isolated left ventricular noncompaction ( lvnc ) is a congenital cardiomyopathy caused by a defect in endomyocardial morphogenesis.1 ) it is characterized by prominent trabeculation and deep intertrabecular recesses , resulting in thickened myocardium consisting of 2 layers - compacted and noncompacted myocardium.2 ) lvnc is occasionally combined with other congenital cardiac diseases3 - 6 ) and genetic cardiomyopathies.7 ) its clinical manifestations include heart failure , thromboembolism , ventricular tachyarrhythmia , and sudden death.8 ) the risk of embolic complications was mostly associated with impaired systolic function.9 ) there were only a few cases about coexisting left ventricular aneurysm.10 - 12 ) we report a rare case of lvnc associated with left ventricular ( lv ) aneurysm , presenting with recurrent embolism . a 40-year - old woman was admitted to our hospital for abdominal and lower back pain lasting for 10 days . in 1999 , she had an embolic stroke of the right middle cerebral artery territory ; however , after 2007 , she was lost to follow - up and discontinued all medications , including antiplatelet and anticoagulant therapy she had none of the classic cardiovascular risk factors such as diabetes or hypertension nor any family history of cardiomyopathy or heart failure . her chest radiography and laboratory data , including b - type natriuretic peptide and cardiac enzymes levels , were unremarkable . abdominal computed tomography , performed because of her abdominal pain , revealed left renal infarction . to evaluate for an embolic source , transthoracic and transesophageal echocardiography were performed . two - dimensional echocardiography showed prominent trabeculations at the interventricular septum and the lateral wall . color doppler imaging revealed deep intertrabecular recesses filled by blood flowing from the ventricular cavity ( fig . the ratio of maximal thickness of the noncompacted to compacted layers in the lateral wall was greater than 2 at end - systole . the 4-chamber and 2-chamber views defined the lvnc in the mid - septal and inferior walls and apical wall thinning ( fig . because wall motion of the apex was akinetic at end - systole and end - diastole , we confirmed the diagnosis as an apical aneurysm . the lv ejection fraction by modified simpson 's rule was 48% ( lv end - diastolic volume 89 ml and lv end - systolic volume 49 ml ) and the ratio of early diastolic mitral inflow velocity to early diastolic mitral septal annular velocity ( e / e ' ) was 7.62 . we performed coronary computed tomography angiography and cardiac magnetic resonance imaging ( mri ) to differentiate the aneurysm from post - infarction aneurysms . on computed tomography 4 ) . cardiac mri showed two - layered appearance of trabeculated and compacted myocardium , it revealed a thin compacted layer preserving contractility and an apical aneurysm with akinetic motion at end - diastole and end - systole ( fig . we therefore diagnosed her with left renal infarction caused by lvnc , coexisting with an lv aneurysm . lvnc is a rare congenital cardiomyopathy characterized by multiple prominent trabeculations with deep intertrabecular recesses.1 ) an arrest of compaction of the developing myocardium is strongly suggested as the probable mechanism of lvnc.1)9 ) recently , the american heart association classified lvnc as a primary genetic cardiomyopathy.13 ) in contrast , the european society of cardiology considers lvnc to be an " unclassified cardiomyopathy".14 ) multiple diagnostic criteria for lvnc have been proposed on the basis of echocardiography and cardiac mri findings . they are as follows : 1 ) thickened myocardium with a 2-layered structure consisting of a thin compacted epicardial layer [ c ] and a much thicker , noncompacted endocardial layer [ n ] or trabecular meshwork with deep endomyocardial spaces ( n / c ratio > 2.0 at end - systole ) ; 2 ) predominant location of the pathology in the mid - lateral , mid - inferior , and apical areas ; 3 ) color doppler evidence of deep intertrabecular recesses filled with blood from the lv cavity ; and 4 ) absence of coexisting cardiac abnormalities ( in isolated lvnc ) . there have been many reports of coexistent congenital cardiac disorders , including atrial septal defect , ventricular septal defect , pulmonary stenosis , anomalous pulmonary venous connection , ebstein 's anomaly , and a bicuspid aortic valve.3 - 6 ) however , only a few cases of lvnc with lv aneurysm have been reported.10 - 12 ) the mechanism of aneurysm is uncertain . sato et al.10 ) proposed impaired microcirculation of noncompacted and compacted layers as the mechanism of aneurysm formation in lvnc . however , in our patient , the epicardial coronary arteries appeared normal on coronary computed tomography angiography and neither perfusion defects nor delayed enhancement were seen on cardiac mri . we therefore thought that our patient 's aneurysm might be congenital rather than degenerative change of lvnc . the classical triad of complications with lvnc is heart failure , ventricular arrhythmia , and systemic embolic events.8 ) because there are limited data regarding treatment of this condition , it is recommended that clinical complications be managed according to the current guidelines for each clinical complication . the prevalence of systemic embolic events in patients with lvnc varied in reports . based on the high rate of embolic events reported in long - term follow - up data , oechslin et al.8 ) recommended anticoagulant therapy for these patients , independent of ventricular systolic function . however , oechslin and jenni9 ) recently recommended anticoagulation therapy for patients with impaired systolic function ( lv ejection fraction < 40% ) because deep intertrabecular recesses and slow blood flow might increase the risk of thrombus formation . we believed that the apical thrombus was the embolic source of her presentation with renal infarction and that the apical aneurysm with slow blood flow was a risk factor for recurrent embolic events . therefore , we suggest that anticoagulation therapy might be needed in patients with lvnc with coexisting lv aneurysm , even in the absence of systolic dysfunction or atrial fibrillation . in conclusion , we described a rare case of lvnc with lv aneurysm presenting as recurrent thromboembolic events . we believe that careful evaluation of lvnc patients for coexisting heart abnormalities such as aneurysms is essential for making the best clinical decisions for their management .
isolated left ventricular noncompaction ( lvnc ) is a rare disorder caused by embryonic arrest of compaction . 
 lvnc is sometimes associated with other congenital cardiac disorders ; however , there have been few reports of its coexistence with a left ventricular aneurysm . 
 a 40-year - old woman was admitted to our hospital for renal infarction . 
 she had a history of embolic cerebral infarction 10 years ago . 
 transthoracic echocardiography showed prominent trabeculae and deep intertrabecular recesses which are filled with blood from the left ventricular ( lv ) cavity . 
 a thrombus in the akinetic apical wall was confirmed by contrast echocardiography . using cardiac computed tomography and magnetic resonance imaging 
 , we rejected a possible diagnosis of suspicion of coronary artery disease . 
 she was diagnosed lvnc with a thrombus in apical aneurysm . here , 
 we report the first patient in korea known to have lvnc accompanying lv congenital aneurysm presenting with recurrent embolism .