Nonischemic cardiomyopathies

Question 1

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

Answer 1

cardiomyopathies

Question 2

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

Answer 2

Cardiac MRI

Question 3

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

Answer 3

ECG-gated cardiac CTA

Question 4

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

Answer 4

hypertrophic cardiomyopathy

Question 5

imaging hallmark of HCM on cardiac MRI

Answer 5

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

Question 6

most common form of HCM

Answer 6

asymmetric septal involvement

Question 7

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

Answer 7

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

Question 8

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

Answer 8

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

Question 9

in asymmetric septal HCM, the commonly involved segment is the

Answer 9

anteroseptal segments, most notable at the base of the LV

Question 10

20 to 30% of patients with asymmetric septal HCM develop

Answer 10

hypertrophic obstructive cardiomyopathy

Question 11

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

Answer 11

hypertrophic obstructive cardiomyopathy

Question 12

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

Answer 12

apical HCM variant

Question 13

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

Answer 13

apical HCM variant

Question 14

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

Answer 14

left apical aneurysms

Question 15

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

Answer 15

HCM; but both will lead to increased T1 values

Question 16

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

Answer 16

Fabry disease

Question 17

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

Answer 17

inferolateral segments of LV despite relatively diffuse hypertrophy

Question 18

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

Answer 18

T1-tissue mapping

Question 19

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

Answer 19

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

Question 20

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

Answer 20

cardiac iron overload

Question 21

as compared with HCM, amyloidosis usually have what T1 value

Answer 21

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

Question 22

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

Answer 22

cardiac amyloidosis

Question 23

most common etiology of cardiac amyloidosis

Answer 23

AL amyloidosis

Question 24

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

Answer 24

AL amyloidosis

Question 25

physiologically, amyloid deposition in the heart leads to

Answer 25

thickening of myocardium with impaired myocardial relaxation and diastolic dysfunction

Question 26

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

Answer 26

only mildly reduced

Question 27

gold standard for diagnosis of amyloidosis

Answer 27

tissue biopsy; apple-green birefringence on polarizing light microscopy

Question 28

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

Answer 28

cardiac amyloidosis

Question 29

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

Answer 29

biatrial dilation

Question 30

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

Answer 30

cardiac amyloid

Question 31

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

Answer 31

enhancement

Question 32

delayed gadolinium enhancement is most classically described as

Answer 32

diffuse subendocardial LV enhancement

Question 33

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

Answer 33

cardiac amyloid

Question 34

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

Answer 34

hypereosinophilic syndrome

Question 35

classic manifestation of hypereosinophilic syndrome is

Answer 35

Loeffler endocarditis

Question 36

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

Answer 36

Loeffler endocarditis

Question 37

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

Answer 37

Nonischemic-dilated cardiomyopathies

Question 38

approximately 50% of dilated cardiomyopathies are _____ in origin

Answer 38

idiopathic or genetic

Question 39

idiopathic-dilated cardiomyopathies are typically seen in what age group

Answer 39

younger patients

Question 40

all cardiomyopahties demonstrate ___ systolic and diastolic volumnes with ____ EF

Answer 40

increase systolic and diastolic volumes with decreased EF

Question 41

ventricular thickness in dilated cardiomyopathies is often

Answer 41

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

Question 42

gadolinium enhancement in dilated cardiomyopathy

Answer 42

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

Question 43

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

Answer 43

arrythmogenic RV cardiomyopathy or RV dysplasia

Question 44

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

Answer 44

arrythmogenic RV cardiomyopathy or RV dysplasia

Question 45

most reliable features of early ARVC

Answer 45

regional wall motion abnormalitis in RV

Question 46

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

Answer 46

ARVC/ARVD

Question 47

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

Answer 47

LV involvement

Question 48

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

Answer 48

myocarditis

Question 49

in acute inflammatory phase of myocarditis, T2W images can demonstrate

Answer 49

global or regional areas of increased intenstiy to signify myocardial edema

Question 50

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

Answer 50

myocardial edema

Question 51

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

Answer 51

3.2:1

Question 52

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

Answer 52

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

Question 53

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

Answer 53

sarcoidosis

Question 54

diagnosis of cardiac sarcnoid can be confirmed through

Answer 54

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

Question 55

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

Answer 55

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

Question 56

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

Answer 56

ischemic cardiomyopathy

Question 57

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

Answer 57

RV delated enhancement

Question 58

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

Answer 58

LV noncompaction

Question 59

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

Answer 59

LV noncompaction

Question 60

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

Answer 60

septum

Question 61

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

Answer 61

children/adults

Question 62

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

Answer 62

significantly increased LV end diastolic volume and reduced LV EF

Question 63

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

Answer 63

Takotsubo cardiomyopathy

Question 64

hallmark of Takotsubo cardiomyopathy is a

Answer 64

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)

Question 65

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

Answer 65

within 2 weeks of symptom onset

Question 66

enhancement pattern in Takotsubo cardiomyopathy

Answer 66

no perfusion defects or delayed enhancements

Question 67

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

Answer 67

iron overload cardiomyopathy

Question 68

iron overload cardiomyopathy ultimately results in

Answer 68

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

Question 69

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

Answer 69

Cardiac MR T2*

Question 70

normal range of T2* values

Answer 70

>20 ms

Question 71

T2* value indicating mild to moderate iron loading

Answer 71

10 to 20 ms

Question 72

T2* value indicating severe iron loading

Answer 72

<10 ms