Diagnostic Challenges and Proposed Diagnostic Algorithm Flashcards
(55 cards)
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
