Historical Perspective Flashcards

1
Q

What is the connection between imaging modalities and the awareness of newly detected morphologic abnormalities?

A

Better understanding and increased awareness are mostly connected to the progress in adequate imaging modalities.

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2
Q

What breakthrough in diagnosis began with echocardiographic findings?

A

The echocardiographic diagnosis of spongy myocardial morphology.

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3
Q

In which patient population was spongy myocardial morphology initially observed?

A

Newborns and infants.

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4
Q

What has improved the detection of spongy myocardial patterns in apparently healthy individuals?

A

Advancements in cardiovascular imaging quality, including modern echo, MRI, and CT modalities.

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5
Q

What is necessary to prevent mis- or over-diagnosis of cardiac anomalies?

A

Improved diagnostic criteria.

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6
Q

What historical context is essential to understand current concepts in diagnosis and management?

A

The historical background of the cardiac anomaly.

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7
Q

What phenomenon did Lauer et al. observe in cases with pulmonary valve atresia?

A

Intramyocardial diverticula and sinusoids.

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8
Q

What did Feldt et al. describe in a 3-month-old girl with complex congenital heart disease?

A

A two-layered myocardial structure with a thick spongy inner layer and a compact outer layer.

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9
Q

What term did Chin et al. propose for the abnormal myocardial pattern?

A

Isolated noncompaction of left ventricular myocardium.

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10
Q

What does the process of ventricular trabeculation begin with?

A

Protrusions of the endocardial layer developing into myocardial trabeculations.

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11
Q

What signaling molecules are involved in cardiac trabeculation?

A

Neuregulin, serotonin 2B receptor, vascular endothelial growth factor, angiopoietin-1.

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12
Q

What is the expected outcome of the heart’s compaction process by gestational week 16?

A

Compression of intertrabecular spaces to capillaries.

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13
Q

What is the relationship between the compaction process and the development of coronary circulation?

A

The compaction process is accompanied by the formation of coronary veins and arteries.

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14
Q

What rotation pattern occurs in the left ventricular myocardium during systole?

A

Clockwise basal rotation followed by counterclockwise apical rotation.

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15
Q

True or False: In cases of left ventricular noncompaction, systolic twisting deformation is present.

A

False.

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16
Q

What does the term ‘left ventricular noncompaction’ (LVNC) reflect?

A

A typical myocardial pattern on echocardiography.

17
Q

What are the potential causes of noncompaction aside from genetic failure?

A

Acquired forms due to conditions like vigorous training and pregnancy.

18
Q

What alternative term may be more appropriate than ‘LVNC’ according to recent observations?

A

Left ventricular hypertrabeculation (LVHT).

19
Q

What does the term ‘excessive trabeculations’ refer to?

A

A myocardial phenotype of unknown origin or a bystander in the presence of additional cardiac lesions.

20
Q

What does LVNC stand for?

A

Left Ventricular Noncompaction

LVNC is characterized by a distinct myocardial pattern observed on echocardiography.

21
Q

What are the key echocardiographic features of LVNC?

A

Typical myocardial pattern with:
* Subepicardially located compacted layer
* Subendocardial noncompacted layer
* At least 4 prominent left ventricular trabeculations
* Deep intertrabecular recesses

22
Q

Is LVNC considered a phenotype or an established cardiomyopathy?

A

Debated; it may be considered a phenotype rather than an established cardiomyopathy.

23
Q

What factors are important in distinguishing genetic NCCM from non-genetic LVNC?

A

Genetic testing plays a crucial role in this distinction.

24
Q

What is the significance of the compacted and noncompacted layers in LVNC?

A

The noncompacted layer should be at least twice as thick as the compacted layer.

25
Q

What diagnostic criteria may indicate NCCM?

A

Criteria include:
* Isolated LVNC with systolic/diastolic left ventricular dysfunction
* Abnormal ECG findings
* Symptoms of heart failure
* Family history of NCCM

26
Q

What symptoms were present in the first published case of isolated NCCM?

A

Palpitations and exertional dyspnea.

27
Q

What diagnostic method is preferred for NCCM?

A

Echocardiography is the method of choice.

28
Q

True or False: Children with NCCM typically present with milder symptoms than adults.

A

False; they often present with severe symptoms and poor prognosis.

29
Q

Fill in the blank: The first published case with isolated NCCM involved a _____ year old woman.

A

33

30
Q

What does NCCM stand for?

A

Noncompaction Cardiomyopathy

31
Q

What is a common complication associated with NCCM?

A

Thromboembolism may be present.

32
Q

What was unique about the left ventricular walls in the first published case of isolated NCCM?

A

Huge and deep spaces within the left ventricular wall were observed.

33
Q

What role does genetic testing play in the management of LVNC and NCCM?

A

It may help distinguish between physiologic and pathologic remodeling.

34
Q

What imaging techniques may supplement echocardiography in diagnosing NCCM?

A

Cardiac MRI and other diagnostic tools.

35
Q

What is a common ECG finding in patients with NCCM?

A

Complete left bundle branch block.

36
Q

What future research direction is suggested for understanding LVNC and NCCM?

A

Combined data on genetic findings, clinical presentation, and myocardial performance will improve risk stratification.

37
Q

What historical significance does the study of LVNC and NCCM hold?

A

Understanding the historical background is essential for grasping current diagnostic and management concepts.