conducted on patients genetically diagnosed with DMD and BMD who could complete CMRI between March 2020 and March 2022. Abnormal CMRI was the presence of any late gadolinium enhancement (LGE) that signifies myocardial fibrosis (LGE positivity), regional wall motion abnormality, or reduced left ventricular ejection fraction (LVEF <55%). Results: A total of 46 patients were included: 38 patients with DMD and eight with BMD. Cardiac abnormality was seen in 23 (50%) patients. LGE was more common than impaired LVEF in DMD (16, 42.1%), while impaired LVEF was more common in BMD (5, 62.5%). LGE was most frequently found in lateral wall (18/19) followed by inferior (6/19), septal (5/19), anterior (2/19), and apex (1/19). Among the various clinicodemographic parameters, only age (r = 0.495, P = 0.002) and disease duration (r = 0.407, P = 0.011) were found to significantly correlate with LGE in patients with DMD. No association was found between the various CMRI parameters and the genotype. Conclusions: The current study highlights the differences in myocardial fibrosis and LV dysfunction between DMD and BMD, along with other CMRI parameters. Notably, a genotype–CMRI correlation was not found in the current cohort, which needs to be further explored.https://journals.lww.com/10.4103/aian.aian_988_23dystrophinopathyduchenne muscular dystrophybecker muscular dystrophycardiomyopathycardiac mri |
| spellingShingle | Manu Santhappan Girija Deepak Menon Kiran Polavarapu Veeramani Preethish-Kumar Seena Vengalil Saraswati Nashi Madassu Keertipriya Mainak Bardhan Priya Treesa Thomas Valasani Ravi Kiran Vikas Nishadham Arun Sadasivan Akshata Huddar Gopi Krishnan Unnikrishnan Ashita Barthur Atchayaram Nalini Cardiac MRI in Duchenne and Becker Muscular Dystrophy Annals of Indian Academy of Neurology dystrophinopathy duchenne muscular dystrophy becker muscular dystrophy cardiomyopathy cardiac mri |
| title | Cardiac MRI in Duchenne and Becker Muscular Dystrophy |
| title_full | Cardiac MRI in Duchenne and Becker Muscular Dystrophy |
| title_fullStr | Cardiac MRI in Duchenne and Becker Muscular Dystrophy |
| title_full_unstemmed | Cardiac MRI in Duchenne and Becker Muscular Dystrophy |
| title_short | Cardiac MRI in Duchenne and Becker Muscular Dystrophy |
| title_sort | cardiac mri in duchenne and becker muscular dystrophy |
| topic | dystrophinopathy duchenne muscular dystrophy becker muscular dystrophy cardiomyopathy cardiac mri |
| url | https://journals.lww.com/10.4103/aian.aian_988_23 |
| work_keys_str_mv | AT manusanthappangirija cardiacmriinduchenneandbeckermusculardystrophy AT deepakmenon cardiacmriinduchenneandbeckermusculardystrophy AT kiranpolavarapu cardiacmriinduchenneandbeckermusculardystrophy AT veeramanipreethishkumar cardiacmriinduchenneandbeckermusculardystrophy AT seenavengalil cardiacmriinduchenneandbeckermusculardystrophy AT saraswatinashi cardiacmriinduchenneandbeckermusculardystrophy AT madassukeertipriya cardiacmriinduchenneandbeckermusculardystrophy AT mainakbardhan cardiacmriinduchenneandbeckermusculardystrophy AT priyatreesathomas cardiacmriinduchenneandbeckermusculardystrophy AT valasaniravikiran cardiacmriinduchenneandbeckermusculardystrophy AT vikasnishadham cardiacmriinduchenneandbeckermusculardystrophy AT arunsadasivan cardiacmriinduchenneandbeckermusculardystrophy AT akshatahuddar cardiacmriinduchenneandbeckermusculardystrophy AT gopikrishnanunnikrishnan cardiacmriinduchenneandbeckermusculardystrophy AT ashitabarthur cardiacmriinduchenneandbeckermusculardystrophy AT atchayaramnalini cardiacmriinduchenneandbeckermusculardystrophy |