DIAGNOSTIC VALUE OF MYOCARDIAL BIOPSY IN THE DIAGNOSTIC ALGORITHM IN CARDIOMYOPATHIES IN CHILDREN

Autori:

IVAN MALČIĆ, DRAŽEN JELAŠIĆ, DALIBOR ŠARIĆ, HRVOJE KNIEWALD, MARIJA JELUŠIĆ, NATAŠA ROJNIĆ-PUTAREK, MIRA ŠĆUKANEC, LEO PAŽANIN, DANIEL DILBER

Sažetak
Cilj ovog rada jest pokazati važnost analize miokardijalnog bioptata primjenom različitih metoda u dijagramu dijagnostičkog toka primarnih kardiomiopatija u djece. Služeći se smjernicama radne skupine o kardiomiopatijama Svjetske zdravstvene organizacije i Međunarodnog društva i federacije o kardiomiopatijama (WHO/ISFC), utvrdili smo kardiomiopatiju u 121 djeteta (50 ‘ i 71 m), što je 38,81 dijete s kardiomiopatijom na 10 000 pregleda u pedijatrijskoj kardiološkoj ambulanti. Dilatacijska kardiomiopatija (DKM) nađena je u 52 bolesnika (42,9%), hipertrofična (HKM) u 43 bolesnika (35,5%), a restrikcijska (RKM) u 6 bolesnika (4,8%). Jedanaest bolesnika (9%) uvršteno je u skupinu specifičnih kardiomiopatija. U 9 bolesnika (7,4%) nije bilo moguće učiniti razvrstavanje. U većine bolesnika s DKM utvrđena je dijagnoza prije navršene treće godine života (RR 1,9, 95% CI 1,4–2,47). Nije bilo statistički značajne razlike u incidenciji DKM u usporedbi s HKM (Z 0,923, p<0,1779), ali je nađena značajno niža učestalost RKM (Z 6,004, p<0,001). Biopsija endokarda i miokarda učinjena je sa svrhom da se utvrdi etiološki čimbenik primarnih kardiomiopatija u 22 bolesnika (12 m i 10 ‘), u dobi od 1 do 17 godina (srednja dob 9,5 god.). Bioptati su analizirani svjetlosnom mikroskopijom (Dallaski kriteriji) u svih bolesnika, 13 bioptata analizirano je imunofluorescencijom, 8 imunohistokemijskim metodama (dva histokemijski uz bojenje kongoanskim crvenilom, jedan pod mikroskopom u polarizacijskom svjetlu), 7 elektronskom mikroskopijom, 5 PCR-metodom na DNA i RNA kardiotropne viruse. Od 10 pacijenata s DKM u četiri je nađena mionekroza kao posljedica akutnog miokarditisa, a u 6 kasni upalni proces, kao posljedica kroničnog imunosnog miokarditisa. U četvero rebiopsirane djece nađeno je potpuno izlječenje. U pet bolesnika s HKM dijagnoza je potvr|ena histološki. Jedan preparat analiziran je elektronskom mikroskopijom zbog sumnje na mitohondriopatiju. Od četiri bolesnika s RKM, kod kojih se sumnja na upalni proces, ovaj je potvrđen patohistološkim nalazom u tri, a u jednog je bolesnika analiza bioptata pokazala primarnu amiloidozu (infiltracijsko-restrikcijska KMP). U jednog je bolesnika patohistološki utvrđena fibroelastoza, a u jednog je nađen tumor srca (fibrom).
Summary

Summary. The purpose of this work was to show the importance of myocardial bioptate analysis using different methods in the diagram of diagnostic flow in primary cardiomyopathies in children. According to the guidelines of the Task Force on Car- diomyopathies of the WHO/ISFC, we identified 121 children (50 f and 71 m) as having cardiomyopathy, giving an average occurrence for all cardiomyopathies of 38.81 for each 10,000 pts examined in our outpatient clinics for paediatric cardiology. The dilated cardiomyopathy (DCM) was identified in 52 pts (42.9%), hypertrophic cardiomyopathy (HCM) in 43 pts (35.5%) and restrictive cardiomyopathy (RCM) in 6 pts (4.8%). We placed 11 pts (9.0%) in the group of specific cardiomyopathies. In nine pts (7.4%), it was impossible to classify the cardiomyopathy. Most of those with DCM had been diagnosed prior to the age of 3 years (RR 1.9, 95% CI 1.4–2.47). There were no statistically significant differences in the incidences of DCM as compared to HCM (Z 0.923, p<0.1779), but we encountered a significantly lower occurrence of RCM (Z 6.044, p<0,001). The biopsy of endocardium and myocardium was done to confirm the etiology of primary cardiomyopathy in 22 pts, 12 m and 10 f, age 1 to 17 (average age 9.5y). The bioptates were analysed by light microscope (Dallas criteria) in all pts, 13 bioptates by direct immunofluorescence, 8 by immunohystochemical method (two hystochemically by the method of coloring with Kongo red, one by the microscopy in polarised light), 7 by electron microscope, and 5 by PCR method where DNA and RNA of car- diotrophic viruses was used. Out of 10 pts with DCM, in 4 myonecrosis as a consequence of acute myocarditis and in 6 signs of late inflammatory processes, as a consequence of chronic immunologic myocarditis, were found. In 4 of them rebiopsy proved complete healing. In 5 pts with HCM the diagnosis was confirmed hystologically. One bioptate was analysed by electron microscope to rule out mitochondriopathy. Out of 4 pts with RCM due to inflammation, in 3 pathohistological findings proved diagnosis and in one showed primary amyloidosis. In one patient pathohystological finding showed fibroelastosis. In one patient heart tumor (fibroma) has been found.

Volumen: 9-10, 2004

Liječ Vjesn 2004;126:227–234

Dalibor Šarić, Daniel Dilber, Dražen Jelašić, Hrvoje Kniewald, Ivan Malčić, LEO PAŽANIN, MIRA ŠĆUKANEC, Marija Jelušić, Nataša Rojnić Putarek

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