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Noncompaction Cardiomyopathy > Congenital Heart Disease and Noncompaction Cardiomyopathy > Flashcards

Congenital Heart Disease and Noncompaction Cardiomyopathy Flashcards

1
Q

What is noncompaction cardiomyopathy (NCCM)?

A

A genetic cardiomyopathy characterized by an abnormal structure of the heart muscle, often diagnosed in adulthood

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2
Q

What is the prevalence of NCCM?

A

Approximately 0.14%

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3
Q

Which congenital heart disease is most commonly associated with NCCM?

A

Ebstein anomaly

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4
Q

What are common congenital heart diseases associated with NCCM?

A
  • Ebstein anomaly
  • Aortic coarctation
  • Tetralogy of Fallot
  • Bicuspid aortic valve
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5
Q

What diagnostic methods are used for NCCM?

A
  • Transthoracic echocardiography
  • Cardiac MRI
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6
Q

What is the diagnostic ratio for NCCM using echocardiography?

A

Greater than 2:1 at the end of diastole

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7
Q

True or False: NCCM can be present from birth.

A

True

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8
Q

What is Ebstein anomaly?

A

A malformation of the tricuspid valve characterized by the displacement of the tricuspid leaflets

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9
Q

What complications are associated with Ebstein anomaly?

A
  • Cyanosis
  • Right-sided heart failure
  • Arrhythmias
  • Sudden cardiac death
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10
Q

What are the common clinical presentations of NCCM?

A
  • Heart failure
  • Supraventricular arrhythmias
  • Ventricular arrhythmias
  • Sudden cardiac death
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11
Q

What are common treatments for NCCM?

A
  • Beta-blockers
  • Angiotensin-converting enzyme (ACE) inhibitors
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12
Q

What is the recommended follow-up for asymptomatic patients with preserved LV function?

A

Annual or biannual cardiologic follow-up

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13
Q

Fill in the blank: The main diagnostic modality for Ebstein anomaly is _______.

A

Transthoracic echocardiography

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14
Q

What is the risk associated with patients having both Bicuspid aortic valve (BAV) and NCCM?

A

Increased risk for heart failure, arrhythmias, thromboembolic events, and sudden cardiac death

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15
Q

What is the relationship between sarcomere gene mutations and NCCM?

A

Similar mutations found in patients with Ebstein anomaly suggest a genetic predisposition

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16
Q

What is pseudo-NCCM?

A

Heavily trabeculated myocardium that can be confused with NCCM, such as in congenitally corrected transposition of the great arteries

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17
Q

What is the significance of longitudinal studies on unaffected carriers of pathogenic mutations?

A

To provide insight into whether noncompaction may develop later in life

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18
Q

What are the challenges faced by patients with congenital heart disease (CHD) and NCCM?

A

Multiple previous surgical interventions and potential for heart failure and life-threatening arrhythmias

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19
Q

What is the recommended approach for anticoagulation therapy in NCCM?

A

Advised only in patients with ejection fraction less than 40%, atrial fibrillation, or previous thrombo-embolic events

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20
Q

True or False: Thromboembolic events are common in NCCM.

A

False

21
Q

What is the importance of awareness among clinicians regarding NCCM?

A

To ensure early diagnosis and management in patients with congenital heart disease

22
Q

What does the term ‘cardiac morphogenesis’ refer to?

A

The development of the heart’s structure during fetal growth, potentially influenced by sarcomere defects

23
Q

What is the recommended diagnostic ratio for NCCM using MRI?

A

Greater than 2.3 at the end of diastole

24
Q

What is the incidence of Ebstein anomaly?

A

Approximately 1 in 200,000 live births

25
Q

What is the significance of the MYH7 mutation in patients with NCCM?

A

It has been reported in sporadic patients and families with NCCM and Ebstein anomaly

26
Q

Which congenital heart disease is most commonly associated with NCCM?

A

Ebstein anomaly

27
Q

What are common congenital heart diseases associated with NCCM?

A
  • Ebstein anomaly
  • Aortic coarctation
  • Tetralogy of Fallot
  • Bicuspid aortic valve
28
Q

What diagnostic methods are used for NCCM?

A
  • Transthoracic echocardiography
  • Cardiac MRI
29
Q

What is the diagnostic ratio for NCCM using echocardiography?

A

Greater than 2:1 at the end of diastole

30
Q

True or False: NCCM can be present from birth.

A

True

31
Q

What is Ebstein anomaly?

A

A malformation of the tricuspid valve characterized by the displacement of the tricuspid leaflets

32
Q

What complications are associated with Ebstein anomaly?

A
  • Cyanosis
  • Right-sided heart failure
  • Arrhythmias
  • Sudden cardiac death
33
Q

What are the common clinical presentations of NCCM?

A
  • Heart failure
  • Supraventricular arrhythmias
  • Ventricular arrhythmias
  • Sudden cardiac death
34
Q

What are common treatments for NCCM?

A
  • Beta-blockers
  • Angiotensin-converting enzyme (ACE) inhibitors
35
Q

What is the recommended follow-up for asymptomatic patients with preserved LV function?

A

Annual or biannual cardiologic follow-up

36
Q

Fill in the blank: The main diagnostic modality for Ebstein anomaly is _______.

A

Transthoracic echocardiography

37
Q

What is the risk associated with patients having both Bicuspid aortic valve (BAV) and NCCM?

A

Increased risk for heart failure, arrhythmias, thromboembolic events, and sudden cardiac death

38
Q

What is the relationship between sarcomere gene mutations and NCCM?

A

Similar mutations found in patients with Ebstein anomaly suggest a genetic predisposition

39
Q

What is pseudo-NCCM?

A

Heavily trabeculated myocardium that can be confused with NCCM, such as in congenitally corrected transposition of the great arteries

40
Q

What is the significance of longitudinal studies on unaffected carriers of pathogenic mutations?

A

To provide insight into whether noncompaction may develop later in life

41
Q

What are the challenges faced by patients with congenital heart disease (CHD) and NCCM?

A

Multiple previous surgical interventions and potential for heart failure and life-threatening arrhythmias

42
Q

What is the recommended approach for anticoagulation therapy in NCCM?

A

Advised only in patients with ejection fraction less than 40%, atrial fibrillation, or previous thrombo-embolic events

43
Q

True or False: Thromboembolic events are common in NCCM.

A

False

44
Q

What is the importance of awareness among clinicians regarding NCCM?

A

To ensure early diagnosis and management in patients with congenital heart disease

45
Q

What does the term ‘cardiac morphogenesis’ refer to?

A

The development of the heart’s structure during fetal growth, potentially influenced by sarcomere defects

46
Q

What is the recommended diagnostic ratio for NCCM using MRI?

A

Greater than 2.3 at the end of diastole

47
Q

What is the incidence of Ebstein anomaly?

A

Approximately 1 in 200,000 live births

48
Q

What is the significance of the MYH7 mutation in patients with NCCM?

A

It has been reported in sporadic patients and families with NCCM and Ebstein anomaly

Noncompaction Cardiomyopathy (9 decks)

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