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PubMed This is a summary of 18 peer-reviewed journal articles Updated
Cardiology

Heart and Lung Health in Becker Muscular Dystrophy

At a Glance

In Becker Muscular Dystrophy, routine heart and lung monitoring is crucial because life-threatening issues, like dilated cardiomyopathy and sleep breathing problems, can occur before symptoms appear. Cardiac MRIs and sleep studies help detect these changes early so protective treatments can begin.

While the most visible sign of Becker Muscular Dystrophy (BMD) is skeletal muscle weakness, the most significant long-term risks often involve the heart and lungs [1]. Because these organs are also muscles that rely on dystrophin, they require specialized, lifelong monitoring—even if you feel physically strong and can walk long distances [2][3].

The Heart: High Stakes, Silent Changes

In BMD, the heart muscle can weaken over time, leading to dilated cardiomyopathy (DCM)—a condition where the heart becomes enlarged and cannot pump blood efficiently [3][1]. A key challenge in BMD is that heart issues do not always match muscle issues; some people have very mild walking trouble but significant heart involvement [2].

Strongly Recommended Cardiac Surveillance

Expert consensus recommends a proactive approach to heart health [4][5]:

  • Early Baseline: A baseline cardiac evaluation (including an EKG and imaging) is suggested in childhood, often before age 10, to establish a “normal” for your heart [6][7].
  • Routine Follow-up: Regular check-ups are essential, and monitoring is strongly recommended for everyone with BMD once they reach age 16 [4].
  • Looking Beyond Standard Echoes: A standard echocardiogram (ultrasound) measures how well the heart pumps, but it may miss early damage. Cardiologists now look for specific changes on your EKG (electrocardiogram), such as deep Q-waves or QRS fragmentation. While these sound highly technical, they simply mean the doctor is looking for subtle disruptions in the heart’s electrical signals that serve as early warning signs of muscle stress [4][8].

The Role of Cardiac MRI

A Cardiac MRI with Late Gadolinium Enhancement (LGE) is currently considered the best tool for BMD [9]. Unlike an ultrasound, this MRI can detect myocardial fibrosis (microscopic scarring) long before the heart’s pumping ability (ejection fraction) starts to drop [10][11]. Detecting this scarring early allows doctors to start protective treatments sooner [5].

Protective Medications

Evidence suggests that medications like ACE inhibitors (e.g., lisinopril) or ARBs (e.g., losartan) can help slow the progression of heart scarring in patients with muscular dystrophy [12]. Some specialists may recommend starting these even if your heart function currently appears normal, as a “prophylactic” or preventive measure to preserve the heart muscle for the future [13].

The Lungs: Monitoring “Silent” Breathing Issues

The diaphragm and other muscles used for breathing can also be affected by BMD. Respiratory decline is typically very slow in BMD, but it requires careful attention during certain milestones [14].

The Impact of Ambulation

While you are walking, your breathing muscles usually remain strong enough for daily life. However, the loss of independent walking is a major clinical milestone that often signals a faster decline in lung function [14][15]. Once ambulation is lost, respiratory monitoring should become more frequent [14].

The Risk of Sleep Hypercapnia

A unique risk in BMD is nocturnal hypercapnia—a buildup of carbon dioxide in the blood during sleep [16]. This can happen even if your daytime breathing tests (PFTs) look completely normal [16].

  • Warning Signs: Morning headaches, daytime sleepiness, or frequent waking at night can be signs that your breathing is shallow during sleep [16].
  • Testing: If you have symptoms but your standard tests are normal, your doctor may suggest a sleep study or a specialized test to measure your nocturnal PCO2 (carbon dioxide levels) [16].

Individualized care is vital. Because BMD is so variable, your heart and lung monitoring schedule should be tailored to your specific symptoms and genetic profile [17][18].

Common questions in this guide

Why do I need heart monitoring if my muscles feel strong with BMD?
In Becker Muscular Dystrophy, heart issues do not always match skeletal muscle weakness. The heart muscle can weaken and develop early scarring even if you can walk long distances and feel physically strong, which is why routine monitoring is strongly recommended.
What is the best test to check my heart health with Becker Muscular Dystrophy?
A Cardiac MRI with Late Gadolinium Enhancement (LGE) is considered the best tool. Unlike standard ultrasounds, this specialized MRI can detect microscopic scarring in the heart long before its pumping ability begins to drop.
What are the signs of breathing problems during sleep in BMD?
Warning signs of carbon dioxide buildup during sleep include morning headaches, daytime sleepiness, and frequent night waking. This can happen even if your standard daytime breathing tests appear completely normal.
Should I take heart medication if my heart function is currently normal?
Yes, some specialists recommend starting protective medications like ACE inhibitors or ARBs before heart function declines. This preventive approach is used to help slow the progression of heart scarring and preserve muscle function for the future.
How does losing the ability to walk affect my lung health?
The loss of independent walking is a major milestone that often signals a faster decline in respiratory function. Once you stop walking, your doctor will likely need to monitor your breathing and lung health much more frequently.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Has a cardiac MRI with Late Gadolinium Enhancement been performed to check for early scarring (fibrosis)?
  2. 2.My latest EKG mentioned Q-waves or QRS fragmentation — what do these mean for my long-term heart health?
  3. 3.Should we start an ACE inhibitor or ARB now to protect my heart muscle, even though my ejection fraction is currently normal?
  4. 4.Even though my daytime breathing tests are normal, can we perform a nocturnal CO2 check or sleep study to screen for silent hypercapnia?
  5. 5.How will our heart and lung monitoring schedule change if my walking speed or distance begins to decrease?

Questions For You

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References

References (18)
  1. 1

    Case report: A rare case of left ventricular noncompaction in two Chinese siblings with becker muscular dystrophy caused by deletion of exons 10 to 12 in the DMD gene.

    Li J, Zhu W, Su G, et al.

    Frontiers in cardiovascular medicine 2023; (10()):1243825 doi:10.3389/fcvm.2023.1243825.

    PMID: 37781315
  2. 2

    Heart Failure as the Initial Clinical Manifestation of Becker Muscular Dystrophy in an Adult.

    Del Rio-Pertuz G, Morataya C, Ratheal K, et al.

    Texas Heart Institute journal 2022; (49(6)) doi:10.14503/THIJ-21-7634.

    PMID: 36472918
  3. 3

    Dilated cardiomyopathy as the initial presentation of Becker muscular dystrophy: a systematic review of published cases.

    Del Rio-Pertuz G, Morataya C, Parmar K, et al.

    Orphanet journal of rare diseases 2022; (17(1)):194 doi:10.1186/s13023-022-02346-1.

    PMID: 35549971
  4. 4

    Electrocardiographic Changes in Jordanian Patients With Becker Muscular Dystrophy.

    Al-Raqad MK, Alwahsh S, Hejazi IS, et al.

    Cureus 2023; (15(10)):e47553 doi:10.7759/cureus.47553.

    PMID: 38022137
  5. 5

    Diagnosis and management of Becker muscular dystrophy: the French guidelines.

    Magot A, Wahbi K, Leturcq F, et al.

    Journal of neurology 2023; (270(10)):4763-4781 doi:10.1007/s00415-023-11837-5.

    PMID: 37422773
  6. 6

    Cardiac Involvement in Becker Muscular Dystrophy: Insights from Echocardiographic Analysis.

    You J, Kim MJ, Cha S, et al.

    Journal of child neurology 2025; (40(7)):510-518 doi:10.1177/08830738251327248.

    PMID: 40123366
  7. 7

    Heart transplantation in patients with dystrophinopathic cardiomyopathy: Review of the literature and personal series.

    Papa AA, D'Ambrosio P, Petillo R, et al.

    Intractable & rare diseases research 2017; (6(2)):95-101 doi:10.5582/irdr.2017.01024.

    PMID: 28580208
  8. 8

    Electrocardiographic prediction of late gadolinium enhancement on cardiac magnetic resonance in Becker muscular dystrophy.

    Bennett JS, Kamp AN, Cripe LH, Hor KN

    Neuromuscular disorders : NMD 2022; (32(1)):43-49 doi:10.1016/j.nmd.2021.09.011.

    PMID: 34955369
  9. 9

    Cardiac function and incidence of unexplained myocardial scarring in patients with primary carnitine deficiency - a cardiac magnetic resonance study.

    Kyhl K, Róin T, Lund A, et al.

    Scientific reports 2019; (9(1)):13909 doi:10.1038/s41598-019-50458-9.

    PMID: 31558765
  10. 10

    The Hypertrophic Cardiomyopathy Phenotype Viewed Through the Prism of Multimodality Imaging: Clinical and Etiologic Implications.

    Rowin EJ, Maron BJ, Maron MS

    JACC. Cardiovascular imaging 2020; (13(9)):2002-2016 doi:10.1016/j.jcmg.2019.09.020.

    PMID: 31864978
  11. 11

    Role of Cardiovascular Magnetic Resonance in Diagnosis and Management of Muscular Dystrophies.

    Russo V, Hudelo J, Marcel M, et al.

    Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance 2026; 102693 doi:10.1016/j.jocmr.2026.102693.

    PMID: 41548716
  12. 12

    Myocardial Fibrosis Progression in Duchenne and Becker Muscular Dystrophy: A Randomized Clinical Trial.

    Silva MC, Magalhães TA, Meira ZM, et al.

    JAMA cardiology 2017; (2(2)):190-199 doi:10.1001/jamacardio.2016.4801.

    PMID: 27926769
  13. 13

    Progressive left ventricular dysfunction and myocardial fibrosis in Duchenne and Becker muscular dystrophy: a longitudinal cardiovascular magnetic resonance study.

    Aikawa T, Takeda A, Oyama-Manabe N, et al.

    Pediatric cardiology 2019; (40(2)):384-392 doi:10.1007/s00246-018-2046-x.

    PMID: 30564867
  14. 14

    Respiratory decline in adult patients with Becker muscular dystrophy: A longitudinal study.

    De Wel B, Willaert S, Nadaj-Pakleza A, et al.

    Neuromuscular disorders : NMD 2021; (31(3)):174-182 doi:10.1016/j.nmd.2020.12.010.

    PMID: 33454189
  15. 15

    Analysis of motor and respiratory function in Duchenne muscular dystrophy patients.

    Luiz LC, Marson FAL, Bresciani Almeida CC, et al.

    Respiratory physiology & neurobiology 2019; (262()):1-11 doi:10.1016/j.resp.2019.01.009.

    PMID: 30660861
  16. 16

    Identification of sleep hypoventilation in young individuals with Becker muscular dystrophy: A pilot study.

    Nakamura Y, Saito Y, Kubota N, et al.

    Brain & development 2018; (40(7)):537-543 doi:10.1016/j.braindev.2018.02.012.

    PMID: 29526517
  17. 17

    Respiratory function in Becker muscular dystrophy: a comprehensive longitudinal study.

    Riguzzi P, Grover E, Schiava M, et al.

    Journal of neurology, neurosurgery, and psychiatry 2026; (97(5)):456-463 doi:10.1136/jnnp-2025-337953.

    PMID: 41760395
  18. 18

    Findings from the Longitudinal CINRG Becker Natural History Study.

    Clemens PR, Gordish-Dressman H, Niizawa G, et al.

    Journal of neuromuscular diseases 2024; (11(1)):201-212 doi:10.3233/JND-230178.

    PMID: 37980682

This page provides educational information on heart and lung monitoring for Becker Muscular Dystrophy. Always consult your cardiologist and pulmonologist for personalized medical advice and testing.

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