NEKOMPAKTNA LIJEVA KLIJETKA

Ivo Božić, Zrinka Jurišić, Dorotea Božić, Vedran Carević, Tonći Batinić, Damir Fabijanić

Nekompaktna lijeva klijetka (NLK) rijetka je kardiomiopatija, koja se danas znatno češće otkriva u kliničkoj praksi zahvaljujući suvremenoj ultrazvučnoj dijagnostici. Bolest je uzrokovana zastojem normalne embrionalne pretvorbe endomiokarda iz mreže isprepletenih mišićnih vlakana u zreli kompaktni miokard. Morfološki se manifestira jedinstvenom dvoslojnom strukturom miokarda, koju čine unutarnji hipertrofični nekompaktni i subepikardijalni tanki kompaktni sloj, najčešće u inferiolateralnim i apikalnim segmentima lijeve klijetke. Unutarnji sloj tvori isprepletena masa mišićnih niti i trabekula s protočnim intertrabekularnim recesusima. Dijagnosticira se u infantilnom i adultnom obliku, koji se javljaju sporadično ili u obitelji, spolno vezano, odnosno autosomno dominantno. Prepoznavanje ove bolesti bitno je zbog visokog pobola i smrtnosti uzrokovanih progresivnim zatajivanjem srca, tromboembolijskim incidentima i malignim aritmijama. Ehokardiografija je metoda izbora u dijagnozi i praćenju bolesnika s NLK. U radu su sažeti najnoviji literaturni podatci o patogenezi, dijagnostičkim postupcima i liječenju ove bolesti.

Left ventricular non-compaction (LVNC) is a rare cardiomyopathy, which is today, due to modern ultrasound technology more frequently detected in clinical practice. It is caused by the failure of normal embryonic development of the myocardium from loosely arranged muscle fibers to the mature compacted form of myocardium. Morphologic presentation consists of unique two-layered structure, a thick noncompacted endocardial and a thin compact epicardial layer, in infero-lateral and apical segments. The endocardial layer contains loosely arranged muscle fibers, prominent trabeculations and deep perfused intertrabecular recesses. It could be diagnosed both as an infantile or adult type, appearing sporadically or among families where it is transferred x-linked or autosomal dominant. The recognition of the disease is mandatory because of its high mortality and morbidity due to the progressive heart failure, thromboembolic events and lethal arrhythmias. Echo­cardiography is the procedure of choice to confirm the diagnosis and in the follow-up of patients with LVNC. A literature review about LVNC pathogenesis, diagnostis, and treatment was discussed.