Successful heart failure therapy in mitochondrial disorder with noncompaction cardiomyopathy.
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.


METHODS
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.


CONCLUSIONS
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.
Finsterer
Josef
J
Neurological Department, Neurological Hospital Rosenhügel, Vienna, Austria. duarte@aonmail.at
Josef
Finsterer
F
Stöllberger
Claudia
C
Claudia
Stöllberger
S
Gelpi
Ellen
E
Ellen
Gelpi
G
D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use*; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use*; D017298:Bisoprolol / Q000627:therapeutic use; D002303:Cardiac Output, Low / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000209:etiology; D002312:Cardiomyopathy, Hypertrophic / Q000150:complications / Q000000981:diagnostic imaging / Q000188:drug therapy*; D006352:Heart Ventricles / Q000000981:diagnostic imaging; D006801:Humans; D008297:Male; D008875:Middle Aged; D028361:Mitochondrial Diseases / Q000150:complications / Q000000981:diagnostic imaging / Q000188:drug therapy*; D017257:Ramipril / Q000627:therapeutic use; D016896:Treatment Outcome; D014463:Ultrasonography
D000319:Adrenergic beta-Antagonists; D000806:Angiotensin-Converting Enzyme Inhibitors; D017257:Ramipril; D017298:Bisoprolol