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Cardiac > Nonischemic cardiomyopathies > Flashcards

Nonischemic cardiomyopathies Flashcards

1
Q

refers to specific diseases of the myocardium, most commonly caused by ischemic heart disease

A

cardiomyopathies

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2
Q

now considered the reference standard for assessing chamber size, left ventricular function and ventricular mass in cardiomyopathies

A

Cardiac MRI

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3
Q

as cardiac MRI is CI in some patients, this also serves as a tool to diagnose certain nonischemic cardiomyopathies through detailed evaluation of cardiac function and morphology

A

ECG-gated cardiac CTA

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4
Q

an autosomal dominant genetic cardiomyopathy seen in 0.05% to 0.2% of the population and is the leading cause of sudden cardiac death in young adults and athletes

A

hypertrophic cardiomyopathy

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5
Q

imaging hallmark of HCM on cardiac MRI

A

focal, regional or diffuse LV hypertrophy with measurements often exceeding 20mm as measured at end-diastole. LV is generally not dilated and EF is usually normal or increased, leading to obliteration of the LV cavity during systole

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6
Q

most common form of HCM

A

asymmetric septal involvement

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7
Q

HCM is differentiated from hypertension-related hypertrophy by the presence of

A

myofiber disarray with focal areas of necrosis and subsequent fibrosis, most often seen in the midmyocardium; hazy midmyocardial enhancement or nodular enhancement in the interventricular septum at the RV insertion points

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8
Q

delayed enhancement is identified in over 80% of HCM patients and is associated with

A

increased incidence of ventricular tachyarrhythmias which can lead to sudden cardiac death

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9
Q

in asymmetric septal HCM, the commonly involved segment is the

A

anteroseptal segments, most notable at the base of the LV

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10
Q

20 to 30% of patients with asymmetric septal HCM develop

A

hypertrophic obstructive cardiomyopathy

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11
Q

complication of asymmetric septal HCM where there is narrowing of the LV outflow tract by the hypertrophies myocardium causing flow acceleration across the LVOT

A

hypertrophic obstructive cardiomyopathy

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12
Q

HCM variant characterized by pronounced ventricular hypertrophy at the LV apex, which leads to the characteristic “spade-like” configuration of the LV on vertical long axis view of the heart

A

apical HCM variant

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13
Q

this HCM variant is associated with HPN, but has an overall better prognosis as it is less commonly associated with sudden cardiac death

A

apical HCM variant

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14
Q

5% of all HCMs with left apical involvement develop ______, which increase the risk for thromboembolic disease and the development of clinical heart failure

A

left apical aneurysms

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15
Q

In T1-tissue mapping, higher T1 values will be seen in HCM or hypertensive disease?

A

HCM; but both will lead to increased T1 values

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16
Q

an x-linked disorder which results in the pathologic accumulation of glycosphingolipid within different tissues, including the heart; clinical and morphologic cardiac manifestations may mimic those of HCM

A

Fabry disease

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17
Q

in fabry disease as compared to HCM, hazy midmyocardial enhancement is often isolated where

A

inferolateral segments of LV despite relatively diffuse hypertrophy

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18
Q

one of the best ways to differentiate between Fabry disease, HCM and cardiac amyloidosis is through

A

T1-tissue mapping

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19
Q

Since fat has a shorter native T1 than myocardium, lipid accumulation in Fabry disease causes

A

significant shortening of native T1 relaxation with values reported at 853 +/- 53 ms at 1.5 T

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20
Q

aside from fabry disease, this other cardiomyopathy also demonstrates such a low native T1 myocardium

A

cardiac iron overload

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21
Q

as compared with HCM, amyloidosis usually have what T1 value

A

higher native T1 value of around 1100 ms at 1.5 T; HCM is usually around 1000 ms

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22
Q

a restrictive cardiomyopathy that is caused by the random abnormal deposition of extracellular amyloid fibrils within the heart, including within the myocardium, atria, coronary arteries and valves

A

cardiac amyloidosis

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23
Q

most common etiology of cardiac amyloidosis

A

AL amyloidosis

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24
Q

caused by deposition of fibrils of monoclonal immunoglobulin light chains associated with B-cell dyscrasias such as multiple myeloma

A

AL amyloidosis

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25
Q

physiologically, amyloid deposition in the heart leads to

A

thickening of myocardium with impaired myocardial relaxation and diastolic dysfunction

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26
Q

while systolic dysfunction does occur, LV EF in patients with amyloid is usually

A

only mildly reduced

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27
Q

gold standard for diagnosis of amyloidosis

A

tissue biopsy; apple-green birefringence on polarizing light microscopy

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28
Q

in MRI, it appears as diffuse concentric LV hypertrophy of the myocardium which initially leads to an elevated LV mass index with inital decrease in ventricular volumes

A

cardiac amyloidosis

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29
Q

in cardiac amyloidosis, this is often present secondary to increased ventricular pressures from the diastolic dysfunction

A

biatrial dilation

30
Q

another clue to diagnosis of this disease is the presence of circumferential subendocardial hypoperfusion on dynamic first pass perfusion sequence obtained during the administration of gadolinium

A

cardiac amyloid

31
Q

the deposition of amyloid in the myocardium leads to dramatic expansion of the extracellular space and thus produce enhancement or nonenhancement?

A

enhancement

32
Q

delayed gadolinium enhancement is most classically described as

A

diffuse subendocardial LV enhancement

33
Q

constellation of LV hypertrophy, diffuse subendocardial hypoperfusion and delated enhancement in the setting of biatrial enlargement, diastolic dysfunction and abnormal T1 kinetics before (T1-tissue mapping) and after (abnormal myocardial nulling) the administration of contract can allow one to reliably make the diagnosis of

A

cardiac amyloid

34
Q

defined by peripheral blood eosinophilia which can lead to multiple system dysfunction and damage

A

hypereosinophilic syndrome

35
Q

classic manifestation of hypereosinophilic syndrome is

A

Loeffler endocarditis

36
Q

a restrictive cardiomyopathy which can involve one or both ventricles. on delayed enhancement sequences, a classic circumferential subendocadial pattern of fibrosis is present corresponding to the eosinophilic destruction of this portion of the myocardium

A

Loeffler endocarditis

37
Q

characterized by cardiac chamber dilatation coupled with impaired contractility of the LV or both the LV and RV

A

Nonischemic-dilated cardiomyopathies

38
Q

approximately 50% of dilated cardiomyopathies are _____ in origin

A

idiopathic or genetic

39
Q

idiopathic-dilated cardiomyopathies are typically seen in what age group

A

younger patients

40
Q

all cardiomyopahties demonstrate ___ systolic and diastolic volumnes with ____ EF

A

increase systolic and diastolic volumes with decreased EF

41
Q

ventricular thickness in dilated cardiomyopathies is often

A

mildly thinned to normal and is associated with increased end-diastole volumes, greater than 140 mL for the LV and greater than 150 mL for the RV

42
Q

gadolinium enhancement in dilated cardiomyopathy

A

often not seen; if present, it characteristically appears as thin, linear midmyocardial enhancement most often involving the interventricular septum

43
Q

accounts for 5% of the cases of sudden cardiac death in patients less than 35 y.o and therefore the diagnosis is aggressively pursued so treatment can be initiated as soon as possible

A

arrythmogenic RV cardiomyopathy or RV dysplasia

44
Q

presents with RV dilation, reduced RV RF and focal areas of RV dyskinesia leading to small “aneurysms”

A

arrythmogenic RV cardiomyopathy or RV dysplasia

45
Q

most reliable features of early ARVC

A

regional wall motion abnormalitis in RV

46
Q

classically described as RV predominant disease, genetic testing and imaging advantage has increased our awareness of the LV dominant and biventricular phenotypes of disease

A

ARVC/ARVD

47
Q

in excess of 75% of ARVC have _____ in advance disease

A

LV involvement

48
Q

focal inflammation of the myocardium, most commonly triggered by a viral infection and can lead to a new dilated cardiomyopathy in an otherwise healthy patient

A

myocarditis

49
Q

in acute inflammatory phase of myocarditis, T2W images can demonstrate

A

global or regional areas of increased intenstiy to signify myocardial edema

50
Q

when the average signal of myocardium is twice that of skeletal muscle as measured on T2W nonfat saturated images, a diagnosis of ____ can be made

A

myocardial edema

51
Q

an abnormal global relative enhancement ratio is defined as greater than ____ and occurs secondary to acute cell membrane rupture which leads to increased intracellular gadolinium deposition

A

3.2:1

52
Q

presence of two out of three criteria allow one to reliably make the diagnosis of myocarditis, namely

A

myocardial edema ratio, global relative enhancement, characteristic delayed enhancement pattern

53
Q

systemic inflammatory disorder characterized by the deposition of noncaseating nonnecrotic granulomas in affected organs

A

sarcoidosis

54
Q

diagnosis of cardiac sarcnoid can be confirmed through

A

either by the presence of noncaseating granulomas on endomyocardial biopsy or clinical criterial which include abnormalitis on ECG, cardiac echo, RMI and radionuclide imaging

55
Q

in early phase of cardiac sarcoidosis, there is focal wall thickening on SSFP or T1W, edema on T2W and regional wall motion abnormalities. enhancement in sarcoidosis is characterized as

A

delayed enhancement that is extremely variable with biventricular linear and/or nodular lesions occuring predominantly in a subepicardial/ midmyocardial distribution

56
Q

in the late stages of cardiac sarcoidosis, it can mimic _____ with wall thinning and transmural enhancement atlthough the enhancement does not correspond to a vascular territory and foci or subepicardial and midmyocardial are often present

A

ischemic cardiomyopathy

57
Q

since late stages of sarcoidosis may mimic ischemic cardiomyopathy, this feature of enhancement is uncommon in ischemic cardiomyopathy

A

RV delated enhancement

58
Q

genetocally heterogeneous disease seen in both pediatric and adult populations, where the normal morphogenesis of myocardial tissue into compact myocardium is disrupted

A

LV noncompaction

59
Q

appears as highly trabeculated LV myocardium and ultimately can cause heart failur, arrhythmias and sudden cardiac death

A

LV noncompaction

60
Q

hypertrabeculation in LV noncompaction spares the ____ but often involves the anterolateral, and inferolateral segments at the base and mid-cavity levels and much of the cardiac apex

A

septum

61
Q

delayed enhancement in LV noncompaction is more commonly detected in ____ and is seen both in areas of hypertrabeculation and in the adjacent midmyocardium

A

children/adults

62
Q

when athletes with hypertrabeculation were compared t patients with LVNC, those with LVNC demonstrated a

A

significantly increased LV end diastolic volume and reduced LV EF

63
Q

also known as apical ballooning syndrome or “broken heart” syndrome, is stress-induced cardiomyopathy, most frequently seen in postmenopausal women after a severe emotional or physical event

A

Takotsubo cardiomyopathy

64
Q

hallmark of Takotsubo cardiomyopathy is a

A

transient hypokinesis or akinesis of the mid and apical segment of LV with hypercontractility of basilar segments (resembling a japanese pot used to catch octopi)

65
Q

edema in Takotsubo cardiomyopathy often resolves when, which is in contrast to STEMI and myocarditis where it can persist 2 to 3 months after cardiac event

A

within 2 weeks of symptom onset

66
Q

enhancement pattern in Takotsubo cardiomyopathy

A

no perfusion defects or delayed enhancements

67
Q

occurs secondary to iron deposition in the myocardium, either related to genetic disorders of iron metabolism of increased circulating iron related to transfusion

A

iron overload cardiomyopathy

68
Q

iron overload cardiomyopathy ultimately results in

A

dilated or restrictive phenotypes of iron overload cardiomyopathy, resulting to clinical heart failure, conduction abnormalities and pulmonary hypertension

69
Q

MR sequence that can be used for quantitative assessment of iron deposition as iron deposition generates local fiel inhomogeneities which result in T2* shortening

A

Cardiac MR T2*

70
Q

normal range of T2* values

A

> 20 ms

71
Q

T2* value indicating mild to moderate iron loading

A

10 to 20 ms

72
Q

T2* value indicating severe iron loading

A

<10 ms

Cardiac (8 decks)

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