Markedly increased intracellular lipid droplets and disruption of intercellular junctions in biopsied myocardium from a patient with arrhythmogenic right ventricular cardiomyopathy

Heart and Vessels

Volumn 23, Issue 6, 2008, Pages 440-444

  Fujita, Shuichi ^a   Terasaki, Fumio ^a   Otsuka, Kaoru ^a   Katashima, Takashi ^a   Kanzaki, Yumiko ^a   Kawamura, Keishiro ^a   Tanaka, Takao ^a   Kitaura, Yasushi ^a  

^a OSAKA MEDICAL COLLEGE   (Japan)

Author keywords

Arrhythmogenic right ventricular cardiomyopathy; Endomyocardial biopsy; Lipid droplets; Ultrastructure

Indexed keywords

FAT DROPLET;

ADULT; ARTICLE; CASE REPORT; CELLULAR DISTRIBUTION; DIZZINESS; ELECTROCARDIOGRAM; ELECTRON MICROSCOPY; FEMALE; HEART MUSCLE BIOPSY; HEART RIGHT VENTRICLE DYSPLASIA; HEART VENTRICLE TACHYCARDIA; HISTOPATHOLOGY; HUMAN; HUMAN TISSUE; NUCLEAR MAGNETIC RESONANCE IMAGING; PRIORITY JOURNAL;

APOPTOSIS; ARRHYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA; BIOPSY; CELL MEMBRANE; DIAGNOSIS, DIFFERENTIAL; ELECTROCARDIOGRAPHY; FEMALE; HEART VENTRICLES; HUMANS; INTERCELLULAR JUNCTIONS; INTRACELLULAR SPACE; LIPIDS; MAGNETIC RESONANCE IMAGING; MICROSCOPY, ELECTRON; MIDDLE AGED; MYOCYTES, CARDIAC;

EID: 57049127139     PISSN: 09108327     EISSN: None     Source Type: Journal    
DOI: 10.1007/s00380-008-1079-0     Document Type: Article

References (10)

1. Mallat Z, Tedgui A, Fontaliran F, Frank R, Durigon M, Fontaine G (1996) Evidence of apoptosis in arrhythmogenic right ventricular dysplasia. N Engl J Med 335:1190-1196
2. James TN, Nichols MM, Sapire DW, DiPatre PL, Lopez SM (1996) Complete heart block and fatal right ventricular failure in an infant. Circulation 93:1588-1600
3. McKenna WJ, Thiene G, Nava A, Fontalian F, Lundqvist CB, Fontaine G, Camerini F (1994) Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy: Task Force of the Working Group Myocardial and Pericardial Disease of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the International Society and Federation of Cardiology. Br Heart J 71:215-218
4. Ghadially FN (1997) Lipid. In: Ultrastructural pathology of the cell and matrix, 4th edn. Butterworths-Heinemann, Boston, pp 1034-1037
5. Terasaki F, Kawamura K, Okabe M, Hayashi T, Deguchi H, Ando S, Usui M, Matsumoto T, Egashira K, Takeshita A (1997) Cardiomyopathy secondary to systemic triglyceride storage disease. Med Electron Microsc 30:88-91
6. Rampazzo A, Nava A, Malacrida S, Beffagna G, Bauce B, Rossi V, Zimbello R, Simionati B, Basso C, Thiene G, Towbin JA, Danieli GA (2002) Mutation in human desmoplakin domain binding to plakoglobin causes a dominant form of arrhythmogenic right ventricular cardiomyopathy. Am J Hum Genet 71:1200-1206
7. Gerull B, Heuser A, Wichter T, Paul M, Basson CT, McDermott DA, Lerman BB, Markowitz SM, Ellinor PT, MacRae CA, Peters S, Grossmann KS, Drenckhahn J, Michely B, Sasse-Klaassen S, Birchmeier W, Dietz R, Breithardt G, Schulze-Bahr E, Thierfelder L (2004) Mutations in the desmosomal protein plakophilin-2 are common in arrhythmogenic right ventricular cardiomyopathy. Nat Genet 36:1162-1164
8. Pilichou K, Nava A, Basso C, Beffagna G, Bauce B, Lorenzon A, Frigo G, Vettori A, Valente M, Towbin J, Thiene G, Danieli GA, Rampazzo A (2006) Mutations in Desmoglein-2 gene are associated to arrhythmogenic right ventricular cardiomyopathy. Circulation 113:1171-1179
9. Basso C, Czarnowska E, Della Barbera M, Bauce B, Beffagna G, Wlodarska EK, Pilichou K, Ramondo A, Lorenzon A, Wozniek O, Corrado D, Daliento L, Danieli GA, Valente M, Nava A, Thiene G, Rampazzo A (2006) Ultrastructural evidence of intercalated disc remodelling in arrhythmogenic right ventricular cardiomyopathy: An electron microscopy investigation on endomyocardial biopsies. Eur Heart J 27:1847-1854
10. Boriani G, Artale P, Biffi M, Martignani C, Frabetti L, Valzania C, Diemberger I, Ziacchi M, Bertini M, Rapezzi C, Parlapiano M, Branzi A (2007) Outcome of cardioverter-defibrillator implant in patients with arrhythmogenic right ventricular cardiomyopathy. Heart Vessels 22:184-192