Successful heart failure therapy in mitochondrial disorder with noncompaction cardiomyopathy

The International Journal of Cardiovascular Imaging
Josef FinstererGelpi Ellen

Abstract

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. 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. This case shows that heart failure in a patient with mitochondrial disorder and previously stable hypertrophic cardiomyopathy a...Continue Reading

References

Dec 9, 2000·Cardiology·J Finsterer, C Stöllberger
Oct 16, 2001·The International Journal of Cardiovascular Imaging·Y HamamichiT Kamiya
Jan 9, 2004·Journal of the American Society of Echocardiography : Official Publication of the American Society of Echocardiography·Claudia Stöllberger, Josef Finsterer
May 1, 2004·Circulation·Josef FinstererGerhard Blazek
Jun 9, 2004·Pediatric Cardiology·D LevT Lerman-Sagie
Sep 11, 2004·Brain : a Journal of Neurology·Massimo Zeviani, Stefano Di Donato
Jan 26, 2005·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·J Finsterer, E Milvay
May 18, 2005·Clinical Nutrition : Official Journal of the European Society of Parenteral and Enteral Nutrition·Hemmi N Bhagavan, Raj K Chopra

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Citations

Dec 4, 2014·International Journal of Cardiology·Josef Finsterer, Sonam Kothari
Feb 24, 2007·American Journal of Medical Genetics. Part a·Josef Finsterer
Dec 21, 2005·International Journal of Cardiology·Josef FinstererGerhard Blazek

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