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Noncompaction Cardiomyopathy > Long-term Prognosis and Management > Flashcards

Long-term Prognosis and Management Flashcards

1
Q

What is the inheritance pattern of noncompaction cardiomyopathy (NCCM)?

A

Autosomal dominant

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2
Q

What are the clinical presentations of NCCM?

A
  • Asymptomatic
  • Supra-ventricular arrhythmias
  • Severe heart failure
  • Malignant ventricular arrhythmias
  • Thromboembolic events
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3
Q

Who first reported a case of heart failure related to NCCM, and when?

A

Engberding and Bender in 1984

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4
Q

What is the long-term prognosis for NCCM patients without heart failure?

A

Excellent long-term survival

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5
Q

What are the major causes of mortality and morbidity in NCCM?

A
  • Heart failure
  • Sudden cardiac death
  • Thrombo-embolic events
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6
Q

What percentage of severe outcomes occurred in a study with 2.3 years of follow-up for NCCM patients?

A

66%

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7
Q

What factors are associated with poor outcomes in NCCM patients?

A
  • Symptomatic patients
  • Heart failure symptoms and signs
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8
Q

What are the main determinants of poor prognosis in infantile and juvenile types of NCCM?

A
  • Heart failure at diagnosis
  • Hypoplasia of the compacted layer of the left ventricular wall
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9
Q

Fill in the blank: The first description of noncompaction of left ventricular myocardium as a separate disease entity was published by ______ in 1990.

A

Chin et al.

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10
Q

What is the typical presentation of NCCM?

A
  • Normal left ventricle size
  • Normal systolic and diastolic function
  • Primary dilated cardiomyopathy with or without restrictive left ventricular hemodynamics
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11
Q

What is the significance of a QRS duration >120 ms in NCCM patients?

A

Independent predictor of mortality

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12
Q

What genetic factors are related to outcomes in NCCM?

A

Age at diagnosis, LV systolic dysfunction, and risk of major adverse cardiovascular events (MACE)

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13
Q

True or False: Children with sporadic NCCM have a higher risk of MACE compared to those with genetic causes.

A

False

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14
Q

What were the most common mutations found in NCCM patients according to a recent study?

A
  • MYH7
  • MYBPC3
  • TTN
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15
Q

What is the reported mortality rate in adult NCCM populations over various studies?

A

Around 21%

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16
Q

What was the death/heart transplantation rate found in symptomatic NCCM patients after a median follow-up of 2.7 years?

A

31%

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17
Q

What is recommended for all NCCM patients due to the variability in outcomes?

A

Ongoing follow-up

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18
Q

What percentage of patients with NCCM were found to have a mutation in a cardiomyopathy gene?

A

Nearly one-third

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19
Q

What is the relationship between genetic status and outcomes in NCCM?

A

Genetic status may add to prediction of risk for major adverse cardiovascular events (MACE)

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20
Q

What is the follow-up period longer than 10 years associated with in NCCM patients diagnosed in childhood?

A

Development of systolic dysfunction of the left ventricle

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21
Q

What does RBBB stand for?

A

Right bundle branch block

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22
Q

What is the New York Heart Association (NYHA) classification used for?

A

To classify the severity of heart failure

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23
Q

What does LVEF stand for?

A

Left ventricular ejection fraction

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24
Q

What is the significance of a low LVEF?

A

It is a strong predictor of worse outcomes

25
Q

Fill in the blank: Patients with heart failure and/or significant LV dysfunction should be treated with _______.

A

Beta-blockers, ACE-inhibitors, and anticoagulants

26
Q

What is the mortality percentage associated with heart transplantation (HTX)?

A

Varies based on study, but significant in patients with certain risk factors

27
Q

True or False: A presentation under the age of 1 year is a predictor of worse outcomes in NCCM.

A

True

28
Q

What cardiac conditions are associated with higher mortality/HTX risks in NCCM patients?

A
  • Heart failure
  • Low LVEF
  • Neuromuscular disease
  • Arrhythmias
29
Q

What is the relationship between each 1% lower LVEF and mortality?

A

Associated with a 0.82 times increased mortality

30
Q

What is the proposed management strategy for patients with NCCM?

A
  • Empirical treatment
  • Clinical experience
  • Drug and device treatment based on primary presentation
31
Q

What are the risk factors for sudden cardiac death in NCCM patients?

A
  • Unexplained syncope
  • Non-sustained VTs
  • Family history of premature sudden cardiac death
32
Q

What is the role of the implantable cardioverter defibrillator (ICD) in NCCM management?

A

Appropriate in patients with sustained VT or VF and selected high-risk features

33
Q

What does the abbreviation NT-proBNP represent?

A

N-terminal pro b-type natriuretic peptide

34
Q

Fill in the blank: In NCCM, increasing knowledge among physicians and better imaging modalities may explain the _______ in prognosis over time.

A

Improvement

35
Q

What are the two key types of studies that can provide evidence to improve outcomes in NCCM?

A
  • Multicenter registry-based studies
  • Large case series
36
Q

What percentage of patients with heart failure were treated with preventive anticoagulation in the observed study?

A

84%

37
Q

What two populations have different prognoses when diagnosed with NCCM?

A
  • Pediatric patients
  • Adult patients
38
Q

What is a significant finding regarding the survival of patients with Duchenne or Becker muscular dystrophy and NCCM?

A

A 37% mortality rate in a median follow-up of 3.2 years

39
Q

What is the importance of family screening in NCCM management?

A

Identifies asymptomatic relatives at risk for sudden cardiac death

40
Q

What does the term ‘thrombo-embolic events’ refer to in the context of NCCM?

A

Complications due to intra-ventricular thrombosis leading to emboli

41
Q

List the factors that have been shown to correlate with poor prognosis in NCCM.

A
  • Age <1 year at presentation
  • Heart failure
  • Low LVEF
  • Arrhythmias
42
Q

What does the term ‘multivariate predictors’ refer to?

A

Factors identified through statistical analysis that predict outcomes

43
Q

What is NCCM?

A

NCCM stands for Non-Compaction Cardiomyopathy.

44
Q

What is the long-term prognosis for NCCM patients without heart failure?

A

Excellent long-term (5 years) survival, fully comparable with the general population.

45
Q

List factors associated with higher mortality in NCCM patients.

A
  • Heart failure at presentation
  • Age <1 year at presentation
  • Lower LVEF
  • Arrhythmias
  • Coexisting neuromuscular disease (NMD)
46
Q

What is a strong predictor of worse outcomes in NCCM?

A

Left ventricular ejection fraction (LVEF).

47
Q

True or False: Patients with heart failure and significant LV dysfunction have a favorable prognosis.

A

False.

48
Q

What treatments are considered inevitable for patients with HF and/or significant LV dysfunction?

A
  • Beta-blockers
  • ACE inhibitors
  • Anticoagulants
49
Q

What is appropriate for all patients presenting with sustained VT or VF?

A

ICD implantation.

50
Q

Fill in the blank: Most mortality in NCCM patients is due to either sudden death or _______.

A

heart failure.

51
Q

What strategies could improve the outcome of NCCM patients?

A

Large multicenter registry-based studies.

52
Q

What is required to clarify current uncertainties in NCCM?

A

More precise answers based on pathologic, clinical, and genetic analyses.

53
Q

What does the genetic heterogeneity of NCCM complicate?

A

The genotype-phenotype relationship.

54
Q

What type of studies should be conducted for NCCM patients?

A

Comprehensive genetic counseling, DNA diagnostics, and cardiologic family screening.

55
Q

What is a significant challenge in NCCM management?

A

Prevention of sudden cardiac death (SCD) and risk stratification.

56
Q

What role do registries play in the study of NCCM?

A

They could aid in clarifying current uncertainties.

57
Q

What might be the outcome of large case series in NCCM?

A

Improved diagnostic strategies and treatment.

58
Q

What recent reports indicate about the prognosis of NCCM?

A

Far more favorable and benign short- and midterm prognosis.

59
Q

What is the significance of family history in NCCM patients?

A

It is a potentially high-risk feature for sudden cardiac death.

Noncompaction Cardiomyopathy (9 decks)

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