Abstract
Objective
Effective heart failure therapy with angiotensin-converting enzyme inhibitors and \(\beta\)-blockers in a patient with mitochondrial disorder and asymptomatic previously stable hypertrophic cardiomyopathy and left ventricular noncompaction (NCCMP) has not been reported.
Case report
In a 58-year-old male with juvenile seizures, impaired hearing, recurrent pancreatitis, diabetes, recurrent emesis and diarrhea, discrete weakness for hip flexion, general wasting, and reduced tendon reflexes, elevated muscle-enzymes, abnormal lactate-stress-test, and mitochondrial dysfunction on muscle biopsy, mitochondrial disorder was diagnosed at age 51 year. Echocardiography revealed myocardial thickening and NCCMP. Cardiac abnormalities did not progress upon repeated follow-ups. At age 57 year he developed acute heart failure during respiratory infection. Echocardiography additionally revealed reduced left ventricular systolic function, and a restrictive filling pattern. Within seven weeks of therapy with ramipril (2.5 mg/day) and bisoprolol (1.25 mg/day) the restrictive filling pattern disappeared and fractional shortening normalized.
Conclusion
This case shows that heart failure in a patient with mitochondrial disorder and previously stable hypertrophic cardiomyopathy and NCCMP promptly resolves under therapy with angiotensin-converting enzyme inhibitors and \(\beta\)-blockers.
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An erratum to this article can be found at http://dx.doi.org/10.1007/s10554-006-9176-6
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Josef, F., Claudia, S. & Ellen, G. Successful heart failure therapy in mitochondrial disorder with noncompaction cardiomyopathy. Int J Cardiovasc Imaging 22, 393–398 (2006). https://doi.org/10.1007/s10554-005-9073-4
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DOI: https://doi.org/10.1007/s10554-005-9073-4