isolated left ventricular noncompaction ( ilvnc ) is a relatively newly defined disorder of the endomyocardium characterized by prominent ventricular trabeculations and deep intertrabecular recesses . clinical manifestations include a depressed left ventricular function , ventricular arrhythmia , and systemic embolization [ 2 , 3 ] . ilvnc has been reported to be associated with several electrocardiographic changes such as st depression and flat or negative t waves , bundle branch block , and wolff - parkinson - white ( wpw ) syndrome [ 24 ] . to the best of our knowledge , we described a case of ilvnc with basal ecg tracing strongly suggestive for type-2 brs . we reported the case of a 59-year - old man with basal ecg tracing strongly suggestive for type-2 brs figure 1(c ) . his heart rate was 70  beats / min , blood pressure was 130/80  mmhg , and body temperature was 36.1c . patient underwent a transthoracic echocardiogram ( tte ) that excluded arrhythmogenic right ventricular dysplasia , septal ischemia , ventricular aneurysm , and fast repolarization and showed noncompaction areas in both ventricles . this exam revealed the existence of two layers of myocardium  a thin , compacted , epicardial layer and a thick , noncompacted endocardial zone . the inner layer consisted of multiple myocardial trabeculations and deep intratrabecular recesses communicating with the left ventricular cavity figures 1(a ) and 1(b ) . the ratio of systolic thickness of noncompacted to compacted myocardium layers was above 2.0 ( n / c > 2.0 ) . in the colour - doppler the deep intertrabecular recesses the holter ecg did not reveal any ipercinetic arrhythmias but confirmed st - segment elevation in the right precordial leads ( v1 and v2 ) . treadmill stress test according to the bruce protocol was normally sustained for bp and ecg ischemic findings . during the recovery phase the saddleback - type st - segment elevation in v1 and v2 changed into a coved - type ecg . the ventricular tachycardia elicited by the flecainide test was hemodynamically stable and normalized without medication . based on the dynamic ecg changes ( conversion from saddleback- to coved - type ecg ) and exercise - induced ventricular tachycardia showing a left bundle branch block pattern , a diagnosis of asymptomatic brugada syndrome was made . ilvnc is a rare congenital cardiomyopathy characterized by excessively prominent trabecular meshwork and deep intratrabecular recesses within the left ventricle , sometimes also affecting the right ventricle and interventricular septum [ 4 , 5 ] . the predominant clinical features are heart failure , arrhythmias , embolic events , and sudden cardiac death ( scd ) . by definition , ilvnc occurs in the absence of other structural heart diseases . the gold standard for the ilvnc diagnosis is the echocardiographic examination . because of an increasing awareness and interest in this disorder , and advances in echocardiographic imaging , reported cases although the etiology of ilvnc has not been fully investigated , this disorder is thought to result from an arrest in endomyocardial morphogenesis during the embryonic period . during early embryonic life , the developing myocardium gradually condenses , and the large spaces within the trabecular meshwork flatten or disappear . the ecg of the previously reported patients with ilvnc showed st depression and flat or negative t waves , bundle branch block , wpw syndrome , and various patterns of arrhythmia [ 24 ] . a basal ecg tracing strongly suggestive for type-2 brs accompanied by ilvnc has not yet been reported . brugada syndrome ( brs ; omim 601144 ) is an autosomal dominant arrhythmogenic pathology characterized by syncope and scd . idiopathic ventricular fibrillation ,  is actually understood as a channelopathy probably consecutive to mutations on scn5a gene ( 3p21 - 23 ) encoding the cardiac sodium channel 's -subunit . brs manifests with syncope and cardiac arrest , typically occurring in the third and fourth decade of life , and usually at rest or during sleep . this eventuality called for an aggressive therapeutic strategy in all patients with brs and , since no pharmacological treatment of proven efficacy still is available , it led to implantable cardioverter defibrillator ( icd ) implants . according to a recent consensus conference type-1 ecg pattern with st - segment elevation in more than one right precordial lead , followed by a negative t wave plus one of the following conditions documented ventricular fibrillation ; self - terminating polymorphic ventricular tachycardia ; a family history of scd ( < 45 years ) ; coved - type ecgs in family members ; electrophysiological inducibility ; syncope or nocturnal agonal respiration . appearance of type-2 ecg pattern with st - segment elevation in more than one right precordial lead upon challenge with a sodium channel blockers ( flecainide or ajmaline ) , followed by a positive or biphasic t wave that results in a saddleback configuration . appearance of type-3 ecg pattern characterized by st - segment elevation ( 1  mm ) in more than one lead either with a coved - type or a saddleback morphology , with conversion to type 1 after challenge with a sodium channel blocker . in their initial report on eight patients , brugada et al . emphasized the lack of structural cardiac abnormalities . it is reported in the literature the association between the typical v1v3 segment elevation and right bundle branch block in some patients with arrhythmogenic right ventricular cardiomyopathy ( arvc ; omim 107970 ) [ 10 , 11 ] . molecular genetic studies identified five allelic variants cosegregating with dilated cardiomyopathy ( dcm ) [ 12 , 13 ] . some authors reported structural degeneration ( fibrosis ) and apoptosis during the analysis of myocardial biopsies of patients with clinical diagnosis of brugada syndrome and an scn5a mutation . taken as a whole , these studies suggest that at least some scn5a mutations manifest as both excitability and structural derangement .   in the presented case , there was no conclusive proof of the aetiology and the coexistence of the brugada - type electrocardiographic pattern and ilvnc might be coincidental . however , the case is consistent with the consideration that up to now none of the autopsies in brugada patients turned out to present a normal heart .   on a clinical basis , the possible coexistence of the brugada syndrome with structural myocardial alterations should always be considered and it advocates the need of careful echo and nmr studies on all brs patients .
isolated left ventricular noncompaction ( ilvnc ) is a cardiomyopathy caused by intrauterine arrest of compaction of the myocardial fibres and meshwork , an important process in myocardial development . 
 ilvnc is clinically accompanied by depressed ventricular function , arrhythmias , and systemic embolization . 
 we reported a case of ilvnc with basal ecg - tracing strongly suggestive for type-2 brugada syndrome ( brs ) . 
 up to now , this is the first report investigating the association between ilvnc and this particular ecg pattern .