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Panminerva Medica 1999 March;41(1)

lingua: Inglese

Pathohistological chang­es in endo­myo­car­dial biop­sy spec­i­mens in ­patients with myo­ton­ic dys­tro­phy

Rakocevic-Stoj­a­no­vic V. 1, Pav­lo­vic S. 1, Sef­e­ro­vic P. 2, Vasil­je­vic J. 3, Lavrnic D. 1, Marin­ko­vic Z. 1 and Apos­tol­ski S.1

From the 1 Institute for Neurology and 2 Institute for Cardiology Clinical Centre of Serbia
3 Institute for Pathology, Medical Faculty, Belgrade, Yugoslavia


Background. Endomyocardial bio­psies in ­patients ­with myo­ton­ic dys­tro­phy (MD) ­have, so far, ­shown chang­es ­such as myo­fi­bril­lar degen­er­a­tion, mit­o­chon­dri­al abnor­mal­ities, ­focal myo­car­ditis, fibro­sis and fat­ty infil­tra­tion of the myo­car­di­um and the con­duc­tion ­system.
Methods. This ­study ­presents the ­results of endo­myo­car­dial biop­sy in 10 ­patients ­with MD. Endomyocardial biop­sy was car­ried out ­using King’s biop­tome.
Results. In two ­patients ­with ­severe MD biop­sy spec­i­mens ­showed chang­es com­pat­ible ­with bor­der ­line myo­car­ditis. In ­five ­patients ­with mod­er­ate to ­severe ­forms of MD fibro­sis and fat­ty infil­tra­tion of the myo­car­di­um ­were ­found in addi­tion to degen­er­a­tive chang­es and hyper­tro­phy of mus­cle ­fibers. Three ­patients ­with ­mild MD had non-spe­cif­ic degen­er­a­tive and hyper­troph­ic myo­car­dial chang­es. The his­to­log­i­cal chang­es ­described ­above ­were ­present in ­patients with­out car­di­o­log­i­cal symp­toms and in ­those ­with nor­mal ECG and echoc­ar­di­o­graph­ic find­ings. Only two of the 10 ­patients in ­whom endo­myo­car­dial biop­sy was ­done com­plained of ­fatigue and occa­sion­al pal­pi­ta­tions ­while the ­rest ­were asymp­to­mat­ic. One ­patient ­with ­focal myo­car­ditis had ECG ­signs of ­left bun­dle ­branch ­block and echoc­ar­di­o­graph­ic evi­dence of ­reduced ­left ven­tric­u­lar con­trac­til­ity. Five ­patients ­with ­signs of endo­myo­car­dial fibro­sis ­only had an abnor­mal Q ­wave on ECG record­ings. The remain­ing ­five ­patients ­with bor­der ­line myo­car­ditis i.e. ­with degen­er­a­tive and hyper­troph­ic myo­car­dial chang­es had nor­mal ECG and echoc­ar­di­o­graph­ic find­ings.
Conclusions. These ­results ­stress the sig­nif­i­cance of endo­myo­car­dial biop­sy in detect­ing myo­car­dial path­o­log­ic chang­es in ­patients ­with MD.

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