04.25 - CV Misc 2 (Nichols) Flashcards
1
Q
What usually causes V Tach in adults
A
IHD
2
Q
100% of Hypertrophic CM is due to
A
Genetic causes (sarcomeric proteins)
3
Q
3 Main Causes of Long QT
A
(1) IHD; (2) Low K, Ca, Mg; (3) Channelopathy
4
Q
3 major forms of Restrictive CM
A
Amyloidosis (TTR or systemic), Endomyocardial Fibrosis, Loeffler Endomyocarditis
5
Q
40 yo Asian male with syncope or SCD due to ventricular tachycardia, especially during sleep
A
Presentation of Brugada Syndrome
6
Q
5 general pathways leading to end-stage DCM
A
(1) Genetic causes; (2) Infection; (3) Alcohol, other toxic; (4) Peripartum CM; (5) Iron Overload
7
Q
8 yo with syncope or SCD due to V Tach during emotional or physical stress
A
Presentation of Catecholaminergic Polymorphic V Tach
8
Q
Are V Tach’s usually mono or polymorphic?
A
Mono
9
Q
Asteroid Bodies are most often seen in
A
Sarcoidosis
10
Q
Asystole =
A
No electrical activity at all
11
Q
Begins in RV with fatty replacement of myocytes, frequently w lymphocyte infiltration and later fibrous scarring
A
Morphological progression of Arrhythmogenic RV CM
12
Q
Cardiac involvement is found in what percent of Sarcoidosis
A
76%
13
Q
Cause of Arrhythmogenic RV CM
A
Mutations in genes encoding desmosomal proteins; Possibly with enteric viral infection of Right Heart
14
Q
Causes of Chylous Effusion
A
Mediastinal Lymphatic Obstruction
15
Q
Causes of Restrictive CM
A
Amyloidosis, Radiation-induced fibrosis; Idiopathic
16
Q
Causes of Seroanguineous Effusion
A
Blunt Chest Trauma, Malignancy, Ruptured MI, Aortic Dissection
17
Q
Causes of Serous Effusion
A
CHF, Hypoalbuminea
18
Q
Characteristic histologic features of HCM
A
Marked myocyte hypertrophy; Haphazard Myocyte Disarray, Interstitial Fibrosis
19
Q
Constrictive Pericarditis
A
Extensive Dense, Fibrotic Scarring
20
Q
Dilated CM when describing a functional entity usually means
A
Idiopathic Dilated CM
21
Q
Distinctive “spongy appearance of ventricles, associated with CHF and arrhythmias
A
Left Ventricular Compaction
22
Q
EKG of Brugada
A
Presistently elevated ST segments descending w/ upward convexity to an inverted T wave (V1-V3)
23
Q
Endomyocardial Fibrosis is characterized by
A
Dense diffuse fibrosis of the ventricular endocardium and Subendocardium
24
Q
Endomyocardial Fibrosis is principally a disease of
A
children and young adults in Africa and other tropical areas
25
Epsilon Wave
Notch in terminal QRS, most prominent in V1
26
Epsilon Wave can be manifestation of
RV CM
27
Extensive replacement of myocytes by adipocytes and fibrous tissue
Arrhythmogenic RV CM
28
Feature of inherited forms of DCM
Congenital conduction abnormalities
29
Functional Manifestation of Dilated CM
Impaired contractility and Systolic Fxn
30
Functional Manifestation of Hypertrophic CM
Impaired compliance and diastolic function
31
Functional Manifestation of Restrictive CM
Impaired compliance and diastolic function
32
Fundamental defect in DCM
ineffective contraction
33
Genetics of Brugada
Autosomal Dominant
34
Gross appearance of Myocarditis
Acute: Normal of Dilated; Advanced: Flabby and Mottled
35
Group of channelopathies causing shortened myocyte AP's and risk of V Tach, SCD
Brugada
36
HCM is fundamentally a disorder of
Sarcomeric Proteins (GOF)
37
Histology of Cardiac Myxoma
Stellate, frequently multinucleated Myxoma cells with other cell types
38
How does DCM usually present
Signs of slowly progressive CHF, including dyspnea, easy fatiguability, and poor exertional capacity
39
How is Brugada recognized
Persistently elevated ST segs, with upward convexity to inverted T wave (V1-V3)
40
In what age range does DCM most commonly present
20-50 years
41
Life-saving preventive tx of Arrhythmogenic RV CM
defibrillator device
42
Life-saving preventive tx of Brugada
defibrillator device
43
Life-saving preventive tx of CPVT
BB's or defib implant
44
Life-saving preventive tx of TDP is
defibrillator device
45
Lyme Myocarditis manifests primarily as
Self-limited conduction system disease
46
Major clinical manifestations of Myxomas
Ball-valve obstruction, Embolization, IL-6
47
Mechanism of Arrhythmias in CPVT
Triggered activity resulting from delayed after-depolarizations due to high intracellular calcium
48
Mechanism of HF in Restrictive CM
Impairment of compliance (diastolic dysfunction)
49
Microscopic findings in Loeffler Endomyocarditis
Eosinophilic infiltrates, Myocardial Necrosis
50
Microscopic findings in Restrictrive CM
Interstitial fibrosis common
51
Morphological progression of Arrhythmogenic RV CM
Begins in RV with fatty replacement of myocytes, frequently w lymphocyte infiltration and later fibrous scarring
52
Morphology of ARVC
RV wall is severely thinned owing to myocyte replacement by massive fatty infiltration and less amounts of fibrosis
53
Mortality after DCM presentation
50% in 2 years
54
Most characterstic cells in Rhabdomyomas
Spider Cells
55
Most common appearance of Myxomas
Soft, translucent, villous lesions with gelatinous appearance
56
Most common cause of Myocarditis in US
Viral Infections (Coxsackieviruses)
57
Most common cause of rhythm disorders
Ischemia
58
Most common form of Restrictive CM worldwide
Endomyocardial Fibrosis
59
Most common major cardiomyopathy pattern
Dilated CM
60
Most common malignancy of heart
Mets
61
Most common primary malignant tumor of heart
Angiosarcoma
62
Most common primary tumor of adult heart
Myxoma, Usually Left Atrial
63
Most common primary tumor of heart in infants and children
Rhabdomyosarcomas
64
Most common systemic disorder associated with pericarditis
Uremia
65
Most frequently affected protein in HCM
Beta-myosin heavy chain
66
Most myocytes in DCM exhibit
Hypertrophy with enlarged nuclei
67
Murmur in HCM?
Harsh Systolic Ejection murmur if dynamic obstruction present (25 % of cases)
68
Mutations in ARVC involve
Desmosomal junctions
69
Mutations in Cardiac Ryanodine Receptor
Familial Catecholaminergic Poly V Tach
70
Mutations in Desmosomal Proteins
Arrhythmogenic RV CM
71
Mutations in Desmosomal proteins causes
Re-entrant V Tach, originating in RV
72
Mutations in genes encoding desmosomal proteins; Possibly with enteric viral infection of Right Heart
Cause of Arrhythmogenic RV CM
73
Noncaseating granulomas in lungs, lymph nodes, liver, etc
Sarcoidosis
74
One unifying feature of HCM mutations
All affect Sarcomeric proteins and increase microfilament activation
75
Pericardial exudate in Acute Viral Pericarditis/Uremia vs Acute Bacterial Pericarditis
Fibrinous vs Fibrinopurulent (Suupurative)
76
Pericarditis classically manifests with
Atypical Chest pain (not related to exertion, worse in recumbency), and a prominent friction rub
77
Presentation of ARVC
Right-sided HF and rhythm disturbances that can cause sudden cardiac death
78
Presentation of Brugada Syndrome
40 yo Asian male with syncope or SCD due to ventricular tachycardia, especially during sleep
79
Presentation of Catecholaminergic Polymorphic V Tach
8 yo with syncope or SCD due to V Tach during emotional or physical stress
80
Presistently elevated ST segments descending w/ upward convexity to an inverted T wave (V1-V3)
EKG of Brugada
81
Primary Pericarditis is most often due to
Viral infection (typically with concurrent Myocarditis)
82
Proteins most often affected in congenital DCM
Cytoskeletal
83
Prototype Restrictive CM
Cardiac Amyloidosis
84
Re-entrant V Tach originating from RV, possibly related to abnormalities in Myocyte Adhesion via desmosomal proteins
What causes arrhythmia in Arrhythmogenic RV CM
85
Restrictive CM is characterized by
Primary decrease in ventricular compliance
86
Secondary cardiomyopathy means
Presenting as the cardiac manifestation of a systemic disorder
87
Secondary Myocardial Dysfunction mimicking Hypertrophic CM
HTN HD, Aortic Stenosis
88
Secondary Myocardial Dysfunction mimicking Restrictive CM
Pericardial Constriction
89
Some fraction of Idiopathic Dilated CM is due to
Previous Viral Myocarditis and Alcohol
90
Spider Cells =
Rhabdomyomas
91
T/F: 80-90% of patients who suffer SCD but are successfully resuscitated do not show any enzymatic or ECG evidence of myocardial necrosis - even if original cause was IHD
TRUE
92
T/F: Clinical progression from myocarditis to DCM is frequent
False, rare
93
T/F: Hypertrophic cardiomyopathy (structural) is a genetic disease
TRUE
94
The characteristic histologic abnormalities in DCM are
nonspecific, and do not typically point to a specific etiologic entity
95
The clinical features of Myocarditis can mimic those of
Acute MI
96
Top 3 most common cancers to met to heart
Lung, Lymphoma, Breast
97
Toxic agents to heart implicated in CM
Alcohol, Doxorubicin, Cobalt
98
Type of V Tach typical of Congenital Long QT is
Torsades de Pointes
99
What causes appearance of Torsades de Pointes
Early After-Depolarizations from multiple sites causes multifocal ventricular tachycardia with polymorphology, varying over time
100
What causes arrhythmia in Arrhythmogenic RV CM
Re-entrant V Tach originating from RV, possibly related to abnormalities in Myocyte Adhesion via desmosomal proteins
101
What causes Catecholaminergic Poly V Tach
Mutations in Cardiac Ryanodine Receptor (SR Ca release channel)
102
What causes most of the 300,000 SCD/year in US
V Tach
103
What causes V Tach in Brugada
Variably blunted Na currents can cause shortened or failed AP's and localized conduction block in RV, predisposing to phase 2 re-entrant V Tach
104
What channel is affected in Brugada Syndrome
Sodium
105
What channel is affected in LQTS1
Iks
106
What channel is affected in LQTS2
Ikr
107
What channel is affected in LQTS3
Ina
108
What is present in childhood with syncope or SCD due to polymorphic ventricular tachycardia?
Congenital Long QT Syndrome
109
What is the normal QTc?
<440 milliseconds
110
What is the risk of early after-depolarizations
V Tach
111
What is the risk of V Tach
SCD
112
What is the site and mechanims of Catecholaminergic Polymorphic VT Channelopathy?
Delayed After-Depolarization in Left Ventricle
113
What is the site and mechanism of Brugada Channelopathy?
Re-entry in Right Ventricle
114
What is the site and mechanism of Long QT Channelopathy
Early After-Depolarization in Left Ventricle
115
What leads show Brugada sign
V1-V3
116
What percent of DCM are caused by mutations affecting cytoskeletal proteins
20-50%
117
What percent of MI's are unrecognized?
up to 30%
118
What percent of patients with HCM have dynamic obstruction of LV outflow
25%
119
What's special about hypersensitivity myocarditis
Numerous eosinophils infiltrating tissue
120
When does HCM typically manifest
Post-pubertal growth spurt
121
Which chambers are affected in DCM
all
122
Which channelopathy benefits from BB's
Familial Catecholaminergic Poly V Tach
123
With extensive _____, healing can result in fibrosis (chronic pericarditis)
suppuration or caseation (TB)
124
X-linked DCM is most frequently associated with
Dystrophin
