JACC: CARDIOVASCULAR IMAGING VOL. -, NO.

-, 2023
ª 2023 THE AUTHORS. PUBLISHED BY ELSEVIER ON BEHALF OF THE AMERICAN

COLLEGE OF CARDIOLOGY FOUNDATION. THIS IS AN OPEN ACCESS ARTICLE UNDER

THE CC BY LICENSE (http://creativecommons.org/licenses/by/4.0/).

STATE-OF-THE-ART PAPER

Excessive Trabeculation of the

Left Ventricle
JACC: Cardiovascular Imaging Expert Panel Paper

Steffen E. Petersen, MD, DPHIL,a,b Bjarke Jensen, MSC, PHD,c Nay Aung, MBBS, PHD,a,b Matthias G. Friedrich, MD,d,e
Colin J. McMahon, MD,f Saidi A. Mohiddin, MBCHB, MD,a,b Ricardo H. Pignatelli, MD,g Fabrizio Ricci, MD, PHD,h
Robert H. Anderson, MD, PHD (HON),i David A. Bluemke, MD, PHDj

ABSTRACT

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. (J Am Coll Cardiol Img 2023;-:-–-)
© 2023 The Authors. Published by Elsevier on behalf of the American College of Cardiology Foundation. This is an open
access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).

E xcessive trabeculation is a ventricular pheno-

type identified by imaging studies, most
frequently echocardiography
magnetic resonance (CMR).1 Similar to ventricular
and cardiac
wall thickness or diameter, the presence of excessive
trabeculation by itself does not define the presence of
cardiomyopathy. As pointed out by Jenni, Oechslin,
and colleagues, 2,3 excessive trabeculation may be a

From the aWilliam Harvey Research Institute, National Institute for Health and Care Research Barts Biomedical Research Centre,
Queen Mary University London, London, United Kingdom; bBarts Heart Centre, St Bartholomew’s Hospital, Barts Health National
Health Service Trust, London, United Kingdom; cDepartment of Medical Biology, Amsterdam Cardiovascular Sciences, University
of Amsterdam, Amsterdam University Medical Center, Amsterdam, the Netherlands; dDepartment of Medicine, McGill University
Health Centre, Montreal, Quebec, Canada; eDepartment of Diagnostic Radiology, McGill University Health Centre, Montreal,
Quebec, Canada; fDepartment of Paediatric Cardiology, Children’s Health Ireland at Crumlin, Dublin, Ireland; gDepartment of
Pediatric Cardiology, Texas Children’s Hospital, Houston, Texas, USA; hDepartment of Neuroscience, Imaging, and Clinical
Sciences, “G.d’Annunzio” University of Chieti-Pescara, Chieti, Italy; iBiosciences Institute, Newcastle University, Newcastle,
United Kingdom; and the jSchool of Medicine and Public Health, University of Wisconsin, Madison, Wisconsin, USA.
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’
institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information,
visit the Author Center.

Manuscript received July 11, 2022; revised manuscript received December 7, 2022, accepted December 22, 2022.

ISSN 1936-878X https://doi.org/10.1016/j.jcmg.2022.12.026

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Excessive Trabeculation—An Expert Panel Paper - 2023:-–-

ABBREVIATIONS normal variant or a physiological response to ventricular walls.10 As we will emphasize, recent data
AND ACRONYMS conditions of increased preload or afterload, on embryogenesis do not support this concept.
such as pregnancy or athletic participation. 4,5 Nonetheless, large series using echocardiography in
CMR = cardiac magnetic
resonance
Because of the considerable variation in children and adults have estimated prevalence of
extent of ventricular trabeculation in the so-called noncompaction cardiomyopathy between
population, individuals with excessive trabeculation 0.02% and 0.14%.11-14 The estimates, however,
in isolation may pose diagnostic and management are limited by selection bias, varying definitions
challenges. 6 For example, excessive trabeculation is of excessive trabeculation, and an unclear relation-
reported to be associated with some pathological con- ship to contemporary views regarding myocardial
ditions, including well-recognized heart muscle dis- development.
orders. In such cases, it frequently remains unclear
DEFINITIONS OF
whether the phenotype itself identifies a very specific
EXCESSIVE TRABECULATION
(and rare) cardiomyopathy (ie, so-called left ventricu-
lar noncompaction cardiomyopathy) or represents a
Several quantitative definitions for excessive
secondary response to altered preload and/or after-
trabeculation have been proposed (Table 1). Most
load in patients with myocardial dysfunction. 7,8 We
frequently, echocardiographers use the criteria pub-
list the references to so-called noncompaction in the
lished by Jenni et al.3 These include, first, the pres-
current international guidelines in the Supplemental
ence of a 2-layered myocardium; second, a ratio of
Table 1.
trabecular to compact myocardium >2:1 measured in
In this expert consensus paper, we summarize the
end-systole in the short-axis view. The typical loca-
published reports relevant to excessive trabeculation
tion of the most pronounced trabeculation in the mid
and its association with cardiomyopathy in both
lateral, apical and mid inferior segments and the
children and adults (see Supplemental Figure 1 for
absence of coexisting cardiac abnormalities is pre-
publication scale and trends). Except for historical
sumed. In a recent meta-analysis seeking to assess
context, we avoid the term “noncompaction” because
the prevalence of so-called noncompaction, 15 almost
new developmental biology research shows that the
two-thirds of published echocardiographic studies
term misrepresents the nature of fetal development
used criteria that included trabecular to compact
of the ventricular walls. We use the term “excessive
myocardium >2:1 at end-systole. Using this reference,
trabeculation” when left ventricular trabecular
prevalence among 23 cohorts was 0.56%. 15 To our
morphology exceeds previously described thresholds.
knowledge, prevalence of excessive trabeculation, at
In addition, we offer considerations for the manage-
0.076%, has been assessed in only 1 population-based
ment of patients in a variety of situations with
neonatal echocardiographic study. 16
excessive trabeculation.
CMR has increasingly been used to characterize
EARLY PUBLISHED REPORTS OF myocardial disorders. Compared to echocardiogra-
LEFT VENTRICULAR phy, CMR has greater contrast resolution and blood-
NONCOMPACTION CARDIOMYOPATHY muscle differentiation, allowing better visualization
of ventricular trabeculation. Like echocardiography,
The early medical published reports on excessive several criteria have been suggested (Table 1), with
trabeculation may be divided into 2 areas of research. the one proposed by Petersen et al 17 most frequently
The first involved infants or young children with applied. This criterion requires a ratio of the trabec-
marked ventricular trabeculation in association with ular to compact myocardial thicknesses >2.3 at
congenital heart disease, which was often fatal. The end-diastole in long-axis views. Cardiac computed
second concerned adults with cardiomyopathy of tomography and, to a lesser extent, invasive ven-
unknown origin and/or arrhythmias, again with triculography can also visualize left ventricular tra-
marked trabeculation of the left ventricle. In infants, beculation. However, there are no generally accepted
the deep endomyocardial spaces found with exag- morphologic diagnostic criteria for either technique.
gerated trabeculations, often called sinusoids, were Excessive trabeculation measured by current
suggested to be secondary to excessive intracavitary criteria occurs in individuals without cardiomyopa-
pressures during embryonic and fetal development in thy. When applied to general populations, the
association with structural heart disease. 9 The finding Petersen criteria were met in about 20% of partici-
in adults, in contrast, was proposed to be secondary pants in 5 population-representative cohorts.15 In the
to an arrest of a presumed process of compaction of MESA (Multi-Ethnic Study of Atherosclerosis), 43% of
embryonic trabeculations to form the compact participants who were asymptomatic without cardiac
JACC: CARDIOVASCULAR IMAGING, VOL. -, NO. -, 2023 Petersen et al 3
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T A B L E 1 Examples of Echocardiographic and CMR Approaches to Determining the Extent of LV Trabeculations

Jenni et al3 Petersen et al17 Jacquier et al105 Stacey et al 139 Captur et al140
Modality Echocardiography CMR CMR CMR CMR

Sample size Noncompaction (n ¼ 34) Noncompaction (n ¼ 7) Noncompaction (n ¼ 16) Noncompaction Noncompaction (n ¼ 30)
No control group Control subjects (n ¼ 170) Control subjects (n ¼ 48) (n ¼ 122) Control subjects (n ¼ 105)
No control group
Study design/external Retrospective/no external Retrospective/no external Retrospective/no external Retrospective/no Retrospective/no external
validation validation cohort validation cohort validation cohort external validation cohort
validation cohort
Definition of Absence of coexisting Bilayered appearance on Diagnosis of Consecutive patients Diagnosis of noncompaction
noncompaction cardiac disease echocardiography noncompaction was from CMR reports on echocardiographic
Numerous excessively combined with increased established on that mention criteria and at least 1 of
prominent pretest probability (eg, echocardiographic trabeculation or the following: positive
trabeculations and deep similar appearance in criteria noncompaction family history, associated
intertrabecular recesses first-degree relatives, neuromuscular disorder,
Intertrabecular spaces filled associated neuromuscular regional wall motion
by direct blood flow disorder, or complications, abnormality,
from the ventricular such as systemic noncompaction-related
cavity, on color Doppler embolization and regional complications
imaging wall motion abnormalities) (arrhythmia, heart failure,
or thromboembolism)
Description Noncompaction to Two-layered myocardium Short-axis cines for total LV Apical short-axis views Loss of base-to-apex
compaction ratio Measured at the most mass and compact 16-24 mm from fractional dimension
Decreased thickening and pronounced trabeculations, mass to define the true apical slice gradient
hypokinesia present avoiding apex trabecular mass Region with the
within, but not limited Measurement perpendicular to Papillary muscle included in largest
to, the noncompacted compact myocardium the myocardial mass noncompaction to
segments compaction ratio
Cardiac phase End-systole End-diastole End-diastole End-systole End-diastole
Cardiac view Short axis Long axes (4- chamber, Short-axis stack Apical short axis Short-axis stack
2-chamber, 3-chamber)
Excessive Noncompaction to Noncompaction to Trabecular mass >20% Noncompaction to Fractal dimension $1.30
trabeculation compaction ratio >2 compaction ratio >2.3 compaction
cutoff ratio $2

These definitions highlight variation in current definitions of excessive trabeculation. Because imaging studies are typically needed to define disease presence without other independent standard of
reference, inclusion bias is typically present in such studies. Note that “noncompaction” refers to terms in the original references, rather than the more contemporary description of excessive trabeculation.
CMR ¼ cardiac magnetic resonance; LV ¼ left ventricular.

disease or hypertension met the Petersen criteria in at However, in 700 patients referred for CMR, high
least 1 myocardial segment.1 Investigators have, fractal dimension was present in 23 patients and was
therefore, also considered parameters such as the also indeterminate in predicting cardiovascular
relative or absolute thickness and mass of the events. 25 Specific functional indices proposed to
trabecular and compact layers, 18 the number of diagnose or risk stratify patients with latent or overt
segments affected,19 the location of the affected cardiomyopathy with excessive trabeculation are
segments,20 the trabecular morphology,21 and the attractive because they target the underlying
morphology of the papillary muscles. 22 functional disturbance.26-29 They do not, however,
It is also possible that conventional descriptors are provide evidence of causality to a possible morpho-
too simplistic to capture our visual impression of logic appearance of excessive trabeculation.
excessive trabeculation. The CMR fractal dimension
is an example of a sophisticated and highly repro- THE EMBRYOLOGY OF VENTRICULAR
ducible mathematical scoring of endocardial DEVELOPMENT; NONCOMPACTION AS
complexity ranging from 1 (a straight line) to 2 A MISNOMER
(complete filling of the 2-dimensional space con-
tained by the ventricular trabeculation).23 In MESA,24 In normal development, the trabecular parts of the
fractal dimensions were larger in individuals with ventricles are known to balloon out from the outer
hypertension, greater left ventricular wall thickness, curvature of an initial primary tube, 30,31 with the
and greater left ventricular mass. African American trabeculations forming secondary to signaling from
participants had greater fractal dimensions than the endocardium. 32-34 By the fifth week of gestation,
White participants did. In this regard, the fractal the ventricular walls are extensively trabeculated
dimension provided insight into factors now under- (Figure 1A). Over the subsequent embryonic, fetal,
stood to result in greater trabecular thickness. and postnatal periods of development, the heart
4 Petersen et al JACC: CARDIOVASCULAR IMAGING, VOL. -, NO. -, 2023
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F I G U R E 1 Developmental Anatomy of the Human LV

A 5 weeks C 21 weeks
A B

LV
T
C
B
7 weeks
LV

LV
T

T
C
C 5 mm

(A) At 5 weeks gestation, Carnegie stage 14 is shown; a substantial trabeculated (T) wall has developed; and the compact wall (C) is thin.
(B) At 7 weeks gestation, Carnegie stage 19 is shown, and the ventricular wall remains much trabeculated. (C) At 21 weeks gestation, the
heart (and fetus) has grown tremendously, notice the images of the 2 embryonic hearts of A and B are inserted to scale. The compact wall is
now much thicker than in the embryonic stages and so is the trabecular layer. This illustrates that a decrease of the trabecular layer, that is,
compaction, is not required for the formation of a thick compact wall. LV ¼ left ventricle.

grows many-fold (Figure 1). 35,36 During these periods, positive growth of the trabecular and compact
both the trabecular and the compact myocardial myocardial layers are in direct opposition to earlier
layers also grow many-fold, but not always in equal notions suggesting that the compact layer is formed as
proportion (Figure 1).37,38 The ratio of thicknesses a result of “compaction” of pre-existing trabecula-
between the layers decreases during development, tions.9,46 The presumed process of “compaction” has
despite an increase in the volume of both, indicating been considered to represent an “intrauterine
greater growth in the compact than the trabecular arrest.” 10,47,48 There is currently no evidence of which
layer (Figure 2). These morphometric observations are we are aware to support this notion. It follows that the
amply supported by pulse labeling and immunohis- term “noncompaction” has no foundation in myocar-
tochemical studies, which show greater proliferation dial development. 38,49,50
of cardiomyocytes in the compact wall than in the
layers making up the trabeculations.39-42 When pro- MOLECULAR BIOLOGY OF THE
liferation is inhibited experimentally in the trabecular DEVELOPING MYOCARDIUM
layer, compact mural thickness is largely unaf-
fected.43 The compact wall is also able to form nor- Studies of developing human and animal hearts have
mally even when excessive trabeculation is induced shown that most cardiomyocytes of the trabecular
by suppression of NKX2-5. 44 For its normal develop- layer initially have a different molecular identity
ment, therefore, growth of the compact wall is largely from those in the neighboring compact layer. 31 Later,
independent of that in the trabecular layer. during the fetal and postnatal periods, car-
Differing rates of growth of different parts of diomyocytes in trabecular and compact layers have
the body is termed “allometric growth.” Allometric mostly achieved similar identities;51 the human
growth is a prevalent driver of morphologic change trabecular layer becomes vascularized. Even in
and development in nature. 45 In this regard, contem- humans with excessive trabeculation, trabecular car-
porary observations that indicate a continuous diomyocytes have lost their embryonic identity and
JACC: CARDIOVASCULAR IMAGING, VOL. -, NO. -, 2023 Petersen et al 5
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F I G U R E 2 How Development Shapes LV Wall Morphology

3.5 200
Trabeculations/Compact
Compact
3.0

Amount of Tissue (Layer Area)

150
Trabeculations/Compact

2.5

2.0 Trabeculations
100
1.5

1.0
50

0.5

0.0 0
5

.5

.5

.5

.5

.5

.5

.5

.5

.5

.5

.5
8.

9.

17

19
10

20
14

16
13

15

18
11

12

Mouse Gestational Age in Days

Developmental changes to the proportions of trabecular and compact myocardium (red line) are driven by different rates of positive growth,
not compaction, of the trabeculations (green dots) and compact tissue (blue dots) that vary by gestational age. Such differing rates of
growth of different parts of the body is termed “allometric growth” and is a frequent driver of morphologic change in development and in
nature. The graphs are redrawn based on data from a mouse model.141

are much more akin to those of the compact wall.52,53 Mendelian genes associated with cardiomyopathy,
Experimental models of excessive trabeculation are such as TTN, TNNT2, and PLN. Loci associated with
discussed in the Supplemental Appendix. lower fractal dimension conferred higher risk of
Histological investigations of individuals with dilated cardiomyopathy and heart failure in both
cardiomyopathy and with excessive trabeculation observational and Mendelian randomization ana-
show intramyocardial fibrosis to be a common lyses. This suggests a potential role of normal
finding. 54 The pattern of fibrosis, however, varies trabecular structure in maintaining cardiac output, or
substantially from case to case. 22,55 In this regard, alternatively, that observed associations with certain
direct comparisons made between the car- genetic forms of cardiomyopathy and excessive tra-
diomyocytes of the trabecular layer and their neigh- beculation may be mediated by the roles that these
boring compact cardiomyocytes are largely missing.54 genes have in development.
Greater hypertrophy of the cardiomyocytes in the For patients in whom so-called noncompaction is
trabecular than compact layers, nonetheless, has diagnosed, causative genetic sequence variations are
been reported in explanted hearts. 56 Therefore, evi- reported in approximately one-third of individuals,
dence to date suggests that trabecular and compact although these estimates are confounded by inclu-
cardiomyocytes are similar, although differences may sion bias. Such biases include patient age, family
exist in their responses to different pathologic and screening, heterogeneity in case ascertainment,
physiological processes. variability in content and size of genetic screening
panels, and inconsistent interpretation of the patho-
DETERMINANTS AND ASSOCIATIONS OF genicity of genetic variants. 58 When a genetic cause
EXCESSIVE TRABECULATION was suspected, autosomal dominant transmission
was most frequently reported, although X-linked
GENETIC DETERMINANTS. In the community-based recessive, autosomal recessive, and mitochondrial
UK Biobank CMR study, left ventricular trabecula- inheritance have also been described. 59
tion measured in fractal dimension was observed to Gene sequence variation associated with so-called
have w20% heritability.57 That genome-wide study noncompaction were recently evaluated in several
identified 16 genome-wide loci harboring genes systematic reviews.58,60,61 In determining the rele-
regulating cytoskeletal arborization that associated vance of genetic associations, these studies also
with trabecular complexity. Several loci contained demonstrated the critical importance of case
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ascertainment. For example, left ventricular dilation Neuromuscular disorders of specific or unknown etiol-
and/or systolic dysfunction were present in more ogy, furthermore, were an independent predictor of all-
than three-fifths of the cases studied. Of the included cause death.54,78 Excess mortality was attributed to
individuals, one-tenth had congenital heart disease. rhythm and conduction system disorders, respiratory
Left ventricular hypertrophy was also frequent. 58,60 muscular involvement, higher prevalence of cardiovas-
The mutant genes identified in these patients had cular autonomic dysfunction, and reduced mobility.54,79
also been associated with well-known cardiomyopa- A high prevalence (20%) of cardiomyopathy with
thies and congenital cardiac malformations. excessive trabeculation was reported prospectively in a
In 2 of these recent systematic reviews, 58,60 a wide large cohort of patients with Duchenne and Becker
range of genes encoding sarcomeric, cytoskeletal, types of muscular dystrophy assessed using echocar-
mitochondrial, desmosomal storage, and ion chan- diographic criteria.75
nels, proteins were linked with a phenotype that in- A retrospective analysis of CMR scans of another
cludes excessive trabeculation. 62 Notably, in one of large cohort with Duchenne muscular dystrophy
these studies, abnormalities in MYH7, MYBPC3, showed almost 30% of patients had excessive trabe-
ACTC1, and TTN represented just over two-fifths of culation in at least 1 cardiac segment when using
the identified sequence variations. 58 Similar themes Petersen criteria.1,80 Longitudinal analysis of serial
60
were identified in the other large systematic review. studies on a subgroup of patients with Duchenne
Case ascertainment was variable and incomplete, and muscular dystrophy documented a rate of change in
the associated genes again suggested that cases could the trabecular to compact ratio of þ0.4 per year. 80
represent phenotypic variants of hypertrophic or This reflected both a progressive increase in the
dilated cardiomyopathies. Truncating variants in thickness of the trabecular layer, as well as progres-
MYH7, ACTN2, and PRDM16 were detected in some sive thinning of the compact wall. The investigators
cases, but such truncating variants have not been suggested these observations supported a concept of
identified as causes of more specific cardiomyopa- dystrophin cardiomyopathy as a progressive disease
thies, suggesting that they may associate more spe- characterized by a fragile cytoskeleton, leading to
cifically with excessive trabeculation.60 PRDM16 has worsening left ventricular systolic function and
been shown to be crucial for normal mural develop- compensatory remodeling of the trabecular myocar-
ment in mouse models. 63 dium over time.80,81
Due to the aforementioned considerations,
contemporary guidelines advocate genetic testing ac- PREGNANCY. Reversible excessive trabeculation is

cording to the presence of the features of a conven- known to develop secondarily to increased preload in
tional cardiomyopathy, 64-67 rather than when the a sizeable proportion of individuals who are pregnant
phenotypic feature of excessive trabeculation is inci- with otherwise normal hearts. The feature had usu-
dentally detected in patients who are asymptomatic ally resolved by 12 weeks subsequent to delivery,
with otherwise normal cardiac findings.68 although with some variability in the regression of
the trabecular layer, this being similar to the variation
ASSOCIATIONS WITH NEUROMUSCULAR DISEASE.
in the reduction of the overall left ventricular mass. 5
Excessive trabeculation has been observed in several
African American women were 3 times more likely to
neuromuscular disorders, including specific genetically
69 develop such features during pregnancy than were
determined conditions such as Barth syndrome,
70 71 Caucasian women.5 This is suggestive of a possible
mitochondrial disorders, nuclear envelopathies,
underlying genetic susceptibility in the adaptive
dystrobrevinopathy,72 myotonic dystrophy, zaspop-
73 74 response of the myocardium to volume and pressure
athy, and myoadenylate deaminase deficiency, as
overload.82
well as Duchenne and Becker types of muscular dys-
trophy.75 A causal relationship with the underlying ge- EXERCISE. A reversible phenotype of excessive tra-
netic defects, however, has yet to be established, with beculation has been reported in athletes. This is
genotypic-phenotypic heterogeneity largely unex- recognized as a morphologic epiphenomenon related
plained.54,76 The combination of phenotype and to high cardiac preload demand associated with
neuromuscular disease, nonetheless, may have clinical intensive physical exercise.83 The prevalence of ratios
77
and prognostic implications. In a large, single-center, fulfilling the excessive trabeculation criteria among
prospective study in which excessive trabeculation competitive athletes by echocardiography ranges
was defined using the Stöllberger criteria, almost 80% between 1.4% and 8.1%. This varies according to
of the patients who were neurologically examined were different definitions, ethnicities, and the specific
found to suffer from a neuromuscular disorder.78 sports disciplines.4,84
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In a younger (mean age 48 years), community- altered in inherited cardiomyopathies.96 An alterna-

based cohort of physically active participants tive explanation is that excessive trabeculation de-
involved in the PESA (Progression of Early Subclinical velops or is unmasked by the increase in cardiac
Atherosclerosis) study, which objectively measured preload known to be associated with chronic renal
vigorous recreational physical activity, was associ- failure. 100
ated with higher prevalence of isolated excessive
PUBLISHED REPORTS OF ADVERSE
trabeculation. This was double in those making up
CONSEQUENCES OF CARDIOMYOPATHY
the highest quintile achieving vigorous physical ac-
WITH EXCESSIVE TRABECULATION
tivity compared to those with no vigorous physical
activity.85 Such a relationship between increased
ARRHYTHMIA. Arrhythmias are common in heart
trabeculation in the setting of cardiac adaptation to
failure, in part because of coexistent myocardial
exercise, however, is not consistent.
fibrosis.101 Most individuals who are symptomatic
At the levels of physical activity reported by in-
present with electrocardiographic abnormalities. 102
dividuals who were nonathletic making up the
Although the complex myocardial architecture of
community-based UK Biobank study, there was no
excessive trabeculation might intuitively be linked to
evidence to suggest a dose-response relationship be-
a propensity for re-entrant tachycardias, there is no
tween physical activity intensity and the extent of
evidence to substantiate this. Indeed, after correction
left ventricular trabeculation.86 A prospective study
for confounders such as ventricular dilation, systolic
of 68 novice runners, with a mean age of 28, evalu-
dysfunction, and myocardial fibrosis, excessive tra-
ated before and after completion of a marathon,
beculation does not appear to confer an additional
found no change in the extent of ventricular
arrhythmic risk.103 The ventricular premature beats
trabeculation. 87
noted in patients with excessive trabeculation most
HEMATOLOGICAL DISORDERS. Excessive trabecula- often originate from regions of myocardial scar, or
tion has been described in up to one-sixth of patients from the ventricular outflow tract,104 the latter being
with b -thalassemia, raising the issue of a differential the least trabeculated portion of the ventricle. 105
diagnosis between cardiomyopathy with excessive THROMBUS. There are numerous reports on presence
trabeculation and thalassemic cardiomyopathy.88-91 of thrombus in the trabecular layer of patients with
Echocardiographic data of patients with sickle cell cardiomyopathy.10 However, cohort studies do not
disease and Black control subjects who are healthy support an elevated risk of severe events in the
and asymptomatic revealed a higher prevalence of setting of excessive trabeculation,106 especially when
excessive trabeculation when compared to patients other parameters are taken into account.107,108
who are normotensive.92 Thrombus lodged between trabeculations, nonethe-
Chemotherapy-related cardiac dysfunction is now less, is well documented in symptomatic cases.10,109
also emerging as being potentially associated with Ventricular thrombus is generally associated
excessive trabeculation.93,94 The phenotype has been with cardiomyopathy, myocardial dysfunction, and
interpreted as a myocardial response to drug toxicity, heart failure,110-112 which coexist with excessive
but it may also be a consequence, rather than the trabeculation.
cause, of the cardiac dysfunction.
LEFT VENTRICULAR DYSFUNCTION. Trabeculations
Ultimately, in patients with hemoglobinopathies
have also been suggested to reduce the compliance of
and other chronic hematologic disorders, the finding
the ventricular wall, 113,114 but these hypotheses have
of excessive trabeculation should be interpreted as an
not been tested.37 In otherwise normal hearts, capil-
adaptive response to increased cardiac preload. 95
larization and density of sarcomeres and mitochon-
With the majority of individuals exhibiting excessive
dria are similar in trabeculated and compact
trabeculation having preserved ventricular function,
myocardium. 115,116 Although it is difficult precisely to
it is unlikely to represent an underlying myopathic
replicate the anatomy of the trabecular meshwork,
process.
modeling of left ventricular function has suggested a
RENAL DISORDERS. A number of reports have positive impact of trabeculation on pump function.57
described a possible association between polycystic When measured by ejection fraction, studies of
kidney disease and a cardiomyopathy said to be human cohorts have revealed either no, or very
caused by excessive trabeculation.96-99 It remains to weak, correlations between the extent of trabecular
be elucidated, however, whether the association can myocardium and function.6,19,57 In the UK Biobank,
be explained by a genetic interaction between the a greater fractal dimension was associated with
genes producing polycystic kidney disease and those higher cardiac index. 57 Some uncertainty remains,
8 Petersen et al JACC: CARDIOVASCULAR IMAGING, VOL. -, NO. -, 2023
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F I G U R E 3 Prognosis of CET and DCM

Outcome Incidence Rate (95% CI) P-Value

1.92 (1.54-2.30)
CV death 0.997
1.92 (1.44-2.39)

3.52 (2.95-4.10)
HF admission 0.003
2.37 (1.90-2.85)

2.23 (1.83-2.64)
Malignant VAs 0.779
2.14 (1.66-2.62)

0 1 2 3 4 5
Number of Events / 100 Person-Years
CET DCM

Prognostic comparison between dilated cardiomyopathy (DCM) (green diamond) and cardiomyopathy with excessive trabeculation (CET)
(red diamond) based on previously published meta-analyses.108,123 Because conditions of greater preload and afterload are associated with
excessive trabeculation, excessive trabeculation as a cause of HF admission should not be inferred from meta-analyses. CV ¼ cardiovascular;
HF ¼ heart failure; VA ¼ ventricular arrhythmia.

however, as in MESA, healthy individuals, but who enhancement by CMR.107 In the absence of late gad-
were in the top quartile for the extent of trabecu- olinium enhancement, or evidence of impaired ven-
lation, had slightly reduced circumferential strain tricular function, no hard cardiac events were
when compared to individuals in the lowest recorded.107 A large single-center study of individuals
117
quartile. with excessive trabeculation revealed that survival at
5 years was comparable to an age- and sex-matched
NATURAL HISTORY OF CARDIOMYOPATHY population when left ventricular systolic function
IN THE SETTING OF EXCESSIVE was preserved. 118
TRABECULATION Few studies have compared the prognosis for pa-
tients with cardiomyopathy and excessive trabecula-
Zemrak et al 19 evaluated individuals who were tion to that of other nonischemic cardiomyopathies.
asymptomatic in the MESA study with excessive tra- Among patients with dilated cardiomyopathy, the
beculation over 10 years of follow-up. These in- extent of trabeculation did not influence event-free
vestigators assessed excessive trabeculation as a ratio survival in either unadjusted or adjusted models.120
of compact versus trabecular layers, as well as the An observational study of patients meeting echocar-
extent of trabeculation according to the number of diographic criteria for excessive trabeculation re-
segments. Neither factor was associated with adverse ported more frequent composite cardiovascular
cardiac remodeling.19 In individuals who are asymp- events when compared with age-matched patients
tomatic with excessive trabeculation in MESA, there with dilated cardiomyopathy,121 albeit without
was no relationship between the observed degree of considering the role of potential confounders.
trabeculation and diffuse fibrosis.117 Another study, after multivariable adjustment over a
Multiple studies have identified the presence of median follow-up period of 5 years, did not find any
left ventricular dysfunction as the principal mediator difference in event-free survival rate in idiopathic
for adverse outcomes in the presence of excessive dilated cardiomyopathy vs cardiomyopathy with
trabeculation.103,108,118,119 In these studies, the pooled excessive trabeculation.122 Figure 3 is based on pre-
cardiovascular mortality of individuals with reduced viously published meta-analyses, 108,123 showing that
ejection fraction was twice that of those with normal the pooled event rate of cardiovascular death and
ventricular function. CMR studies using late gado- malignant ventricular arrhythmias was comparable
linium enhancement provide additional prognostic between dilated cardiomyopathy and cardiomyopa-
information. A meta-analysis of patients with exces- thy with excessive trabeculation. A higher incident
sive trabeculation found an increased risk of hard rate of heart failure hospitalization associated
cardiac events in patients with late gadolinium with cardiomyopathy with excessive trabeculation
JACC: CARDIOVASCULAR IMAGING, VOL. -, NO. -, 2023 Petersen et al 9
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F I G U R E 4 Case Report 1

A C

B D

A 63-year-old man presented with a history of nonsustained ventricular tachycardia and paroxysmal atrial fibrillation treated with atrial ablation. CMR was performed
for further evaluation. Cine images at end-diastole in 4-chamber (A) (Video 1) and 2-chamber long-axis views (B) (Video 2) show excessive trabeculation with
biventricular dilation with an EF of 48%. Scattered areas of late gadolinium enhancement were present with a nonischemic pattern (C and D). Genetic testing showed a
MYH7 allelic variant. Stress perfusion cardiac magnetic resonance (not shown) showed diffuse perfusion abnormalities in multiple myocardial segments. Although
excessive trabeculation is present, the presentation of ventricular dilatation, low EF, and nonischemic myocardial scar and genetic abnormality is the same as in
dilated cardiomyopathy. Patient treatment is based on the symptoms and the prognostic risks of arrhythmia, stroke, and contractile impairment. CMR ¼ cardiac
magnetic resonance; EF ¼ ejection fraction.

deserves further evaluation in prospective and impairment and presence of myocardial injury, rather
adequately designed studies. than the extent of trabeculation.
The bulk of current evidence suggests that the
phenotypic feature of excessive trabeculation has no DOES EXCESSIVE TRABECULATION HAVE
independent prognostic relevance in otherwise DIFFERENT IMPLICATIONS FOR CHILDREN
healthy individuals with no clinical suspicion of COMPARED TO ADULTS?
inherited cardiac conditions or symptoms. In patients
with excessive trabeculation and a known cardiomy- Like adults, children with normal ventricular size and
opathy, in contrast, the risk for major adverse clinical function may have excessive trabeculations,
events appears to be associated with the latter and is frequently representing a normal variant. Congenital
apparently independent of the coexisting trabecula- heart defects, such as Ebstein malformation and
tion (Figure 4, Videos 1 and 2). Independent prog- isomerism of the atrial appendages, may coexist
nostic markers include the severity of left ventricular with excessive trabeculation and complicate the
10 Petersen et al JACC: CARDIOVASCULAR IMAGING, VOL. -, NO. -, 2023
Excessive Trabeculation—An Expert Panel Paper - 2023:-–-

picture.124-127 Between these extremes of normal

F I G U R E 5 Case Report 2
variants and overt disease, patients are encountered
for whom the extent of trabeculation is neither
normal nor markedly abnormal. These patients pose
challenges in management, including the uncertain
criteria for instigating metabolic and genetic testing,
the need for antiplatelet therapy, and the frequency
of follow-up (Figures 5 and 6, Videos 3 to 7).

DOES EXCESSIVE TRABECULATION IN

ATHLETES WARRANT A DIFFERENT
APPROACH THAN FOR THE NONATHLETE?

Using echocardiography, the prevalence of exces-

sive trabeculation fulfilling the criterion for recog-
nition as a potential cardiomyopathy among
competitive athletes ranges from around 1%84 to
just under 10%. 4 However when using data avail-
able from forensic registries, no instances of sudden
death in athletes have been directly attributed to
excessive trabeculation.128-133 No adverse cardiac
events, furthermore, have been reported in the in-
dividuals with normal left ventricular function,
regardless of the extent of left ventricular
trabeculation.65
Among athletes, excessive trabeculation is of
concern in individuals who have either left ventric-
ular systolic dysfunction or dilation, cardiac symp-
toms, or abnormal electrocardiographic findings
unrelated to training (Figure 7). Alternatively, they
may have a positive family history of cardiomyopa-
thy. 65 Since 2013, academic centers for sports cardi-
ology in England ceased to investigate athletes who
were asymptomatic with normal cardiac function and
normal electrocardiograms, but with echocardio-
graphic criteria for excessive trabeculation. 133
Athletes with symptoms, abnormal electrocardio-
graphic findings, or a family history of cardiomyopa-
(A) Fetal echocardiogram at 25 weeks in a child with hydrops
thy should undergo cardiopulmonary exercise
fetalis demonstrated severely depressed biventricular systolic
testing, Holter monitoring, and CMR.84,134,135 dysfunction with excessive trabeculation (Video 3). (B)
Balanced atrioventricular remodeling, normal or Supraventricular tachycardia in this fetus developed 1 week
supranormal indices of diastolic function, normal later. Forty-eight hours after delivery, left ventricular EF
longitudinal systolic function, and preserved reduced to 33% (Video 4). (C) Five years later, the same
patient demonstrated progressive left ventricular dilatation
contractility reserve all suggest physiological adap-
with an left ventricular end-diastolic diameter Z score
tation to the intensity and/or frequency of physical of þ2.7 and borderline EF of 50% and global longitudinal
workload. 135-137 The recommendations for competi- strain of 17% (Video 5). The working diagnosis was
tive athletes that have features of cardiomyopathy cardiomyopathy with excessive trabeculation. Neonates and
are not altered by the additional presence of exces- children with excessive trabeculation have been
understudied, with low rates of longitudinal follow-up.
sive trabeculation. 65,138 No data exist to indicate that
Such patients should routinely undergo follow-up, with close
athletes with isolated excessive trabeculation and clinical evaluation and potentially neuromuscular disease
normal myocardial structure and function should be testing. If familial disease is suspected, genetic testing may
routinely disqualified from participation in high- be indicated. Abbreviation as in Figure 4.
intensity exercise and competitive sport.
JACC: CARDIOVASCULAR IMAGING, VOL. -, NO. -, 2023 Petersen et al 11
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F I G U R E 6 Case Report 3

(A) A 3-month-old boy presented with biventricular systolic dysfunction with excessive trabeculation (Video 6). (B) At 6 months of age, this
evolved to a restrictive phenotype requiring placement of a left ventricular assist device (Berlin heart) (Video 7). At 12-months of age
(not shown), the patient underwent orthotopic heart transplantation. The clinical management and significance of excessive trabeculation and
associated disorders in young patients is not well established. As in this example, the evolution of myocardial dysfunction may not be
predictable on baseline examination. For patients with myocardial dysfunction in particular, close clinical follow-up is suggested.
12 Petersen et al JACC: CARDIOVASCULAR IMAGING, VOL. -, NO. -, 2023
Excessive Trabeculation—An Expert Panel Paper - 2023:-–-

F I G U R E 7 Case Report 4

BASELINE ASSESSMENT

A C D

Flow ml/s
B

Holodiastolic flow reversal in the descending aorta

RF 54%; RVol 77 ml

Trigger Time ms

2-YEAR FOLLOW-UP

E F G

Images of a 38-year-old master triathlete with history of catheter ablation for persistent atrial fibrillation. Echocardiography at preparticipation screening documented
the presence of mildly reduced left ventricular systolic function, mild eccentric aortic regurgitation, and a severely dilated LV with excessive trabeculation (positive
Jenni and Chin criteria) that resulted in the diagnosis of so-called left ventricular noncompaction. CMR confirmed the presence of a severely dilated LV with mildly
impaired systolic function and excessive trabeculation (A) according to the Petersen criteria (noncompacted [trabecular] to compact layer ratio: 3.1). Severe eccentric
aortic regurgitation was present (B and C) in addition to a thrombus within the left atrial appendage (A and D). Six months after surgical aortic valve replacement
(E) and left atrial appendage occlusion, significant left ventricular reverse remodeling occurred with near-complete normalization of left ventricular function, volumes,
and diameters (end-diastolic frame [F]; end-systolic frame [G]), further leading to a reduction of Petersen criteria for excessive trabeculation (noncompacted
[trabecular] to compact layer ratio: 2.4). Moderate-to-severe left ventricular dilatation must be carefully investigated in athletes, irrespective of extent of ventricular
trabeculation. If the excessive trabeculation had been part of a cardiomyopathy, arguably, these substantial improvements in left ventricular volumes and function
would not have been observed. RF ¼ regurgitant fraction; RVol ¼ regurgitant volume; other abbreviations as in Figures 1 and 4.
JACC: CARDIOVASCULAR IMAGING, VOL. -, NO. -, 2023 Petersen et al 13
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C ENTR AL I LL U STRA T I O N The Emerging Picture of (Excessive) Left Ventricular Trabeculation

LV Non-Compaction

Excessive Trabeculation

We encourage adoption of the term Excessive trabeculation may be present as a normal variant
Excessive Trabeculation or as a response to increased preload

Clinical management should not be determined In infants and children, less data is available. Consider occult
by the trabecular pattern neuromuscular disease or other genetic/metabolic etiology
Petersen SE, et al. J Am Coll Cardiol Img. 2023;-(-):-–-.

LV ¼ left ventricular.

FUTURE RESEARCH the implications of the trabecular phenotype. As

diagnostic and genetic testing has become more so-
Controversies remain regarding the clinical signifi- phisticated, the underlying etiology of these and
cance of excessive trabeculation in both adults and other cases of cardiomyopathy will increasingly be
children. For adults, patients who are asymptomatic understood. This may result in an ability to make
with excessive trabeculation but normal ventricular more specific etiologic diagnoses as opposed to a
function and normal chamber size appear to require general statement of cardiomyopathy with excessive
little or no long-term follow-up unless other clinical trabeculation. Large, multi-institutional databases of
concerns exist, such as family history or electrocar- patients with cardiomyopathy and excessive trabe-
diogram abnormalities. No prospective cohort or culation would allow tracking of long-term outcomes,
registry data are currently available to validate final diagnoses, and potential treatments. Further
this approach over a 5- or 10-year period. Ongoing research to determine whether cardiomyopathy with
collection of outcomes and covariates (including ge- excessive trabeculation have a different natural his-
netic analysis) in large studies such as UK Biobank tory compared to dilated cardiomyopathy without
may prove beneficial to understanding the indepen- excessive trabeculation may be desirable. Such
dent physiologic impact of excessive trabeculation in studies require careful statistical adjustment for
these otherwise healthy individuals. confounders, thus increasing complexity.
For adults with cardiomyopathies combined with For neonates, children, and adolescents, the
excessive trabeculation, questions remain regarding research focus is somewhat different. Longitudinal
14 Petersen et al JACC: CARDIOVASCULAR IMAGING, VOL. -, NO. -, 2023
Excessive Trabeculation—An Expert Panel Paper - 2023:-–-

cardiomyopathy and/or sudden death. Heritable links

HIGHLIGHTS
between such patients and those with neuromuscular
 Current evidence shows that the extent disorders, other cardiomyopathies, including combi-
of trabeculated vs compact myocardium nations with excessive trabeculation in the adult,
in the adult is determined by differential should be explored to help guide management and
(allometric) growth of each myocardial treatment.
layer. Because trabeculated myocardium
does not coalesce to form the compact CONCLUSIONS
myocardial wall, the term “left ventricu-
lar noncompaction” is inaccurate and use This review summarizes the evidence and un-
should be discouraged. certainties regarding the phenotypic feature of
excessive trabeculation and its potential associa-
 Excessive trabeculation, as diagnosed
tions with cardiomyopathies (Central Illustration).
using existing criteria, may be present as
Because the trabeculated myocardium does not
a normal variant or as a response to pre-
coalesce to form the compact myocardial wall, the
load conditions.
traditional terminology of left ventricular non-
 In adults incidentally found to have compaction should be discouraged. Excessive tra-
excessive trabeculation with normal beculation is frequently detected in the presence of
myocardial function and morphology, features of a heart muscle disorder, but it is also
clinical management is determined by frequently encountered as a normal variation or
other cardiovascular symptoms or ab- develops as a reversible component of physiological
normalities, without regard to trabecular cardiac adaptation. The recognition of a highly tra-
pattern. beculated left ventricle is not known to influence
prognosis nor management in adults. In neonates
 In adults diagnosed with hypertrophic or
and children, caution is warranted because there
dilated cardiomyopathy where excessive
are multiple reports with genetic abnormalities and
trabeculation is also present, the extent
neuromuscular disorders in this vulnerable popula-
of ventricular trabeculation has not been
tion. Future efforts on characterizing the outcomes
demonstrated to alter management nor
and characteristics of those exhibiting excessive
prognosis. Following guidelines devel-
trabeculation are warranted.
oped specifically for those conditions is
recommended. ACKNOWLEDGMENTS Dr Anderson thanks the Hu-
man Developmental Biology Resource for providing
 In infants and children, caution is war- the images of the hearts at gestational weeks 5 and 7
ranted because fewer data are available and Damián Sánchez-Quintana for the image of the
regarding the etiology and prognosis of heart at gestational week 21.
excessive trabeculation. Clinical evalua-
tion for occult neuromuscular disease or FUNDING SUPPORT AND AUTHOR DISCLOSURES
another genetic/metabolic etiology may
Dr Petersen has received support from the National Institute for
be appropriate.
Health and Care Research Barts Biomedical Research Centre; and has
consulted with Circle Cardiovascular Imaging Inc. Dr Friedrich has
received support from the McGill Health Centre Foundation; has
served as an advisor to and is a shareholder of Circle Cardiovascular
studies of the early development of the myocardium
Imaging Inc; and is a founder and shareholder of Area19 Medical Inc.
may shed light on the origin of varying phenotypes. All other authors have reported that they have no relationships
Severe malformations of the trabecular layer, poorly relevant to the contents of this paper to disclose.

developed left ventricular papillary muscles, along

with frequent congenital heart disease and sudden ADDRESS FOR CORRESPONDENCE: Prof Steffen
death have been suggested as a distinct cardiomy- Petersen, William Harvey Research Institute, National
opathy in neonates and young children. 22 Although Institute for Health and Care Research Barts
very rare in the older child or adult, improved un- Biomedical Research Centre, Queen Mary University
derstanding of any relationship to adult-type exces- of London, Charterhouse Square, London EC1M 6BQ,
sive trabeculation is important to understand risk of United Kingdom. E-mail: [email protected].
JACC: CARDIOVASCULAR IMAGING, VOL. -, NO. -, 2023 Petersen et al 15
- 2023:-–- Excessive Trabeculation—An Expert Panel Paper

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KEY WORDS cardiovascular imaging,
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138. Maron BJ, Udelson JE, Bonow RO, et al. 140. Captur G, Muthurangu V, Cook C, et al. AP PE NDIX For an expanded discussion
Eligibility and disqualification recommendations Quantification of left ventricular trabeculae using of experimental models as well as a supple-
for competitive athletes with cardiovascular ab- fractal analysis. J Cardiovasc Magn Reson. mental figure, table, and references please see
normalities: Task Force 3: Hypertrophic Cardio- 2013;15(1):36. the online version of this paper.