In most cases, an affected person inherits the mutation. Isolated left ventricular noncompaction ivnc was first described just over a decade ago,1 and is now gaining prominence as a rare, but important, differential in the diagnosis of patients presenting with cardiac failure. The diagnosis was made postnatally with ultrasonography and was verified by cardiac mri. Hall, phdz abstract whether left ventricular noncompaction lvnc is a distinct cardiomyopathy or a morphologic trait shared by different cardiomyopathies remains controversial.
It has been proposed that lvnc results from arrest of the compaction process during the. Within the caribbean setting, there are limited data regarding its prevalence or documented existence. In addition to history and physical examination, diagnosis of cardiomyopathy includes electrocardiography and echocardiography testing. Isolated left ventricular non compaction cardiomyopathy in pregnancy khanna, sandeep a,b. Noncompaction cardiomyopathy outcomes american college. Noncompaction cardiomyopathy what is left ventricular noncompaction cardiomyopathy.
Nccm can also be detected when competitive athletes undergo preparticipation. Nccm is characterized by excessive trabeculations typically involving the left ventricle lv with 2. Peter morcos n, ulrika andersson m e and eric adler d. The isolated noncompaction cardiomyopathy was reported for the first time by chin et al 2 in 1990, who described eight cases of the disease 2. Noncompaction cardiomyopathy ncc is a rare genetic cardiomyopathy characterized by a thin, compacted epicardial layer and an extensive noncompacted endocardial layer. If you continue browsing the site, you agree to the use of cookies on this website.
Primary cardiomyopathy genetic hcm arvdc lv non compaction glycogen storage mitochondrial myopathy muscular dystrophies neuromuscular disorders ion channels disorder lqts, brugada, sqts, cvpt mixed dilated cardiomyopathy restrictive non hypertrophied and non dilated acquired inflammatory. In left ventricular noncompaction cardiomyopathy lvnc the lower left chamber of the heart, called the left ventricle, contains bundles or pieces of muscle that. Left ventricular noncompaction lvnc australian genetic. Left ventricular noncompaction cardiomyopathy lvnc, which was. Current guidelines from professional organizations recommend different stra tegies for diagnosing and treating patients with lvnc. Left ventricular noncompaction cardiomyopathy journal of the. Left ventricular noncompaction cardiomyopathy the lancet. Value of cardiovascular mr in diagnosing left ventricular noncompaction cardiomyopathy and in discriminating between other cardiomyopathies.
Left ventricular noncompaction, the most recently classified form of cardiomyopathy, is characterised by abnormal trabeculations in the left ventricle, most frequently at the apex. It can affect the hearts ability to work efficiently as a pump, and affect the electrical signalling of the heart. Noncompaction cardiomyopathy nccm is typified by deep invaginations of the myocardium and is caused by an arrest of normal myocardial morphogenesis. Left ventricular noncompaction lvnc is the most recently classified cardiomyopathy, first described in 1990, and has gained increasing attention in the past two decades. Left ventricular non compaction lvnc is a cardiomyopathy characterized by prominent left ventricular trabeculae, deep intertrabecular recesses, and thin compacted layer. Left ventricular noncompaction cardiomyopathy thoracic key. Aortic coarctation was initially repaired surgically.
A new diagnosis of left ventricular noncompaction in a. Morrison and others published pediatric mixed left ventricular noncompaction and restrictive cardiomyopathy bridged to. Division of cardiology, university of california san diego, california, usa. Left ventricular noncompaction lvnc is a cardiomyopathy characterized anatomically by deep.
Anticoagulation therapy in specific cardiomyopathies. Progressive left ventricular noncompaction and systolic. Noncompaction cardiomyopathy is a rare form of cardiac disease that presents with symptoms of congestive heart failure, ventricular arrhythmias or thromboembolism. This gives the left ventricle a characteristic spongy look a bit like honeycomb. Alternatively, an idiopathic dilated cardiomyopathy could have been the primary disease and the progression of noncompaction may represent a compensatory response to worsening systolic dysfunction, although this is less likely given that mild noncompaction and systolic dysfunction were present before left ventricular dilation. Left ventricular noncompaction in a child with bicuspid. Lv non compaction noncompaction of the left ventricular myocardium lvnc is being increasingly recognized and its diagnosis has moved from the autopsy table or previously poorly recognized entity to a widely recognized. Noncompaction cardiomyopathy cardiology online education. The etiology of lvnc is presumed to be an arrest of the normal process of intrauterine endomyocardial morphogenesis although this theory is not universally held 2, 28. Echocardiographic comparison between left ventricular non. At age 3 months, recoarctation and heart failure developed. Bustamante, sergio c colombian journal of anesthesiology.
Lvnc, also known as spongy myocardium, is a distinct form of cardiomyopathy occurring inutero when segments of spongy myocardium fail to transform into compact, mature musculature resulting in prominent myocardial trabeculae, deep intratrabecular recesses, and decreased cardiac function. Limitations in the diagnosis of noncompaction cardiomyopathy by. Whether left ventricular noncompaction lvnc is a distinct cardiomyopathy or a morphologic trait shared by different cardiomyopathies. Mutations in genes that are associated with other types of cardiomyopathies particularly hypertrophic and. Cardiomyopathies due to left ventricular noncompaction. Isolated left ventricular noncompaction lvnc has usually been viewed as a rare cardiomyopathy in athletes. Left ventricular noncompaction lvnc is a rare congenital cardiomyopathy characterized by a spongy feature of the myocardium. Isolated left ventricular non compaction cardiomyopathy in. Prognosis of isolated left ventricular noncompaction in.
In most cases, echocardiography in experienced hands is sufficient to make the diagnosis. However, with advances in diagnostic imaging techniques and increased use of preparticipation screening electrocardiograms ecgs, apparent lvnc is being recognized in an increasing number of athletes. Left ventricular noncompaction lvnc is a cardiomyopathy characterized by two layered structures composed of prominent trabecular. This means that an affected person has a 1 in 2 50% chance of passing the gene alteration on to children and males and females are affected equally. Left ventricular noncompaction lvnc is a rare cardiomyopathy and should be considered in the differential diagnosis of patients presenting with unexplained heart failure. This causes channels to form in the heart muscle, called trabeculations. Different types of cardiomyopathy associated with isolated ventricular noncompaction. The 2dimentional echocardiography, 3dimentional echocardiography, color doppler echocardiography and contrastenhanced echocardiography are of critical importance for diagnosis and family. A thin, compacted epicardial layer under an extensive noncompacted endocardial layer can be noticed with the common cardiac image techniques. Left ventricular noncompaction a distinct cardiomyopathy or a trait shared by different cardiac diseases.
Pdf pediatric mixed left ventricular noncompaction and. In 2 distinct entities, left ventricular noncompaction lvnc and peripartum cardiomyopathy ppcm, routine anticoagulation therapy is often used in current practices. Noncompaction cardiomyopathy ncm is a myocardial disorder, which is thought to occur due to the failure of left ventricle lv compaction during embryogenesis, leading to distinct morphological characteristics in the ventricular chamber. Also known as isolated noncompaction of the left ventricular myocardium or spongiform.
It results from the failure of myocardial development during embryogenesis during development, the majority of the heart muscle is a spongelike meshwork of interwoven myocardial fibers. Eloisa arbustini, md, frank weidemann, md,y jennifer l. Isolated ventricular noncompaction with restrictive cardiomyopathy. Cardiomyopathy phenotypes and outcomes for children with left. The following are key points to remember from this report on a multicenter retrospective study from the netherlands that analyzed patients with noncompaction cardiomyopathy nccm. Left ventricular noncompaction lvnc is a distinct form of cardiomyopathy characterized by hypertrabeculation of the left ventricle. Left ventricular noncompaction is an increasingly recognized familial cardiomyopathy characterized. Left ventricular noncompaction lvnc can share the same presentation as dcm including asymptomatic disease, progressive deterioration of cardiac function, arrhythmias, thromoboembolic events, and sudden cardiac death. Lvnc is a condition where the muscular wall of the left ventricle of the heart appears spongy. Left ventricular noncompaction cardiomyopathy cincinnati.
Sports participation in noncompaction cardiomyopathy. During development, the heart muscle is a spongelike network of muscle fibers. This unclassified cardiomyopathy, previously known as spongy left ventricular myocardium, is characterised by prominent myocardial trabeculations. Limitations in the diagnosis of noncompaction cardiomyopathy by echocardiography. Left ventricular noncompaction genetics home reference nih. Left ventricular noncompaction an introduction to lvnc. Left ventricular noncompaction cardiomyopathy lvnc is a rare condition with high morbidity and mortality due to malignant arrhythmias, systemic thrombotic embolism and heart failure. Left ventricular noncompaction cardiomyopathy lvnc, noncompaction cardiomyopathy nccm is characterized by the presence of excessive left ventricular trabeculae, deep intratrabecular recesses, and a thin compacted myocardial layer. Noncompaction cardiomyopathy a diagnostically challenging cardiomyopathy 2. Echo assessment of cardiomyopathy pdf slideshare uses cookies to improve functionality and performance, and to provide you with relevant advertising. Lvnc is a condition of the heart where the walls of the left ventricle the bottom chamber of the left side of the heart are non.
Lvnc is a condition of the heart where the walls of the left ventricle the bottom chamber of the left side of the heart are noncompacted. Isolated left ventricular noncompaction lvnc is a genetic cardiomyopathy characterized by prominent ventricular trabeculations and deep intertrabecular recesses, or sinusoids, in communication with the left ventricular cavity. Noncompaction cardiomyopathy ncc, is a rare congenital cardiomyopathy that affects both children and adults. Left ventricular noncompaction cardiomyopathy lvnc is a newly recognized form of cardiomyopathy still in the early phases of medical understanding. Prognosis of isolated left ventricular noncompaction in adults ncvg the safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Abstract whether left ventricular noncompaction lvnc is a distinct cardiomyopathy or a morphologic trait shared by different cardiomyopathies remains controversial.
Families with left ventricular noncompaction lvnc have been shown to pass the disease on in two different ways, via autosomal dominant or xlinked inheritance. Catastrophic stroke in a patient with left ventricular non. Myocardial noncompaction is a rare disorder of uncertain etiology. The current approach to diagnosis and management of left. The isolated noncompaction cardiomyopathy was reported for the first time by chin et al 2 in 1990, who described eight. The clinical manifestations of this disease include ventricular arrhythmia, heart failure, and systemic thromboembolism. Noncompaction cardiomyopathy is a clinically heterogeneous disease, which is characterized by the presence of excessively prominent trabeculations in the myocardium accompanied by crypts and a very thin layer of compacted heart muscle. Echocardiography is the method of choice to establish a diagnosis and determine a treatment plan for patients with noncompaction of ventricular myocardium nvm. Dilated cardiomyopathy can be genetic or acquired and typically presents. We present the case of a 3yearold boy with bicuspid aortic valve, aortic coarctation, and left ventricular noncompaction.
Listing a study does not mean it has been evaluated by. Investigation is typically with a transthoracic echocardiogram tte. Echo assessment of cardiomyopathy pdf linkedin slideshare. The first reported cases of noncompaction cardiomyopathy ncc were associated with congenital heart disease with obstructed outflow tract of the left and right ventricle, complex cyanotic congenital malformations and coronary anomalies 1. Lvnc is characterized by a pattern of prominent trabecular meshwork and deep intertrabecular recesses communicating with the left ventricular lv cavity. Left ventricular noncompaction can have different inheritance patterns in most cases, including when the condition is caused by mutations in the myh7 or mybpc3 gene, left ventricular noncompaction is inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder. It can be associated with left ventricular dilation or hypertrophy, systolic or diastolic dysfunction, or both, or various forms of congenital heart disease. In left ventricular noncompaction cardiomyopathy lvnc the lower left chamber of the heart, called the left ventricle, contains bundles or pieces of muscle that extend into the chamber. The low prevalence of patients with this cardiomyopathy presents a unique challenge for large, prospective trials to assess its.
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