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Noncompaction Cardiomyopathy > Malignant Arrhythmias and Sudden Cardiac Death > Flashcards

Malignant Arrhythmias and Sudden Cardiac Death Flashcards

1
Q

What is Noncompaction Cardiomyopathy (NCCM)?

A

A distinct primary genetic cardiomyopathy recognized by the AHA and classified as unclassified cardiomyopathy by the ESC.

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2
Q

What are the key complications associated with NCCM?

A
  • Chronic heart failure
  • Lethal arrhythmias
  • Thromboembolic events
  • Sudden cardiac death (SCD)
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3
Q

What is the reported mortality rate in adults with NCCM?

A

18%

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4
Q

What is the reported mortality rate in children with NCCM?

A

0–13%

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5
Q

How is Sudden Cardiac Death (SCD) defined?

A

Death from cardiac causes with an abrupt loss of consciousness less than 1 hour after onset of symptoms.

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6
Q

What percentage of sudden cardiac deaths are attributed to coronary artery disease?

A

65–70%

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7
Q

In NCCM patients, what are the primary causes of sudden cardiac death?

A
  • Ventricular tachycardia (VT)
  • Ventricular fibrillation (VF)
  • Asystole
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8
Q

What is the most effective therapy to prevent sudden death in NCCM patients?

A

Implantable cardioverter defibrillator (ICD)

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9
Q

What is the incidence of NCCM in infants?

A

0.80 per 100,000 individuals per year

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10
Q

What is the incidence of NCCM in adults?

A

0.05%

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11
Q

What arrhythmias are commonly associated with NCCM?

A
  • Bundle branch reentry
  • Idiopathic VT
  • Right ventricular outflow tract origin
  • Left bundle branch and right bundle branch morphologies
  • Bidirectional and fascicular VT
  • Polymorphic VT and VF
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12
Q

What percentage of adults with NCCM experience ventricular arrhythmias?

A

38–47%

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13
Q

What are the proposed causes of arrhythmias in NCCM patients?

A
  • Arrest in embryogenic development of the heart
  • Disturbed compaction of the myocardium
  • Inappropriate maturation of the cardiac conduction system
  • Subendocardial ischemia
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14
Q

What is the significance of early repolarization (ER) in NCCM patients?

A

ER is associated with malignant ventricular arrhythmias and is found in a high percentage of NCCM patients.

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15
Q

What is the clinical presentation of NCCM?

A
  • Chest pain
  • Dyspnea
  • Palpitations
  • Edema
  • Syncope
  • Embolic ischemic stroke
  • Myocardial infarction
  • Sudden cardiac death
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16
Q

What are the major clinical manifestations of NCCM?

A
  • Heart failure
  • Supraventricular or ventricular arrhythmias
  • Thrombo-embolic events
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17
Q

What is the relationship between ECG findings and NCCM?

A

Approximately 90% of NCCM patients have ECG abnormalities, but these findings are not specific.

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18
Q

What is the frequency of atrial fibrillation in adult NCCM patients?

A

5–29%

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19
Q

What is Wolff-Parkinson-White syndrome?

A

A preexcitation syndrome characterized by the presence of an accessory pathway that predisposes to arrhythmias.

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20
Q

What is the typical diagnostic approach for NCCM?

A

Careful history taking and physical examination, with attention to cardiac symptoms and family history.

21
Q

What percentage of NCCM patients experience sudden cardiac death according to a large pediatric study?

A

6%

22
Q

What is the significance of the diagnosis of NCCM in asymptomatic individuals?

A

The true prevalence may be higher as asymptomatic individuals may go unnoticed.

23
Q

What was the outcome for the 35-year-old woman with NCCM mentioned in the case report?

A

She had an out-of-hospital cardiac arrest and was successfully resuscitated, later receiving an ICD.

24
Q

What is a key challenge in epidemiological research of NCCM?

A

Lack of consensus on absolute diagnostic criteria.

25
Q

What is the potential future trend regarding NCCM incidence?

A

The incidence is expected to increase due to greater awareness and improved imaging modalities.

26
Q

What are the symptoms of complete atrioventricular block?

A

Palpitations, (near) syncope, heart failure, potential tachycardiomyopathy

27
Q

What percentage of adult patients with NCCM experience atrial fibrillation?

A

5–29%

28
Q

What is Wolff-Parkinson-White syndrome?

A

A preexcitation syndrome characterized by an accessory pathway that predisposes to tachyarrhythmias and sudden death

29
Q

In which population is Wolff-Parkinson-White syndrome more common?

A

Pediatric patients (13–15%) compared to adult patients (0–3%) with NCCM

30
Q

What mutation is associated with sinus bradycardia and NCCM?

A

HCN4 mutation

31
Q

What is the prevalence of ventricular tachycardia (VT) in adult NCCM patients?

A

Observed in almost half of patients during a 30-month follow-up

32
Q

What percentage of NCCM patients showed impaired left ventricular systolic function?

A

82%

33
Q

True or False: A normal left ventricular systolic function excludes the risk of VT in NCCM patients.

A

False

34
Q

What is the purpose of implantable cardioverter defibrillator (ICD) therapy?

A

To prevent sudden cardiac death

35
Q

What is primary prevention in the context of ICD therapy?

A

For individuals at high risk for sustained VT but who have not yet had an episode

36
Q

What are common risk factors for sudden cardiac death (SCD) in NCCM patients?

A
  • Increased left ventricular size
  • Decreased left ventricular systolic function
  • Presence of ventricular arrhythmias
37
Q

What are the recommended monitoring methods for NCCM patients?

A

Periodic echocardiogram and Holter recordings

38
Q

What is the annual incidence of SCD in the general population?

A

0.1–0.2%

39
Q

Fill in the blank: The Jenni criteria are used for __________ in NCCM.

A

diagnosing NCCM

40
Q

What is the relationship between age and risk of SCD in pediatric NCCM patients?

A

Greatest risk in patients less than 1 year of age

41
Q

What factors did not seem to indicate risk for SCD in NCCM patients?

A
  • Sex
  • Localization
  • Degree of (non)compaction
42
Q

What is the effectiveness of ICD therapy in NCCM patients?

A

Highly effective for preventing sudden cardiac death

43
Q

What is the recommended approach for risk stratification of SCD in NCCM patients?

A

Empiric individualized risk stratification after thorough clinical evaluation

44
Q

What did the study by Caliskan et al. find regarding ICD shocks in NCCM patients?

A

Eight patients presented with appropriate ICD shocks due to sustained VT

45
Q

What are malignant ventricular arrhythmias frequently encountered in NCCM?

A
  • Ventricular tachycardia
  • Ventricular fibrillation
  • Sudden cardiac death
46
Q

What should be considered when deciding on ICD implantation?

A

Extensive counseling and shared-decision making with the patient

47
Q

True or False: All appropriate ICD interventions in the studied population were due to (fast) VTs.

A

True

48
Q

What remains speculative regarding NCCM patients?

A

Definitive risk factors for SCD

Noncompaction Cardiomyopathy (9 decks)

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