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Cardiac Follow-up in Duchenne Muscular Dystrophy

Routine cardiac evaluation and early cardioprotective therapy for DMD patients.

Written by: Saygı Hospital Health Guide Editorial Board
Published:

This content is for general information; please consult your physician for diagnosis and treatment.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Kardiyoloji department. Book Appointment →

What is Cardiac Follow-up in Duchenne Muscular Dystrophy?

Duchenne muscular dystrophy (DMD) is the most common childhood muscular dystrophy, caused by X-linked mutations in the dystrophin gene. Nearly all affected boys develop cardiac involvement in addition to progressive muscle weakness.

Cardiac disease typically begins with late gadolinium enhancement (subepicardial inferolateral) on cardiac MRI, followed by dilated cardiomyopathy and heart failure. Arrhythmia risk is also increased.

Early detection with cardiac MRI and cardioprotective therapy with ACE inhibitors (perindopril), beta-blockers and spironolactone starting around age 10 are recommended.

Symptoms

Progressive muscle weakness
Gowers sign
Loss of ambulation
Respiratory failure
Signs of heart failure
Arrhythmias
Scoliosis

Risk Factors

X-linked inheritance
Male sex
Family history
Positive dystrophin mutation
Advanced-age DMD
Steroid-resistant disease
Respiratory support dependence

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Diagnosis of DMD
  • Routine cardiac screening at age 10
  • Annual echocardiography and MRI
  • Cardiac symptoms with respiratory failure
  • Detection of arrhythmia
  • LGE on cardiac MRI

Treatment Methods

01
Early ACE inhibitor (perindopril) therapy
02
Addition of beta-blocker
03
Spironolactone
04
Corticosteroids (deflazacort)
05
Exon-skipping therapies
06
Gene therapy research
07
Integrated respiratory support

Which Department to Visit?

You can visit our Kardiyoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.