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Review

Role of the Renin–Angiotensin–Aldosterone System in Dystrophin-Deficient Cardiomyopathy

1
Pediatric Cardiology Division of Puerta del Mar University Hospital, University of Cadiz, 11009 Cadiz, Spain
2
Biomedical Research and Innovation Institute of Cadiz (INiBICA), Research Unit, Puerta del Mar University Hospital, University of Cadiz, 11009 Cadiz, Spain
3
Pediatric Neurology Division of Puerta del Mar University Hospital, University of Cadiz, 11009 Cadiz, Spain
4
Pediatric Division of Doctor Cayetano Roldan Primary Care Center, 11100 San Fernando, Spain
5
Pediatric Division of Motril-San Antonio Primary Care Center, 18600 Motril, Spain
6
Pediatric Nephrology Division of Puerta del Mar University Hospital, University of Cadiz, 11009 Cadiz, Spain
*
Author to whom correspondence should be addressed.
Int. J. Mol. Sci. 2021, 22(1), 356; https://doi.org/10.3390/ijms22010356
Received: 16 December 2020 / Revised: 27 December 2020 / Accepted: 28 December 2020 / Published: 31 December 2020
Dystrophin-deficient cardiomyopathy (DDC) is currently the leading cause of death in patients with dystrophinopathies. Targeting myocardial fibrosis (MF) has become a major therapeutic goal in order to prevent the occurrence of DDC. We aimed to review and summarize the current evidence about the role of the renin–angiotensin–aldosterone system (RAAS) in the development and perpetuation of MF in DCC. We conducted a comprehensive search of peer-reviewed English literature on PubMed about this subject. We found increasing preclinical evidence from studies in animal models during the last 20 years pointing out a central role of RAAS in the development of MF in DDC. Local tissue RAAS acts directly mainly through its main fibrotic component angiotensin II (ANG2) and its transducer receptor (AT1R) and downstream TGF-b pathway. Additionally, it modulates the actions of most of the remaining pro-fibrotic factors involved in DDC. Despite limited clinical evidence, RAAS blockade constitutes the most studied, available and promising therapeutic strategy against MF and DDC. Conclusion: Based on the evidence reviewed, it would be recommendable to start RAAS blockade therapy through angiotensin converter enzyme inhibitors (ACEI) or AT1R blockers (ARBs) alone or in combination with mineralocorticoid receptor antagonists (MRa) at the youngest age after the diagnosis of dystrophinopathies, in order to delay the occurrence or slow the progression of MF, even before the detection of any cardiovascular alteration. View Full-Text
Keywords: dystrohinopathy; duchenne muscular disease; becker muscular disease; dystrophic deficient cardiomyopathy; cardiac fibrosis; renin angiotensin system; angiotensin 2; angiotensin converter enzyme inhibitors; angiotensin receptor blockers dystrohinopathy; duchenne muscular disease; becker muscular disease; dystrophic deficient cardiomyopathy; cardiac fibrosis; renin angiotensin system; angiotensin 2; angiotensin converter enzyme inhibitors; angiotensin receptor blockers
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MDPI and ACS Style

Rodriguez-Gonzalez, M.; Lubian-Gutierrez, M.; Cascales-Poyatos, H.M.; Perez-Reviriego, A.A.; Castellano-Martinez, A. Role of the Renin–Angiotensin–Aldosterone System in Dystrophin-Deficient Cardiomyopathy. Int. J. Mol. Sci. 2021, 22, 356. https://doi.org/10.3390/ijms22010356

AMA Style

Rodriguez-Gonzalez M, Lubian-Gutierrez M, Cascales-Poyatos HM, Perez-Reviriego AA, Castellano-Martinez A. Role of the Renin–Angiotensin–Aldosterone System in Dystrophin-Deficient Cardiomyopathy. International Journal of Molecular Sciences. 2021; 22(1):356. https://doi.org/10.3390/ijms22010356

Chicago/Turabian Style

Rodriguez-Gonzalez, Moises, Manuel Lubian-Gutierrez, Helena M. Cascales-Poyatos, Alvaro A. Perez-Reviriego, and Ana Castellano-Martinez. 2021. "Role of the Renin–Angiotensin–Aldosterone System in Dystrophin-Deficient Cardiomyopathy" International Journal of Molecular Sciences 22, no. 1: 356. https://doi.org/10.3390/ijms22010356

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