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Cardiac Involvement in MELAS Syndrome

Cardiac manifestations of mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes syndrome.

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 Involvement in MELAS Syndrome?

MELAS (Mitochondrial Encephalomyopathy, Lactic Acidosis, Stroke-like episodes) syndrome is most often associated with the m.3243A>G mutation in the mt-tRNA leucine (UUR) gene.

Cardiac involvement occurs in 30–40% of patients as left ventricular hypertrophy, conduction disease, dilated cardiomyopathy and arrhythmias. The risk of sudden cardiac death is high.

Treatment includes arginine/citrulline for acute stroke-like episodes, coenzyme Q10, L-carnitine, and standard management of heart failure and arrhythmia. A multidisciplinary approach is essential.

Symptoms

Stroke-like episodes
Migraine-like headache
Seizures
Heart failure
Conduction disease
Lactic acidosis
Encephalopathy

Risk Factors

m.3243A>G mutation
Maternal inheritance
Hearing loss
Diabetes mellitus
Short stature
Family history
Myopathy

When to See a Doctor?

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

  • Stroke-like episode
  • Suspected cardiomyopathy
  • Family history of MELAS
  • Sensorineural hearing loss
  • Unexplained lactic acidosis
  • Conduction disease

Treatment Methods

01
Arginine for acute attack
02
Daily coenzyme Q10
03
L-carnitine
04
Standard heart failure therapy
05
Pacemaker or ICD when indicated
06
Anticonvulsants
07
Multidisciplinary approach

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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You can make an appointment with our specialists or contact us for your concerns.

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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.