Left ventricular hypertrabeculation/noncompaction

doi:10.1016/S0894-7317(03)00514-5

Journal of the American Society of Echocardiography

Volume 17, Issue 1, January 2004, Pages 91-100

https://doi.org/10.1016/S0894-7317(03)00514-5 Get rights and content

Abstract

In normal human hearts the left ventricle (LV) has up to 3 prominent trabeculations and is, thus, less trabeculated than the right ventricle. Rarely, more than 3 prominent trabeculations can be found at autopsy and by various imaging techniques in the LV. For this abnormality, different synonyms are used such as spongy myocardium, LV noncompaction, and LV hypertrabeculation (LVHT).

In this review it is stated that: (1) LVHT has a higher prevalence than previously thought and the prevalence of LVHT seems to increase with the improvement of cardiac imaging; (2) because LVHT is most frequently diagnosed primarily by echocardiography, echocardiographers should be aware and trained to recognize this abnormality; (3) LVHT is frequently associated with other cardiac and extracardiac, particularly neuromuscular, disorders; (4) there are indications that the cause of LVHT is usually a genetic one and quite heterogeneous; and (5) controversies exist about diagnostic criteria, nomenclature, prognosis, origin, pathogenesis, and the necessity to classify LVHT as a distinct entity and cardiomyopathy by the World Health Organization.

Section snippets

History

LVHT is a fairly long-known abnormality of the LV and was first described in 1932 in a newborn at autopsy who showed aortic atresia and coronary-ventricular fistulae.¹² Intra vitam LVHT was diagnosed for the first time in a 3-month-old female infant with dextrocardia, ventricular septal defect, and subpulmonary stenosis.⁴ In this patient, the ventriculography visualized the spongelike appearance of the LV wall during diastole, with marked retention of the contrast material in the

Pathoanatomic findings

LVHT is most frequently described as an abnormal finding of the LV apex and its adjacent parts of the lateral and inferior wall. In only 4 cases, LVHT of the interventricular septum has been described.19, 29, 41, 74 We currently have no explanation for the observed regional uneven distribution of LVHT within the LV. Possibly, the regional nonuniformity of the LV wall might contribute to this uneven distribution of LVHT. From anatomic studies it is known that in normal human hearts the thickest

Pathohistologic findings

Pathohistologic findings of LVHT, obtained intra vitam by endocardial biopsy or postmortem are unspecific. Either they are reported as being normal11, 20, 50, 60, 66, 71 or as showing subendocardial fibrosis/fibroelastosis,6, 18, 22, 25, 29, 41, 42, 53, 55, 57, 63, 64, 74 myocardial fibrosis,11, 12, 29, 56 myocardial disorganization,¹⁹ myocardial hypertrophy and degeneration,⁵⁰ scarring of the myocardium,⁶⁸ or signs of inflammation.¹¹ Electron microscopy had been performed only in a few cases

Diagnostic criteria

Because of the lack of commonly accepted pathoanatomic diagnostic criteria, different echocardiographic criteria for LVHT are currently used. One group defines LVHT as “numerous, excessively prominent trabeculations and deep intertrabecular recesses.”¹¹ In our experience, this definition is not clear-cut as it does not give a number above which the phenomenon is abnormal. On the contrary, we suggest the anatomically confirmed definition of >3 trabeculations within 1 imaging plane, apically from

Sex and age distribution

Among the 223 published cases in human beings (C.S., unpublished data, 2002),3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36, 37, 38, 39, 40, 41, 42, 43, 44, 45, 46, 47, 48, 49, 50, 51, 52, 53, 54, 55, 56, 57, 58, 59, 60, 61, 62, 63, 64, 65, 66, 67, 68, 69, 70, 71, 72, 73, 74, 75, 76 72 (32%) were female, 147 (66%) were male, and in 4 (2%) sex was not indicated. Possible explanations for this uneven sex distribution

Prevalence

The prevalence of LVHT found in different echocardiographic studies differs from 0.05 to 0.24%/y.62, 69, 76 The differences between the prevalence figures most probably result from different echocardiographic skill and awareness of this abnormality and from the imaging quality of the echocardiographic machine. Echocardiographic machines, using second harmonic imaging, visualize much better the apical regions of the LV, including detection of trabeculations and recesses, which might have been

Association with other cardiac and extracardiac, particularly neuromuscular, disorders

The association with other cardiac and extracardiac abnormalities is listed in Table 1. Coronary angiography had been performed in 74 patients and showed coronary abnormalities in 7 patients, significant coronary arteriosclerosis in 2 patients, and was completely normal in 65 patients. Symptoms of heart failure were reported by 52% of the patients, and palpitations or syncope by 16%. The electrocardiogram finding was abnormal in 74% of the patients, and showed most frequently ST- and T-wave

Familial occurrence

Although there are no systematic investigations on the familial occurrence of LVHT, there are single reports that demonstrate LVHT to occur in multiple family members. Familial occurrence has been reported in siblings,6, 9, 22, 26, 32, 39, 50, 53, 67, 76 half-brothers,⁶ parents and children,43, 50, 62 and in parental consanguinity.26, 42, 50 Two patterns of familial occurrence have been described. Some families show apparent X-linked recessive inheritance.6, 21, 22, 43 However, other family

Association with genetic findings

In LVHT cases, several mutations have been described.15, 22, 32, 33, 34, 35, 43, 59 The mutations concern the G4.5 gene encoding a protein family called the taffazins, genes encoding dystrophin or α-dystrobrevin, genes for transcription factors limited for the most part to the heart and mitochondrial mutations. In other cases, however, studies have been performed that failed to find any mutations in the G4.5 gene.⁵² So far it appears that LVHT is a morphologic abnormality with genetic

Pathogenetic concepts

Several pathogenetic concepts have been proposed to explain the occurrence of LVHT. First, LVHT is hypothesized to represent persistence of embryonal sinusoids and results from an arrest in the compaction process of the myocardium.¹² Although this hypothesis is substantiated by the morphologic similarity of LVHT with the human heart during the eighth to tenth embryonic week, up to now there is no confirmation for this pathogenetic hypothesis.² Arguments in favor of the embryonal hypothesis are

Prognosis

The prognosis of patients with LVHT is assessed controversially. In earlier reports, LVHT was reported to be associated with a high mortality as a result of heart failure and sudden cardiac death.6, 44 In the meantime, many cases of LVHT have been published with a better prognosis.9, 70 The longest follow up of a patient with LVHT is 20 years. This patient had remained free of symptoms for more than 18 years and then developed heart failure, which responded well to therapy.⁶¹ These differences

Therapy

There is no specific therapy for LVHT. However, because LVHT is often associated with other cardiac, neurologic, and noncardiac nonneurologic abnormalities, specific therapy is indicated and effective in patients with LVHT. LVHT associated with heart failure is effectively treated by angiotensin-converting enzyme inhibitors, beta-blockers, and diuretics.47, 61, 70, 73 In addition, in end stages of heart failure, heart transplantation might be necessary, which has been performed in 12 patients

Conclusion

In conclusion: (1) LVHT has a higher prevalence than previously thought and the prevalence of LVHT seems to increase with the improvement of cardiac imaging; (2) because LVHT is most frequently diagnosed primarily by echocardiography, echocardiographers should be aware and trained to recognize this abnormality; (3) LVHT is frequently associated with other cardiac and extracardiac, particularly neuromuscular, disorders; (4) there are indications that the cause of LVHT is usually a genetic one

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Excessive Trabeculation of the Left Ventricle: JACC: Cardiovascular Imaging Expert Panel Paper
2023, JACC: Cardiovascular Imaging
Excessive trabeculation, often referred to as “noncompacted” myocardium, has been described at all ages, from the fetus to the adult. Current evidence for myocardial development, however, does not support the formation of compact myocardium from noncompacted myocardium, nor the arrest of this process to result in so-called noncompaction. Excessive trabeculation is frequently observed by imaging studies in healthy individuals, as well as in association with pregnancy, athletic activity, and with cardiac diseases of inherited, acquired, developmental, or congenital origins. Adults with incidentally noted excessive trabeculation frequently require no further follow-up based on trabecular pattern alone. Patients with cardiomyopathy and excessive trabeculation are managed by cardiovascular symptoms rather than the trabecular pattern. To date, the prognostic role of excessive trabeculation in adults has not been shown to be independent of other myocardial disease. In neonates and children with excessive trabeculation and normal or abnormal function, clinical caution seems warranted because of the reported association with genetic and neuromuscular disorders. This report summarizes the evidence concerning the etiology, pathophysiology, and clinical relevance of excessive trabeculation. Gaps in current knowledge of the clinical relevance of excessive trabeculation are indicated, with priorities suggested for future research and improved diagnosis in adults and children.
Left ventricular noncompaction: a disease or a phenotypic trait?
2022, Revista Espanola de Cardiologia
El miocardio no compactado es una entidad mal definida y en controversia, con una amplia expresividad fenotípica: desde un simple rasgo anatómico hasta una enfermedad con grave afección cardiaca. Los criterios diagnósticos actuales se basan únicamente en hallazgos morfológicos de hipertrabeculación y tienen una baja especificidad para identificar casos de miocardiopatía. El tratamiento del miocardio no compactado también es heterogéneo y no existen guías de práctica clínica específicas. La insuficiencia cardiaca, las arritmias ventriculares y las embolias sistémicas son las complicaciones cardiovasculares más frecuentes. En esta revisión, se tratan las limitaciones diagnósticas de los diferentes criterios disponibles y se propone una aproximación holística alternativa (que incluye variables funcionales por imagen, de caracterización tisular genética y estudio familiar) que puede ayudar en el diagnóstico diferencial de casos con hipertrabeculación. Se describe la genética de esta entidad y el solapamiento con otras miocardiopatías. Por último, se centra en aspectos debatidos del tratamiento clínico y se propone utilizar las mismas variables ya comentadas para la estratificación pronóstica e individualizar el seguimiento de los pacientes.
Left ventricular noncompaction is a poorly defined and controversial entity, with wide phenotypic expression: from a simple anatomical trait to a disease with overt cardiac affection. Current diagnostic criteria rely exclusively on morphologic features of hypertrabeculation, which have low specificity for identifying true cardiomyopathy cases. The management of left ventricular noncompaction is also heterogeneous, and there are no dedicated clinical practice guidelines. The most common cardiovascular complications are heart failure, ventricular arrhythmias, and systemic embolisms. In this review, we discuss the diagnostic limitations of the available criteria, and propose a comprehensive alternative approach (including functional imaging variables, tissue characterization, genetics, and family screening) that may help in the differential diagnosis of hypertrabeculation cases. We also describe the genetic background of the disease and discuss the overlap with other cardiomyopathies. Finally, we focus on controversial issues in clinical management and suggest the use of the previously-mentioned variables for risk stratification and for individualization of patient follow-up.
Molecular and cellular basis of embryonic cardiac chamber maturation
2021, Seminars in Cell and Developmental Biology
Heart malformation is the leading cause of human birth defects, and many of the congenital heart diseases (CHDs) originate from genetic defects that impact cardiac development and maturation. During development, the vertebrate heart undergoes a series of complex morphogenetic processes that increase its ability to pump blood. One of these processes leads to the formation of the sheet-like muscular projections called trabeculae. Trabeculae increase cardiac output and permit nutrition and oxygen uptake in the embryonic myocardium prior to coronary vascularization without increasing heart size. Cardiac trabeculation is also crucial for the development of the intraventricular fast conduction system. Alterations in cardiac trabecular development can manifest as a variety of congenital defects such as left ventricular noncompaction. In this review, we discuss the latest advances in understanding the molecular and cellular mechanisms underlying cardiac trabecular development.
Imaging in ventricular noncompaction
2021, Progress in Pediatric Cardiology
Citation Excerpt :
Autopsy studies have demonstrated that more than 3–4 trabeculations are uncommon in the normal left ventricle [8]. Based on this, diagnostic criteria that included counting >3 trabeculations from one imaging plane apical to the LV papillary muscles and that fill by color Doppler were proposed [9–11] and have become known as the Stollberger criteria. Noted amongst most of these studies was regional distribution of noncompacted segments, typically localized at the LV apex and mid-ventricular segments [5–7,9].
Ventricular noncompaction is a complex clinical and imaging diagnosis typically evaluated by echocardiography and cardiac magnetic resonance imaging. Variable phenotypes from benign variants of normal to severe forms of symptomatic cardiomyopathy may share imaging characteristics. While the gold standard for diagnosis of ventricular noncompaction cardiomyopathy by current imaging modalities remains elusive, advanced quantification methods in both echocardiography and magnetic resonance imaging are improving.
This review seeks to describe the current state of imaging methods and modalities that may aid in the diagnosis and differentiation of isolated, benign ventricular noncompaction from cardiomyopathy with noncompaction.
A multimodality imaging approach may allow imagers and clinicians to discriminate between benign and pathologic forms. However, an integrated imaging, clinical and genetic effort will be necessary to advance our understanding of this heterogenous cardiomyopathy.
Hypertrabeculation; a phenotype with Heterogeneous etiology
2021, Progress in Cardiovascular Diseases
Left ventricular hypertrabeculation (LVHT) is a phenotype with multiple etiologies and variable clinical presentation and significance. It is characterized by a 2-layer myocardium with an enlarged trabecular layer and a thinner compacted layer. The prevalence has been increasing due to advances in cardiac imaging. Initial attention was focused on the congenital noncompaction syndrome, and the presence of LVHT was always attributed to this etiology. However, due to the lack of consensus diagnostic criteria, LVHT has now been reported in a broad spectrum of cardiomyopathies, congenital heart diseases, monogenetic disorders, neuromuscular diseases, and even healthy individuals. LVHT is often associated with systolic dysfunction, arrhythmias, and thromboembolic events. Given the etiologic heterogeneity, the prognosis and outcomes are primarily determined by comorbidities, and treatment is dictated by known guidelines. We present hypertrabeculation (HT) as a phenotype and discuss the varied landscape in the classification, etiology, diagnosis, and management of the condition.
Left ventricular mechanics and cardiovascular outcomes in non-compaction phenotype
2021, International Journal of Cardiology
This study aims at understanding left ventricular (LV) mechanics of non-compaction (LVNC) phenotype using echocardiographic strain analysis and at assessing the association of functional parameters with cardiovascular (CV) outcomes.
Longitudinal (GLS) and circumferential strain (GCS) as well as rotation of the LV were analyzed in 55 LVNC patients and 55 matched controls. Cardiovascular outcomes were documented for a median follow-up duration of 6 years. GLS and GCS were impaired in LVNC. Similary, regional longitudinal and circumferential strain as well as twist were reduced. CV events occurred in 28 LVNC patients. Apical peak circumferential strain (APCS), peak systolic rotation of apical segments (APSR), and twist were strongly associated with events. This was independent of and incremental to LVEF and non-compacted to compacted myocardial thickness ratio (NC:C ratio). The association of twist with events was also independent of and slightly superior to GLS.
GLS, GCS, regional strain, and twist were impaired in LVNC. APCS, APSR, and twist exhibited strong association with CV events independent of and incremental to LVEF and NC:C ratio, and in case of twist even GLS. Thus, STE-derived parameters may complement the echocardiographic assessment of LVNC patients in clinical routine.

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Review articleLeft ventricular hypertrabeculation/noncompaction

Abstract

Section snippets

History

Pathoanatomic findings

Pathohistologic findings

Diagnostic criteria

Sex and age distribution

Prevalence

Association with other cardiac and extracardiac, particularly neuromuscular, disorders

Familial occurrence

Association with genetic findings

Pathogenetic concepts

Prognosis

Therapy

Conclusion

J Am Coll Cardiol

Am J Cardiol

Am J Cardiol

J Am Coll Cardiol

J Am Soc Echocardiogr

Am J Hum Genet

J Heart Lung Transplant

Am Heart J

J Am Soc Echocardiogr

J Am Coll Cardiol

Mayo Clin Proc

Rev Esp Cardiol

Cardiovasc Pathol

Am J Cardiol

J Am Coll Cardiol

Am J Cardiol

J Am Soc Echocardiogr

Am J Hum Genet

Dev Biol

Dev Biol

J Biol Chem

J Biol Chem

J Biomech

Developmental patterning of the myocardium

Anat Rec

Large vascular sinuses in the myocardium of a dog

Anat Rec

Isolated noncompaction of left ventricular myocardiuma study of eight cases

Circulation

Isolated left ventricular abnormal trabeculation in adults is associated with neuromuscular disorders

Clin Cardiol

Hypertrabeculated left ventricle in mitochondriopathy

Heart

Isolated noncompaction of the ventricular myocardiuma study in an adult male and literature review

Ital Heart J

Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compactiona step towards classification as a distinct cardiomyopathy

Heart

Congenital heart disease with multiple cardiac anomaliesreport of a case showing aortic atresia, fibrous scar in myocardium and embryonal sinusoidal remains

Am J Med Sci

Noncompaction of ventricular myocardiumCT appearance

AJR Am J Roentgenol

Isolated noncompaction of the ventricular myocardiumultra fast computed tomography and magnetic resonance imaging

Int J Card Imaging

Left ventricular non-compaction in a patient with Becker's muscular dystrophy

Heart

MR appearance of isolated noncompaction of the left ventricle

J Magn Reson Imaging

Dysplastic cardiac development presenting as cardiomyopathy

Arch Pathol Lab Med

Myocardial dysgenesis with persistent sinusoids in a neonate with Noonan's phenotype

Pediatr Pathol

Evolutionary persistence of spongy myocardium in humans

Circulation

Xq28-linked noncompaction of the left ventricular myocardiumprenatal diagnosis and pathologic analysis of affected individuals

Am J Med Genet

Isolated noncompaction of the myocardiuman exceedingly rare cardiomyopathy; a case report

Ital Heart J

Nao compactacao isolada do miocárdio ventricular esquerdo

Rev Port Cardiol

Genetic heterogeneity of isolated noncompaction of the left ventricular myocardium

Am J Med Genet

Persistance du myocarde spongieuxà propos d'un cas

Arch Mal Coeur Vaiss

Noncompaction of ventricular myocardium and arrhythmias

Review article
Left ventricular hypertrabeculation/noncompaction