title: [Evaluation of cardiac function by various cardiac imaging techniques in mitochondrial cardiomyopathy: a case report].
abstract: A 39-year-old man with cardiomyopathy due to point mutation of mitochondrial DNA(3243) was admitted to our hospital because of exertional dyspnea accompanied by hearing disturbance and diabetes mellitus. Echocardiography revealed asymmetric hypertrophy of the anterolateral and posterior walls and systolic dysfunction of the left ventricle (fractional shortening = 18%). Pulsed Doppler mitral inflow velocity wave showed a pseudonormalized pattern. Iodine-123 betamethyl-p-iodophenyl-pentadecanoic acid (123I-BMIPP) myocardial scintigraphy showed decreased accumulation in the anterolateral, posterior, and apical walls. Left ventriculography showed moderately decreased ejection fraction (43%), and left ventricular end-diastolic pressure was mildly elevated (18 mmHg). Angiography showed normal coronary arteries, but coronary flow reserve measured by administering intravenous adenosine triphosphate was impaired in the left anterior descending and left circumflex arteries compared to the right coronary artery. Intracellular accumulations of abnormal mitochondria were detected by histologic examination of the cardiac and skeletal muscles. Evaluation of cardiac function showed that the area of myocardial hypertrophy was nearly consistent with the region of decrease in 123I-BMIPP accumulation and coronary flow reserve.
authors.affiliation: Division of Cardiology, Zentsuji National Hospital, Kagawa.
authors.forename: H
authors.initials: H
authors.lastname: Shinomiya
authors.forename: N
authors.initials: N
authors.lastname: Fukuda
authors.forename: N
authors.initials: N
authors.lastname: Takeichi
authors.forename: T
authors.initials: T
authors.lastname: Soeki
authors.forename: H
authors.initials: H
authors.lastname: Shinohara
authors.forename: Y
authors.initials: Y
authors.lastname: Yui
authors.forename: Y
authors.initials: Y
authors.lastname: Tamura
authors.forename: Y
authors.initials: Y
authors.lastname: Fukada
authors.forename: M
authors.initials: M
authors.lastname: Nakamura
authors.forename: K
authors.initials: K
authors.lastname: Miyatake
authors.forename: C
authors.initials: C
authors.lastname: Yutani
mesh_terms: D000328:Adult; D003326:Coronary Circulation; D004272:DNA, Mitochondrial / Q000235:genetics; D004452:Echocardiography / Q000379:methods*; D005227:Fatty Acids; D006321:Heart / Q000000981:diagnostic imaging / Q000503:physiopathology*; D006801:Humans; D007457:Iodine Radioisotopes; D007462:Iodobenzenes; D017241:MELAS Syndrome / Q000000981:diagnostic imaging* / Q000503:physiopathology; D008297:Male; D017240:Mitochondrial Myopathies / Q000000981:diagnostic imaging* / Q000235:genetics / Q000503:physiopathology; D017354:Point Mutation; D011877:Radionuclide Imaging
chemical_list: D004272:DNA, Mitochondrial; D005227:Fatty Acids; D007457:Iodine Radioisotopes; D007462:Iodobenzenes; C049338:iodofiltic acid