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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
Fill in the blank: Patients with heart failure and/or significant LV dysfunction should be treated with _______.
Beta-blockers, ACE-inhibitors, and anticoagulants
26
What is the mortality percentage associated with heart transplantation (HTX)?
Varies based on study, but significant in patients with certain risk factors
27
True or False: A presentation under the age of 1 year is a predictor of worse outcomes in NCCM.
True
28
What cardiac conditions are associated with higher mortality/HTX risks in NCCM patients?
* Heart failure * Low LVEF * Neuromuscular disease * Arrhythmias
29
What is the relationship between each 1% lower LVEF and mortality?
Associated with a 0.82 times increased mortality
30
What is the proposed management strategy for patients with NCCM?
* Empirical treatment * Clinical experience * Drug and device treatment based on primary presentation
31
What are the risk factors for sudden cardiac death in NCCM patients?
* Unexplained syncope * Non-sustained VTs * Family history of premature sudden cardiac death
32
What is the role of the implantable cardioverter defibrillator (ICD) in NCCM management?
Appropriate in patients with sustained VT or VF and selected high-risk features
33
What does the abbreviation NT-proBNP represent?
N-terminal pro b-type natriuretic peptide
34
Fill in the blank: In NCCM, increasing knowledge among physicians and better imaging modalities may explain the _______ in prognosis over time.
Improvement
35
What are the two key types of studies that can provide evidence to improve outcomes in NCCM?
* Multicenter registry-based studies * Large case series
36
What percentage of patients with heart failure were treated with preventive anticoagulation in the observed study?
84%
37
What two populations have different prognoses when diagnosed with NCCM?
* Pediatric patients * Adult patients
38
What is a significant finding regarding the survival of patients with Duchenne or Becker muscular dystrophy and NCCM?
A 37% mortality rate in a median follow-up of 3.2 years
39
What is the importance of family screening in NCCM management?
Identifies asymptomatic relatives at risk for sudden cardiac death
40
What does the term 'thrombo-embolic events' refer to in the context of NCCM?
Complications due to intra-ventricular thrombosis leading to emboli
41
List the factors that have been shown to correlate with poor prognosis in NCCM.
* Age <1 year at presentation * Heart failure * Low LVEF * Arrhythmias
42
What does the term 'multivariate predictors' refer to?
Factors identified through statistical analysis that predict outcomes
43
What is NCCM?
NCCM stands for Non-Compaction Cardiomyopathy.
44
What is the long-term prognosis for NCCM patients without heart failure?
Excellent long-term (5 years) survival, fully comparable with the general population.
45
List factors associated with higher mortality in NCCM patients.
* Heart failure at presentation * Age <1 year at presentation * Lower LVEF * Arrhythmias * Coexisting neuromuscular disease (NMD)
46
What is a strong predictor of worse outcomes in NCCM?
Left ventricular ejection fraction (LVEF).
47
True or False: Patients with heart failure and significant LV dysfunction have a favorable prognosis.
False.
48
What treatments are considered inevitable for patients with HF and/or significant LV dysfunction?
* Beta-blockers * ACE inhibitors * Anticoagulants
49
What is appropriate for all patients presenting with sustained VT or VF?
ICD implantation.
50
Fill in the blank: Most mortality in NCCM patients is due to either sudden death or _______.
heart failure.
51
What strategies could improve the outcome of NCCM patients?
Large multicenter registry-based studies.
52
What is required to clarify current uncertainties in NCCM?
More precise answers based on pathologic, clinical, and genetic analyses.
53
What does the genetic heterogeneity of NCCM complicate?
The genotype-phenotype relationship.
54
What type of studies should be conducted for NCCM patients?
Comprehensive genetic counseling, DNA diagnostics, and cardiologic family screening.
55
What is a significant challenge in NCCM management?
Prevention of sudden cardiac death (SCD) and risk stratification.
56
What role do registries play in the study of NCCM?
They could aid in clarifying current uncertainties.
57
What might be the outcome of large case series in NCCM?
Improved diagnostic strategies and treatment.
58
What recent reports indicate about the prognosis of NCCM?
Far more favorable and benign short- and midterm prognosis.
59
What is the significance of family history in NCCM patients?
It is a potentially high-risk feature for sudden cardiac death.

Noncompaction Cardiomyopathy (9 decks)

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