Noncompaction Of Myocardium Research Articles

Anticoagulant drugs in noncompaction: a mandatory therapy?

Noncompaction of left ventricular myocardium is a rare congenital cardiomyopathy resulting from an incomplete myocardial morphogenesis that leads to the persistence of the embryonic myocardium. This condition is characterized by a thin compacted epicardial and an extremely thickened endocardial layer with prominent trabeculations and deep intertrabecular recesses. It is not clear, in noncompaction of myocardium, whether intertrabecular recesses could be responsible for thrombi formation and thromboembolic complications. The prevalence of stroke and echocardiographic finding of thrombus was evaluated in a continuous series of 229 patients (men and women) affected by noncompaction of the left ventricular myocardium, who were included in the SIEC registry. We excluded patients affected by atrial fibrillation. The mean age of the patients was 49.5 years. Fifty percent of the patients were affected by a ventricular systolic dysfunction. The mean period of follow-up was 7.3 years. Only four patients had a history of ischemic stroke. A large thrombus into the left ventricular chamber was observed in a 1-year-old child affected by Behcet's disease (high risk of thrombi formation). Noncompaction of the left ventricular myocardium, by itself, does not seem to be a risk factor for stroke or embolic results, so there is no indication for oral anticoagulant therapy.

Apical Hypertrophic Cardiomyopathy in Childhood: A Long-Term Follow-Up Report of Two Cases

We present two children with apical hypertrophic cardiomyopathy (APH), both of whom remained asymptomatic for more than 15 years. The inverted T-wave on electrocardiograms and myocardial hypertrophy mostly confined to the apical region on echocardiograms showed no significant changes during follow-up. Magnetic resonance imaging revealed a cavitylike portion at the apex in one case, but the diagnosis of noncompacted myocardium was unlikely because there was no blood communication with the true left ventricular cavity. The other case had typically thick and solid myocardium at the apex. The findings in these patients demonstrate that APH might present in childhood and suggest that the prognosis might be good.

Biventricular Noncompaction: A Case Report

Myocardial noncompaction is an extremely uncommon cardiomyopathy. It can present as an isolated cardiac malformation or in association with other congenital anomalies. The left ventricle is usually affected, but a biventricular involvement rarely occurs. A predominant right ventricular involvement has not been reported. Here, we report an 18-year-old woman with biventricular noncompaction that predominantly involves the right ventricle.

Noncompaction Myocardium Imaging with Multiple Echocardiographic Modalities

Noncompaction myocardium of the left ventricle (LV) is a genetically heterogeneous congenital cardiomyopathy, that has only been described during the last two decades. Echocardiography plays a pivotal role as a first line diagnostic tool of this rare abnormality. The present case describes the imaging of the noncompacted myocardium using varying echocardiographic modalities.

Characterization of the electroanatomic substrate in a case of noncompaction left ventricle

An apparently healthy 43-year-old man was submitted to cardiological evaluation for sport activity. Two-dimensional echocardiography led to suspicion of noncompaction deformity, later confirmed by magnetic resonance imaging (MRI), left ventricular catheterization and endomyocardial biopsies. To exclude life-threatening arrhythmias, the patient was submitted to an electrophysiological study and to a real-time three-dimensional electroanatomic reconstruction of left ventricle. The electroanatomic map revealed extensive area of electrical abnormalities. Extent and localization of scar areas mostly corresponded to the areas of enhancement observed at MRI. The present report is the first on electroanatomic substrate evaluation in a noncompaction left ventricle. Our findings show that ventricular noncompaction is characterized by electrical abnormalities including low voltage and scar areas, mainly related to the presence and extent of myocardial fibrosis rather than noncompacted myocardium. Electroanatomic mapping may contribute to detect and quantify fibrotic areas in patients presenting this rare cardiomyopathy.

The perspective of ventricular noncompaction as seen by a nonagenarian

The persistence of so-called spongy myocardium was first reported in humans in the 1960s. Subsequently, a few reports described the condition, utilizing inconsistent facts and terminology. In 1990 the first report appeared using the term "noncompaction", and detailed its clinical implications. Following this report, more descriptions of patients with noncompaction entered the literature. Prior to the availability of high resolution cross-sectional echocardiography, most cases of noncompacted myocardium escaped detection, as few patients underwent angiography. In this essay, I seek to adjudicate and clarify several confusions and controversies in the current literature including the acquired nature of hypertrabeculation, the fate of the so-called sinusoids, the relationship to coronary arteries, and noncompaction as another congenital malformation. The embryonic timing of cessation and reinitiation of compaction is conjectured as an explanation of the varied configurations of noncompaction as seen as a clinical entity. The clinical outcomes of decreased contractility, arrhythmia, and thromboembolism, have been stressed in the current literature, but more study is needed of the gray area between the normal variant and minimal noncompaction. A plea is made for the standardization of methods and terminology.

Myocardial noncompaction and myocardial bridging: A novel coexistence?

Noncompaction of the ventricular myocardium is an increasingly recognized unclassified congenital cardiomyopathy that is frequently misdiagnosed as dilated or apical hypertrophic cardiomyopathy. Coexistence of myocardial noncompaction and myocardial bridging might be a pitfall in the diagnosis of myocardial noncompaction as previous reports have very well elucidated the coexistence of apical hypertrophic cardiomyopathy and myocardial bridging. With this report we aimed to emphasize the significance of watchful echocardiographic evaluation in appropriate diagnosis of myocardial noncompaction.

An unusual case of isolated non-compacted right ventricular myocardium

Isolated ventricular non-compaction is a rare type of cardiomyopathy resulting from arrested myocardial development during embryogenesis. This rare entity can be easily diagnosed by characteristic appearance of prominent myocardial trabeculations and deep inter-trabecular spaces. The clinical manifestations include heart failure signs, ventricular arrhythmias, and cardio-embolic events. Although the usual site of involvement is the left ventricle, the right ventricle (RV) can rarely be affected. Here, we report a case of 23-year-old male patient with isolated RV non-compaction.

Revisiting definition and classification of cardiomyopathies in the era of molecular medicine

Primary myocardial diseases have always attracted the attention of the scientific community because of their obscure aetiopathogenesis, and for years there was confusion and controversy regarding their nosography and taxonomy. Since the first WHO official classification,1 tremendous progress has been made.2 Novel entities have been discovered, requiring an update of the classification in 1995,3 and the aetiology of many forms has been clarified. The Working Group of Myocardial and Pericardial Disease of the European Society of Cardiology (ESC) recently published a position statement4 different from the 2006 American Heart Association (AHA) scientific statement.5 The scope of the present editorial is to deal with the nosographic impact of the advances made since 1995 and to comment on the ESC position statement which has been designed to provide a valid tool for routine clinical practice. The discovery of novel cardiomyopathies, namely arrhythmogenic right ventricular cardiomyopathy (ARVC), primary restrictive cardiomyopathy, and non-compacted myocardium, made a revision of the 1980 classification compulsory, and this led to many substantial, though often questionable, changes.3 Moreover, taking into consideration that the aetiology was becoming clearer and clearer, the definition was changed from ‘heart muscle disease of unknown aetiology’ into ‘myocardial disease associated with cardiac dysfunction’, the term dysfunction meaning both mechanical and electrical abnormality. In the new classification, ARVC6,7 and restrictive cardiomyopathy8 were added to the primary forms, whereas the non-compacted myocardium was left in the limbo of ‘unclassified cardiomyopathies’. The secondary forms, which in the classification of the 1980s were called specific heart muscle diseases, were given the name of ‘specific cardiomyopathies’, and within this group myocarditis was added. Unfortunately, the concept of specific cardiomyopathy was widened too much, so as to include chronic ischaemic, valvular, and hypertensive diseases. Although the 1995 classification made significant contributions … *Corresponding author. Tel: +39 049 8272283, Fax: +39 049 8272284, Email: gaetano.thiene{at}unipd.it

Myocardial noncompaction presenting with Brucella endocarditis

Noncompaction of ventricular myocardium is a unique congenital cardiomyopathy with its own clinical presentation and course. It is more frequently associated with complications of congestive heart failure, thromboembolism and malignant ventricular arrhythmias; however, concomitance of valvular pathologies or infective endocarditis with noncompaction of ventricular myocardium has seldom been reported. We describe herein the first case of left ventricular myocardial noncompaction presenting with Brucella endocarditis with aortic and mitral valve involvements, whom subsequently underwent successful medical and surgical treatment.

Fetal and Neonatal Presentation of Noncompacted Ventricular Myocardium: Expanding the Clinical Spectrum

Noncompaction of the ventricular myocardium (NCVM) is a rare cardiomyopathy characterized by varying degrees of ventricular dysfunction and numerous, prominent trabeculations with deep intertrabecular recesses caused by arrest in myocardial embryogenesis. NCVM is rarely described in fetal and neonatal patients, and only isolated reports exist to date. We conducted a review of clinical and echocardiographic data from 6 neonates found to have NCVM to elucidate aspects of prenatal manifestations, initial presentations, and clinical course/outcome. Six neonates met criteria for diagnosis of NCVM. Five were initially evaluated during fetal life, whereas one patient presented for initial cardiology examination as a neonate. Three of the 5 fetuses had NCVM recognized at the initial examination. Both unrecognized fetuses also had severe prenatal left ventricular dilation and dysfunction. Left ventricular enlargement or increased wall thickness with decreased ejection fraction was evident in all patients at presentation. Mean ejection fraction at presentation was 36% and improved to 57% during an average follow-up of 2 years. Associated congenital cardiac anomalies were noted in 3 patients. After initial improvement, two patients had transient, late decreases in ejection fraction, which improved with medication adjustment. There have been no deaths. Fetuses with enlarged and poorly functioning left ventricles should be evaluated for NCVM, which may not be easily recognized on initial fetal studies. Unlike previous reports of neonatal NCVM, all 6 neonates, including the 3 requiring inotropic support, showed significant early recovery of cardiac function with aggressive therapy.

Primary Noncompaction of the Ventricular Myocardium from the Morphogenetic Standpoint

This review compiles the current knowledge of normal and abnormal myocardial morphogenesis to facilitate an unambiguous diagnosis of primary myocardial noncompaction. During the early stages of development, the formation of trabeculae with the resulting increase in myocardial surface is a adaptation of the rapidly growing heart to improve nourishment by exchange diffusion from the cardiac lumen. Once the coronary vasculature has developed, the switch to cardiac nutrient supply through active circulation from the subepicardial space is paralleled by gradual compaction of the myocardial trabeculae. This results in a decrease of the inner, trabeculated myocardial layer with a parallel increase in thickness of the outer, compact myocardial layer. Similar to the direction of coronary arterial development, this process proceeds from the epicardium toward the endocardium and from the base of the heart to the apex. Based on developmental data, congenital myocardial noncompaction represents a failure of normal embryonic myocardial maturation. The time of arrest of this process will determine the extension of myocardial noncompaction within the ventricle. Whereas disturbances of myocardial microcirculation are frequent in these hearts, direct communications between the myocardial cavity and the coronary arteries (sinusoids) do not belong to this morphogenetic entity.

Isolated noncompaction of the left ventricular myocardium

Isolated noncompaction of the left ventricular myocardium (INVM), first described in 1984, is an unclassified cardiomyopathy and is assumed to occur as an arrest of the compaction process during the normal development of the heart. Between weeks 5 to 8 of human fetal development, the ventricular myocardium undergoes gradual compaction with transformation of the relatively large intertrabecular spaces into capillaries while the residual spaces within the trabecular meshwork gradually flatten or disappear. In the case of INVM, the spaces within the intertrabecular meshwork persist while no other cardiac abnormalities exist. Although there is substantial evidence supporting the developmental hypothesis, other pathogenetic processes responsible for INVM have been discussed. It can be assumed that INVM will be better understood in the future as the molecular genetic basis of cardiomyopathies will be further unravelled. Echocardiography has been shown to be the method of choice in diagnosis of INVM. The diagnostic criteria can be summarized as: 1) appearance of at least four prominent trabeculations and deep intertrabecular recesses; 2) appearance of blood flow from the ventricular cavity into the intertrabecular recesses as visualized by color Doppler imaging; 3) the segments of noncompacted myocardium mainly involve the apex and the inferior mid and lateral mid of the left ventricular wall and typically show a two-layered structure with an endsystolic ratio greater than two between the noncompacted subendocardial layer and the compacted subepicardial layer; 4) absence of coexisting cardiac abnormalities. Magnetic resonance imaging using modern gradient echo sequences has also been shown to diagnose INVM accurately. The clinical presentation of INVM is characterized by a high prevalence of heart failure, thromboembolic events and arrhythmias including ventricular tachycardia and atrial fibrillation. The establishment of a registry, which was initiated by the "Arbeitsgemeinschaft Leitende Kardiologische Krankenhausärzte (ALKK)" recently, may provide further clues for diagnosis, risk stratification, and management of this disease.

Isolated left ventricular noncompaction in a patient presenting with a subacute myocardial infarction

Isolated left ventricular noncompaction is a rare cardiomyopathy that is often not recognised. So far, it is not well established how best to manage this abnormality. We describe a patient in whom the diagnosis of isolated left ventricular noncompaction was made after presentation with a subacute myocardial infarction. Because of nonsustained ventricular tachycardias during hospitalisation, which were inducible and deteriorated into ventricular fibrillation on electrophysiological examination after coronary artery bypass grafting, he received an implantable defibrillator. Whether the ventricular tachycardias were due to the myocardial infarction or to the noncompacted myocardium remains uncertain. (Neth Heart J 2007;15:109-11.).

Non‐compaction cardiomyopathy in an adult with hereditary spherocytosis

A 23-year old male (199 cm, 88 kg) presented muscular weakness due to skeletal myopathy and symptoms of heart failure NYHA functional class II. Total creatine kinase was increased up to 830 U/l, but troponin was negative. Prior episodes of intermittent atrial fibrillation were reported and 6 years ago splenectomy was performed due to hereditary spherocytosis. Cardiac magnetic resonance imaging revealed the spongy appearance of non-compacted left ventricular myocardium. This impaired fetal morphogenesis occurred predominantly in the apical to midventricular anterior, lateral and inferior segments. Non-compaction cardiomyopathy was initially described in paediatric patients. Occasional associations with other congenital disorders are known, e.g., Barth syndrome, which is an X-linked disease characterized by cardio-skeletal myopathy of variable severity and neutropenia. To our knowledge, combined occurrence of non-compaction cardiomyopathy, skeletal myopathy and hereditary spherocytosis has not previously been reported.

Noncompaction of the ventricular myocardium mimicking ischemic cardiomyopathy

A 68-year-old woman was admitted to our hospital because of left ventricular failure. Myocardial perfusion single-photon emission computed tomography demonstrated a non-reversible perfusion defect in the anterolateral left ventricular segments and reduced ejection fraction, findings consisted with ischemic cardiomyopathy accompanied by lateral wall infarction. Coronary angiogram was normal, but the left ventriculogram showed prominent trabeculations in the apical and anterolateral segments. The left ventricular ejection fraction was 28%. Cine magnetic resonance imaging demonstrated prominent trabeculations and deep intertrabecular recesses, findings consistent with noncompaction of the ventricular myocardium. Myocardial hypoperfusion or necrosis in the noncompacted myocardium may be the cause of myocardial damage and possibly the basis of left ventricular failure.

Double-Orifice Mitral Valve Associated with Noncompaction of Left Ventricular Myocardium

Double-orifice mitral valve (DOMV) is a rare anomaly commonly associated with other congenital heart diseases. We present two patients with DOMV and noncompaction of the left ventricular myocardium (NLVM). Case 1 was a 5-year-old male diagnosed with dilated cardiomyopathy. His echocardiogram showed thin myocardium with dilatation at the basal of the left ventricle, thick noncompacted myocardium around the apex of the left ventricle, and DOMV (complete bridge type) with mild mitral regurgitation. Case 2 was an 11-year-old male diagnosed with complete atrioventricular block. His echocardiogram showed thick noncompacted myocardium with mild hypokinesis from the posterior to lateral wall and DOMV (complete bridge type) with mild mitral regurgitation. DOMV is commonly associated with congenital anomaly and always has an abnormal subvalvar apparatus. The mitral valve and its apparatus embryologically originate from the endomyocardium, which is thought to be the origin of noncompacted myocardium. We speculate that patients with DOMV may have NLVM.

Multiple accessory pathways, dual AV nodal physiology, non-compacted myocardium and patent foramen ovale in a patient with Ebstein's anomaly: Report of a case

Ebstein's anomaly is a rare congenital cardiac disease initially described by Wilhelm Ebstein in 1866. The primary pathology involves significant apical displacement of the septal tricuspid valve leaflet and the presence of a redundant, elongated, anterior tricuspid valve leaflet. This congenital malformation has multiple known associated morphological and electrophysiological cardiac pathologies. In the present article, we report, to the best of our knowledge for the first time in the literature, a case of Ebstein's anomaly with multiple accessory bypass tracts, dual AV nodal physiology, non-compacted left ventricular myocardium and a patent foramen ovale. Indeed, complete evaluation of the left heart is required in cases of Ebstein's anomaly to accurately assess left ventricular myocardial structure.

Long-term Survival of a Patient with Isolated Noncompaction of the Ventricular Myocardium

Isolated noncompaction of the myocardium (INCM) is a rare, congenital, unclassified cardiomyopathy. It is caused by a disorder in endomyocardial morphogenesis in the absence of other structural disease. INCM is characterized by numerous prominent trabeculations and deep intertrabecular recesses in the myocardium. Frequently, INCM is associated with an increased incidence of heart failure, arrhythmias, and cardioembolic events with high morbidity and mortality. We describe a 28-year-old woman experiencing symptoms of heart failure since she was 4 years old. She had been intensively investigated and misdiagnosed as having dilated, restrictive, and apical hypertrophic cardiomyopathies. Cardiac magnetic resonance and echocardiography recently revealed the actual diagnosis of INCM. The patient is alive and well, taking vasodilators and warfarin. Herein, we describe the long-term follow-up of this patient and demonstrate that some patients have a favorable prognosis. In addition, the improvement in noninvasive cardiac imaging has revealed a higher prevalence of INCM, previously undetected.

697 Echocardiographic changes in adult patients with non-compacted myocardium

697 Echocardiographic changes in adult patients with non-compacted myocardium