Description

A patient with a mitochondrial disorder may have cardiac involvement. A defect in oxidative phosphorylation will affect the ability of myocytes to meet energy needs of the heart.


 

Cardiac findings may include:

(1) heart failure

(2) cardiomyopathy (hypertrophic, dilated, peripartum, other)

(3) left ventricular hypertrophy

(4) abnormal heart rate (bradycardia, high-grade heart block)

(5) cardiac arrhythmia (ventricular pre-excitation, Wolff-Parkinson-White, tachyarrhythmias)

(6) minor ECG changes

(7) sudden death

 

Reasons to consider mitochondrial cardiomyopathy:

(1) unexplained heart failure

(2) lactic acidosis

(3) worsening on exposure to a drug affecting respiratory chain function (valproic acid, statin, tetracycline, erythromycin, etc)

(4) inheritance from mother

(5) extra-cardiac manifestations of mitochondrial disorder

 


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