Diagnostic Challenges and Proposed Diagnostic Algorithm Flashcards
What are the two proposals for classification of NCCM?
2006 American Heart Association (AHA) and 2008 European Society of Cardiology (ESC) classifications
AHA considers NCCM a distinct primary genetic cardiomyopathy, while ESC questions its classification as a separate cardiomyopathy.
Why is appropriate clinical diagnosis important in NCCM?
It is crucial for the clinical management of individual patients due to differing therapeutic and prognostic strategies.
What are the minimal requirements for diagnostic tools to establish NCCM diagnosis?
A comprehensive cardiovascular examination is recommended.
What is the primary imaging modality for diagnosing NCCM?
Echocardiography
What is the significance of a thorough personal and familial history in NCCM diagnosis?
It helps identify potential genetic links and risk factors.
Which patient group may present a benign form of excess trabeculation?
Athletes
List key tools required for establishing an accurate NCCM diagnosis.
- History and physical examination
- ECG
- Holter monitoring
- Exercise stress test
- Echocardiography
- Cardiac MRI
- Cardiac CT
- Angiography
- Positron Emission Tomography (PET)
- Genetic testing
True or False: Nearly 90% of children and adults with NCCM have abnormal ECG findings.
True
What are the five key morphologic features of NCCM?
- Presence of a two-layered myocardial structure
- Increased NC to C layer ratio
- Evidence of intertrabecular recesses communication with LV cavity
- Absence of other congenital or acquired heart disease
- Preferential location and distribution of excess trabeculations
What is the California criteria for diagnosing NCCM?
Defined by the presence of X/Y < 0.5 at end-diastole in the parasternal short-axis view, where X is the distance from the epicardial surface to the trabecular recess and Y is the distance to the peak of trabeculation.
What do the Zurich criteria require for diagnosing IVNC?
- Absence of coexisting cardiac abnormalities
- A two-layered structure
- Maximal end-systolic NC to C ratio > 2
- Color Doppler evidence of deep perfused intertrabecular recesses
What is the focus of the Vienna criteria in diagnosing NCCM?
Number of LV trabeculations
What does the New York criteria focus on in assessing NCCM severity?
Thickness and area of noncompacted regions.
What are the German Criteria for NCCM diagnosis?
- At least 4 prominent trabeculae
- Demonstrable blood flow between LV cavity and recesses
- 2-layered myocardial structure with NC to C ratio ≥ 2
- No other cardiac abnormalities
What is the purpose of the Zurich modified Criteria?
To prevent overdiagnosis of NCCM with a specific focus on compacted thickness.
What is the Wisconsin Criteria based on?
End-diastole ratio of NC to compacted myocardium > 2 on parasternal short-axis view.
What is the Rotterdam Criteria used for?
To classify the severity of NCCM based on echocardiographic measurements.
What is the Wisconsin criterion for diagnosing NCCM?
End-diastole: ratio of noncompacted (NC) to compacted (C) myocardium = 3.0
Indicates absence of radial thickening of NC myocardium
What is the Jenni criterion for diagnosing NCCM?
End-systole: NC/C = 2.7
This ratio is lower than the end-diastolic ratio due to radial thickening of the C layer
What does the Chin criterion measure for NCCM?
End-diastole: compacted myocardium (X) / (compacted plus noncompacted myocardium (Y) = 0.22
What defines LVNC according to the Stollberger criteria?
Trabeculations (four or more) protruding from the LV wall, located apically to the papillary muscles and visible in one imaging plane
What is the main limitation of echocardiographic criteria?
Dependence on acoustic window and endocardial border definition
What is the Rotterdam criteria for NCCM diagnosis?
Combination of cardiac symptoms, abnormal ECG, and/or family history with structural or functional abnormalities
Includes criteria for diagnosing NCCM ‘trait’ or LVNC
What are the echocardiographic features indicative of NCCM?
- Abnormal segmental myocardial thickening of LV or RV
- NC/C ratio ≥2 in PSLX end-systole
- No septal hypertrophy (<12mm)
- Global or segmental cardiac dysfunction
What does the California criteria specify for NCCM?
NC to C ratio ≥2 on the parasternal short axis view at end-diastole
What are the advanced echocardiographic diagnostic tools mentioned?
- Contrast echocardiography
- Tissue Doppler Imaging (TDI)
- Speckle tracking echocardiography
- Three-dimensional echocardiography
How does Tissue Doppler Imaging (TDI) assist in NCCM diagnosis?
Quantifies regional and global myocardial velocity and strain
What is the role of MRI in NCCM assessment?
Used as a second-line tool if echocardiography is non-diagnostic, providing excellent tissue-blood contrast
What MRI criterion did Petersen et al. propose for NCCM?
End-diastolic ratio of compacted to noncompacted myocardium >2.3
What is a limitation of MRI criteria for NCCM?
Derived from small, selected populations and related to slice selection and partial volume effects
What is the significance of contrast echocardiography in NCCM?
Provides better endocardial border delineation in patients with NCCM
What can impaired LV rotation and twisting indicate in NCCM patients?
May accurately discriminate patients with NCCM from healthy controls
Fill in the blank: The _______ criteria propose that there are at least 4 prominent trabeculae and deep intertrabecular recesses.
German
True or False: The echocardiographic features of NCCM include septal hypertrophy greater than 12mm.
False
What is the maximum NC to C ratio proposed by Jacquier et al. for NCCM diagnosis?
Trabeculated NC mass >20% of the global LV mass
What does the speckle tracking echocardiography reveal about NCCM?
Impairment of LV strain, rotation, and twist is related to NCCM severity
What does NCCM stand for?
Noncompaction Cardiomyopathy
What imaging technique was used by Dawson et al. to study normal LV trabeculation versus pathological noncompaction?
Cardiac MRI
What is the significance of a noncompaction ratio of >2 in the diagnosis of NCCM?
It indicates potential NCCM diagnosis
What percentage of participants in Weir-McCall et al.’s study met at least one diagnostic criterion for NCCM?
14.8%
Which MRI criteria for diagnosing NCCM showed the most specificity?
Noncompacted to compacted myocardial mass ratio
What is the proposed NC/C ratio cut-off to distinguish NCCM from other cardiomyopathies?
2.2
What are the four steps in the proposed diagnostic algorithm for NCCM?
- Assess clinical presentation
- Assess trabeculations as a continuum
- Assess extent and localization of noncompacted segments
- Assign an LVNC/NCCM subtype
True or False: The prevalence of NCCM among adults is reported as 0.014–0.26%.
True
What are some normal variants that can be mistaken for pathological trabeculations?
- False tendons
- Multiple bellies of papillary muscles
- Additional papillary muscles
What is the main dilemma in diagnosing NCCM?
Distinguishing normal variants from pathologic trabeculations
The study by Andreini et al. found that severity of trabeculations has less prognostic significance than what parameters?
- LV dilation
- Systolic dysfunction
- Fibrosis
What imaging challenges are associated with deep trabeculations in NCCM?
Difficulties in tracing the true endocardium
Fill in the blank: The prevalence of NCCM varies based on the _______ applied.
[criteria]
What is a key practical aspect of NCCM diagnosis regarding imaging criteria?
Poor reproducibility
What is the significance of a NC/C ratio in end-systole less than 1.0?
Indicates normal trabeculation
What percentage of patients with dilated cardiomyopathy exhibited a hypertrabeculation pattern resembling NCCM?
43%
What does the acronym AHA stand for in the context of NCCM guidelines?
American Heart Association
What does the acronym ESC stand for in the context of NCCM guidelines?
European Society of Cardiology
What is the relationship between NCCM and genetic cardiomyopathy?
NCCM is predominantly a genetic cardiomyopathy
