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Assessing for Noncompaction
Cardiomyopathy.
Is it Too Often Diagnosed?
Soheila Talebi.MD
Cardiologist / Cardiovascular imaging
Assistant Professor of Medicine
Mount Sinai St. Luke's
No Disclosures
Cardiomyopathy
Cardiomyopathies represent a group of diseases of the
myocardium with adverse effects on the heart muscle size,
shape, and function.
Traditionally cardiomyopathies were defined according to
phenotypical appearance.
Non compaction Cardiomyopathy
(NCCM)
The traditional classifications seem over simplistic
An increased awareness of this myocardial phenotype & the high quality of advanced cardiovascular imaging have a potential for :
Overdiagnosis
Overtreatment
Unnecessary follow-up
Incorporate the morphologic findings with molecular and genetic information.
Physiologic Reversible Remodeling
Sources: https://giphy.com/gifs/running-run-walk-5UDsrMd6ctz6KDXyIo and https://giphy.com/gifs/feels-really-pregnancy-EvSiDRE8oO4PC
Ethnic
Ethnic subgroups, in particular black individuals
who appear to have more pronounced
trabeculation, and are therefore more likely to
be mislabeled
Trabeculated (Non-Compacted) and Compact Myocardium in Adults: The Multi-Ethnic Study of Atherosclerosis
Pathologic remodeling
Hypertrophy of the myocardium does not
automatically imply hypertrophic cardiomyopathy.
As volume or pressure load results in hypertrophy of
the myocardium, it can also result in the
morphological appearance of LVNC, which is
different from NCCM
Failure of proliferation of
compact wall rather than
failure compaction of the
pre-existing trabecular layer
Genetic
Genetics play an important role in NCCM because 17% to 50% of patients have a family member with cardiomyopathy
In most families, an autosomal dominant pattern of inheritance is observed with variable penetrance
Sarcomere gene mutations are the most common identified and most of the genes involved are also implicated in hypertrophic and dilated CM.
Clinical manifestations include :
Clinical symptoms range from severe prenatal
manifestations to asymptomatic cardiomyopathy
presenting in adults
LV dysfunction, congestive heart failure
Ventricular tachycardia
Sudden cardiac death,
Thromboembolic complications
Role Of Imaging
Imaging has a fundamental role in the
diagnosis of LVNC such as :
Echocardiography
Cardiac MRI
CT Scan
Advantages of cardiac MRI
Cine imaging performed in different cardiac planes well
depicts the two-layered myocardium
MRI better shows myocardial trabeculae at end-diastole.
The extent of myocardial trabeculations can be accurately
assessed.
Cardiac MRI should be performed to confirm the diagnosis
of LVNC
Cardiac MRI
An end-diastolic ratio between noncompacted and compacted
layers greater than 2.3 is considered diagnostic of myocardial
noncompaction.
A thin compacted layer is a prerequisite for the diagnosis. The
maximal compacted thickness of < 8 mm was proposed as a
specific marker and prevent overdiagnosis
Cardiac MRI
Noncompacted myocardium should be measured in a plane
perpendicular to the compacted myocardium.
Generally use short-axis images to measure compacted and
noncompacted segments in the mid-cavity and basal
segments.
Cardiac MRI
When myocardial trabeculations are located principally at the
apex, short-axis images may not be perpendicular to the LV long
axis.
In this case four-chamber views to assess noncompaction-to-
compaction ratio is recommended
Late Gadolinium Enhancement
Erwin Oechslin, and Rolf Jenni JACC 2018;71:723-726
2018 American College of Cardiology Foundation
Key points
NCCM cardiomyopathy should not be defined only by Morphology
Physiologic remodeling & Pathologic remodeling
Prominent trabeculation is seen in some ethnic subgroup
In imaging report the term “noncompaction cardiomyopathy” should be replaced with a description of the trabeculations, along with risk factors such as ejection fraction, abnormal myocardial enhancement and LV dilation
Healthy subject not be labeled as a patient with LVNC, with a
life-long impact on this individual and on the health care
system
The critical judgment of a clinician must determine the
individual care plan, because the myocardial morphologic
phenotype is only 1 piece of the diagnostic puzzle