Rare cardiomyopathy in haemodialysis patients: noncompaction of the ventricular myocardium

Isolated noncompaction of the ventricular myocardium is a rare, unclassified cardiomyopathy characterized by the presence of numerous prominent trabeculations and deep intratrabecular recesses which communicate with the left ventricular cavity. We describe a case of noncompaction of the ventricular myocardium in a 67 years old, asymptomatic patient on regular hemodialysis. The male patient, on haemodilaysis for three and a half yrs (mesangioproliferative glomerulonephritis was a cause of end-stage renal disease), with no previous signs or symptoms of congestive heart failure or ventricular tachycardia or systemic embolisation, had a routine echocardiography assessment. We found enlarged left (60 mm) and right ventricle (32 mm) with small ejection fraction (EF 48%), but the left ventricle wall was thickened (septum 13 mm and posterior wall 13 mm) with many small lacunes and chanells with blood flow in it. Spongious muscle made more than two thirds of the ventricle wall of both chambers. No thrombus was seen. Diastolic dysfunction was also present (EA). The most frequent symptoms in these patients are: heart failure, ventricular tachycardia, sudden cardiac death, cardioembolic events and syncopa. Because of that, all patients need oral anticoagulant therapy, and regular Holter ECG--to identify patients with ventricular tachycardia for whom implantabile cardioverter--defbrilator is indicated. Although non-compaction left ventricle is a very type of cardiomyopathy, it is important to be aware of its presence and criteria for diagnosis (left ventricle wall thickened with many trabeculations and deep intertrabecular recessess with blood flow in it), and the need for adequate therapy and follow-up.