This review focuses on recent advances in the association between left ventricular hypertrabeculation/noncompaction (LVHT), a form of unclassified cardiomyopathy, and neuromuscular disorders (NMD). So far, LVHT has been found in single patients with dystrophinopathy, dystrobrevinopathy, laminopathy, zaspopathy, myotonic dystrophy, infantile glycogenosis type II (Pompe's disease), myoadenylate-deaminase deficiency, mitochondriopathy, Barth syndrome, Friedreich ataxia, and Charcot-Marie-Tooth disease. Most frequently LVHT is found in patients with Barth syndrome and mitochondrial disorders. The prevalence of LVHT in NMD patients is not known. On the contrary, NMD can be detected in up to four fifths of the patients with LVHT. Because LVHT is associated with an increased risk of rhythm abnormalities and heart failure, it is essential to detect LVHT as soon as possible. Because of adequate therapeutic options, all patients with NMD should undergo a comprehensive cardiological examination as soon as their neurological diagnosis is established. In reverse, all patients with LVHT should undergo a comprehensive neurological investigation following the detection of LVHT.
An X-linked mitochondrial disease affecting cardiac muscle, skeletal muscle and neutrophil leucocytes
MIDAS syndrome respectively MLS syndrome: a separate entity rather than a particular lyonization pattern of the gene causing Goltz syndrome
Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of cardiomyopathies
Xq28-linked noncompaction of the left ventricular myocardium: prenatal diagnosis and pathologic analysis of affected individuals
Clinical features of isolated noncompaction of the ventricular myocardium: long-term clinical course, hemodynamic properties, and genetic background
Myoadenylate deaminase deficiency. A common inherited defect with heterogeneous clinical presentation
Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis
Myoadenylate deaminase deficiency with progressive muscle weakness and atrophy caused by new missense mutations in AMPD1 gene: case report in a Japanese patient
Effects of carvedilol on left ventricular function, mass, and scintigraphic findings in isolated left ventricular non-compaction
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Spontaneous left ventricular hypertrabeculation in dystrophin duplication based Becker's muscular dystrophy
Noncompaction of ventricular myocardium, complete atrioventricular block and minor congenital heart abnormalities: case report of an unusual coexistence
Isolated non-compaction of the myocardium diagnosed in the fetus: two sporadic and two familial cases
Left ventricular hypertrabeculation/noncompaction and association with additional cardiac abnormalities and neuromuscular disorders
Associations between cardiorespiratory responses to exercise and the C34T AMPD1 gene polymorphism in the HERITAGE Family Study
Noncompaction of the ventricular myocardium: report of two cases with bicuspid aortic valve demonstrating poor prognosis and with prominent right ventricular involvement
Isolated left ventricular non-compaction: cardiomyopathy with homogeneous transmural and heterogeneous segmental perfusion
Clinical characterization of left ventricular noncompaction in children: a relatively common form of cardiomyopathy
A case of superoinferior ventricular heart with situs ambiguus, dextroventricular loop, and levo transposition of the arteries: prenatal and postnatal echocardiographic diagnosis
Mutations in Cypher/ZASP in patients with dilated cardiomyopathy and left ventricular non-compaction
Cardioverter defibrillator implantation in a child with isolated noncompaction of the ventricular myocardium and ventricular fibrillation
Intractable ventricular tachycardia and bridging to heart transplantation with a non-pulsatile flow assist device in a patient with isolated left-ventricular non-compaction
Use of an Amplatzer duct occluder for closing an aortico-left ventricular tunnel in a case of noncompaction of the left ventricle
Isolated left ventricular noncompaction is rarely caused by mutations in G4.5, alpha-dystrobrevin and FK Binding Protein-12
Familial dilated cardiomyopathy and isolated left ventricular noncompaction associated with lamin A/C gene mutations
Comparison of echocardiographic features of noncompaction of the left ventricle in adults versus idiopathic dilated cardiomyopathy in adults
Myoadenylate-deaminase gene mutation associated with left ventricular hypertrabeculation/non-compaction
Double-orifice mitral valve associated with nonisolated left ventricular noncompaction--a case report
The variable clinical presentation of, and outcome for, noncompaction of the ventricular myocardium in infants and children, an under-diagnosed cardiomyopathy
Noncompaction of ventricular myocardium in a patient with congenitally corrected transposition of the great arteries treated surgically: case report
Isolated left ventricular noncompaction: a diagnosis to consider with left ventricular wall akinesis
End-stage cardiac failure caused by isolated ventricular non-compaction: cardiac transplantation in a 5-year-old boy
Isolated left ventricular noncompaction of the myocardium as a cause of embolic superior mesenteric artery occlusion
Alterations of nuclear envelope and chromatin organization in mandibuloacral dysplasia, a rare form of laminopathy
Left ventricular hypertrabeculation/noncompaction as a cardiac manifestation of Duchenne muscular dystrophy under non-invasive positive-pressure ventilation
Left ventricular hypertrabeculation/noncompaction with PMP22 duplication-based Charcot-Marie-Tooth disease type 1A
Cardiac MRI versus echocardiography in assessing noncompaction in children without neuromuscular disease
Exclude pregnancy, vigorous exercise and myopathy before diagnosing noncompaction in healthy subjects
Cardiac and neuromuscular implications of left bundle branch block in left ventricular hypertrabeculation/noncompaction
Left ventricular hypertrabeculation/noncompaction associated with coronary heart disease and myopathy
Deficiency of mannose-binding lectin, myopathy, calcified endomyocardial fibrosis, and left ventricular noncompaction
Cerebrovascular events in left ventricular hypertrabeculation/noncompaction with and without myopathy
Stroke from paroxysmal atrial flutter or left ventricular hypertrabeculation/noncompaction, visible only on transesophageal echocardiography
Atrial fibrillation in left ventricular noncompaction with and without neuromuscular disorders is associated with a poor prognosis
Isolated noncompaction of the left ventricular myocardium complicated by thromboembolic cerebrovascular accident in a patient with essential thrombocythemia
Cardiac involvement over 10 years in myotonic and Becker muscular dystrophy and mitochondrial disorder
Coarctation of the descending aorta, patent ductus arteriosus, deficiency of right superior vena cava, and persistent left superior vena cava in a five-month infant demonstrated by multislice computed tomography
Transient ischemic attack and electrocardiographic abnormalities revealing left ventricular noncompaction
Large recesses associated with left ventricular noncompaction: an unusual presentation of the disease
Don't miss extra-cardiac manifestations of familial left ventricular hypertrabeculation/non-compaction
Bilateral perisylvian polymicrogyria, periventricular nodular heterotopia, and left ventricular noncompaction in a girl with 10.5-11.1 Mb terminal deletion of 1p36
Left ventricular hypertrabeculation (noncompaction) with prominent calcifications in a patient with mannose-binding lectin deficiency and unclassified myopathy
Non-indicated oral anticoagulation in left ventricular hypertrabeculation/noncompaction may jeopardize these patients
A combination of left ventricular hypertrabeculation/noncompaction and muscular dystrophy in a stroke patient
Prognostic impact of left ventricular noncompaction in patients with Duchenne/Becker muscular dystrophy--prospective multicenter cohort study
Rare combination of left ventricular noncompaction, bicuspid aortic valve and myocardial bridging. Rare case or common genetic mutations?
Left ventricular noncompaction associated with hypertrophic cardiomyopathy: echocardiographic diagnosis and genetic analysis of a new pedigree in China
Correlation of trabeculae and papillary muscles with clinical and cardiac characteristics and impact on CMR measures of LV anatomy and function
Novel MYH7 mutation associated with mild myopathy but life-threatening ventricular arrhythmias and noncompaction
Right ventricular noncompaction associated with long QT in a fetus with right ventricular hypertrophy and cardiac arrhythmias
Left Ventricular Noncompaction: Diagnostic Approach, Prognostic Evaluation, and Management Strategies
Echocardiographic and clinical markers of left ventricular ejection fraction and moderate or greater systolic dysfunction in left ventricular noncompaction cardiomyopathy
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