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Noncompaction Cardiomyopathy > Diagnostic Challenges and Proposed Diagnostic Algorithm > Flashcards

Diagnostic Challenges and Proposed Diagnostic Algorithm Flashcards

1
Q

What are the two proposals for classification of NCCM?

A

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.

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2
Q

Why is appropriate clinical diagnosis important in NCCM?

A

It is crucial for the clinical management of individual patients due to differing therapeutic and prognostic strategies.

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3
Q

What are the minimal requirements for diagnostic tools to establish NCCM diagnosis?

A

A comprehensive cardiovascular examination is recommended.

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4
Q

What is the primary imaging modality for diagnosing NCCM?

A

Echocardiography

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5
Q

What is the significance of a thorough personal and familial history in NCCM diagnosis?

A

It helps identify potential genetic links and risk factors.

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6
Q

Which patient group may present a benign form of excess trabeculation?

A

Athletes

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7
Q

List key tools required for establishing an accurate NCCM diagnosis.

A
  • History and physical examination
  • ECG
  • Holter monitoring
  • Exercise stress test
  • Echocardiography
  • Cardiac MRI
  • Cardiac CT
  • Angiography
  • Positron Emission Tomography (PET)
  • Genetic testing
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8
Q

True or False: Nearly 90% of children and adults with NCCM have abnormal ECG findings.

A

True

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9
Q

What are the five key morphologic features of NCCM?

A
  • 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
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10
Q

What is the California criteria for diagnosing NCCM?

A

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.

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11
Q

What do the Zurich criteria require for diagnosing IVNC?

A
  • 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
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12
Q

What is the focus of the Vienna criteria in diagnosing NCCM?

A

Number of LV trabeculations

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13
Q

What does the New York criteria focus on in assessing NCCM severity?

A

Thickness and area of noncompacted regions.

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14
Q

What are the German Criteria for NCCM diagnosis?

A
  • 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
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15
Q

What is the purpose of the Zurich modified Criteria?

A

To prevent overdiagnosis of NCCM with a specific focus on compacted thickness.

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16
Q

What is the Wisconsin Criteria based on?

A

End-diastole ratio of NC to compacted myocardium > 2 on parasternal short-axis view.

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17
Q

What is the Rotterdam Criteria used for?

A

To classify the severity of NCCM based on echocardiographic measurements.

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18
Q

What is the Wisconsin criterion for diagnosing NCCM?

A

End-diastole: ratio of noncompacted (NC) to compacted (C) myocardium = 3.0

Indicates absence of radial thickening of NC myocardium

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19
Q

What is the Jenni criterion for diagnosing NCCM?

A

End-systole: NC/C = 2.7

This ratio is lower than the end-diastolic ratio due to radial thickening of the C layer

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20
Q

What does the Chin criterion measure for NCCM?

A

End-diastole: compacted myocardium (X) / (compacted plus noncompacted myocardium (Y) = 0.22

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21
Q

What defines LVNC according to the Stollberger criteria?

A

Trabeculations (four or more) protruding from the LV wall, located apically to the papillary muscles and visible in one imaging plane

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22
Q

What is the main limitation of echocardiographic criteria?

A

Dependence on acoustic window and endocardial border definition

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23
Q

What is the Rotterdam criteria for NCCM diagnosis?

A

Combination of cardiac symptoms, abnormal ECG, and/or family history with structural or functional abnormalities

Includes criteria for diagnosing NCCM ‘trait’ or LVNC

24
Q

What are the echocardiographic features indicative of NCCM?

A
  • 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
25
Q

What does the California criteria specify for NCCM?

A

NC to C ratio ≥2 on the parasternal short axis view at end-diastole

26
Q

What are the advanced echocardiographic diagnostic tools mentioned?

A
  • Contrast echocardiography
  • Tissue Doppler Imaging (TDI)
  • Speckle tracking echocardiography
  • Three-dimensional echocardiography
27
Q

How does Tissue Doppler Imaging (TDI) assist in NCCM diagnosis?

A

Quantifies regional and global myocardial velocity and strain

28
Q

What is the role of MRI in NCCM assessment?

A

Used as a second-line tool if echocardiography is non-diagnostic, providing excellent tissue-blood contrast

29
Q

What MRI criterion did Petersen et al. propose for NCCM?

A

End-diastolic ratio of compacted to noncompacted myocardium >2.3

30
Q

What is a limitation of MRI criteria for NCCM?

A

Derived from small, selected populations and related to slice selection and partial volume effects

31
Q

What is the significance of contrast echocardiography in NCCM?

A

Provides better endocardial border delineation in patients with NCCM

32
Q

What can impaired LV rotation and twisting indicate in NCCM patients?

A

May accurately discriminate patients with NCCM from healthy controls

33
Q

Fill in the blank: The _______ criteria propose that there are at least 4 prominent trabeculae and deep intertrabecular recesses.

A

German

34
Q

True or False: The echocardiographic features of NCCM include septal hypertrophy greater than 12mm.

A

False

35
Q

What is the maximum NC to C ratio proposed by Jacquier et al. for NCCM diagnosis?

A

Trabeculated NC mass >20% of the global LV mass

36
Q

What does the speckle tracking echocardiography reveal about NCCM?

A

Impairment of LV strain, rotation, and twist is related to NCCM severity

37
Q

What does NCCM stand for?

A

Noncompaction Cardiomyopathy

38
Q

What imaging technique was used by Dawson et al. to study normal LV trabeculation versus pathological noncompaction?

A

Cardiac MRI

39
Q

What is the significance of a noncompaction ratio of >2 in the diagnosis of NCCM?

A

It indicates potential NCCM diagnosis

40
Q

What percentage of participants in Weir-McCall et al.’s study met at least one diagnostic criterion for NCCM?

A

14.8%

41
Q

Which MRI criteria for diagnosing NCCM showed the most specificity?

A

Noncompacted to compacted myocardial mass ratio

42
Q

What is the proposed NC/C ratio cut-off to distinguish NCCM from other cardiomyopathies?

A

2.2

43
Q

What are the four steps in the proposed diagnostic algorithm for NCCM?

A
  • Assess clinical presentation
  • Assess trabeculations as a continuum
  • Assess extent and localization of noncompacted segments
  • Assign an LVNC/NCCM subtype
44
Q

True or False: The prevalence of NCCM among adults is reported as 0.014–0.26%.

A

True

45
Q

What are some normal variants that can be mistaken for pathological trabeculations?

A
  • False tendons
  • Multiple bellies of papillary muscles
  • Additional papillary muscles
46
Q

What is the main dilemma in diagnosing NCCM?

A

Distinguishing normal variants from pathologic trabeculations

47
Q

The study by Andreini et al. found that severity of trabeculations has less prognostic significance than what parameters?

A
  • LV dilation
  • Systolic dysfunction
  • Fibrosis
48
Q

What imaging challenges are associated with deep trabeculations in NCCM?

A

Difficulties in tracing the true endocardium

49
Q

Fill in the blank: The prevalence of NCCM varies based on the _______ applied.

A

[criteria]

50
Q

What is a key practical aspect of NCCM diagnosis regarding imaging criteria?

A

Poor reproducibility

51
Q

What is the significance of a NC/C ratio in end-systole less than 1.0?

A

Indicates normal trabeculation

52
Q

What percentage of patients with dilated cardiomyopathy exhibited a hypertrabeculation pattern resembling NCCM?

A

43%

53
Q

What does the acronym AHA stand for in the context of NCCM guidelines?

A

American Heart Association

54
Q

What does the acronym ESC stand for in the context of NCCM guidelines?

A

European Society of Cardiology

55
Q

What is the relationship between NCCM and genetic cardiomyopathy?

A

NCCM is predominantly a genetic cardiomyopathy

Noncompaction Cardiomyopathy (9 decks)

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