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Cardiomyopathies, Tumors/masses/emboli Flashcards

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USMLE® Step 1 flashcards
1
Q

This is a predictor of late recovery of LV systolic function in idiopathic dilated cardiomyopathy?

A

LV sphericity index

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2
Q

Describe sphericity index

A
  • Sphericity index = LV long axis length / LV short axis diameter (mid-ventricle)
  • Represents the extent of chamber remodeling from a normal ellipitical ventricular shape to an abnormal spherical shape
  • Can be determined in end-systole or end-diastole
  • >>> spherical LV’s = worse prognosis
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3
Q

Explain the effects of Dobutamine on sphericity index

A

Improvement in sphericity index in response to dobutamine is a predictor of late recovery of LV systolic function

  • Dobutamine –> less spherical ventricle –> greater likelihood of reverse remodeling
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4
Q

In patients with recovered tachycardia-induced cardiomyopathy, what is generally not well tolerated?

Why?

A

Recurrent arrhythmia (after normal rhythm has been restored)

  • can lead to a rapid decline in LV function
  • due to persistence of structural abnormalities of the LV
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5
Q

What can serve as a predictor of likelihood of recurrence of peripartum cardiomyopathy (LV systolic dysfunction) with subsequent pregnancy’s?

A

Dobutamine stress Echo –> Marked inotropic contractile reserve

  • useful tool to predict safety of a recurrent pregnancy
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6
Q

Define peripartum cardiomyopathy diagnosis

A
  • Heart failure symptoms with an LVEF < 45%
  • During:
    • last month of pregnancy
    • 5 months postpartum
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7
Q

What percentage of patients with peripartum cardiomyopathy fully recover?

A

50%

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8
Q

What is the most specific cardiac finding of Sarcoidosis?

Other highly specific findings?

A

Thinning of the basal ventricular septum

  • dilated cardiomyopathy with noncoronary territory wall motion abnormalities
  • aneurysms involving the posterior, lateral, basal wall and apex
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9
Q

Define cardiotoxicity (CTRCD)

A
  • > 10 percentage points reduction in LVEF, to an LVEF < 53%
    • if present, confirmatory testing should be repeated 2-3 weeks later
  • Subclinical disease
    • LVEF drop < 10 percentage points
    • GLS > 15% drop
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10
Q

What is the next step in evaluation for CTRCD

  • LVEF drop by < 10%
  • GLS drop by > 15%
A

Confirmatory study to assess LVEF should be repeated 2-3 weeks later

(after initial abnormal study)

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11
Q

This finding is consistent with hypertensive LVH rather than physiologic hypertrophy of athlete’s heart?

A

RWT > 0.42

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12
Q

What is an additional finding that is common in athletes heart but not hypertensive LVH?

A

RV dilation

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13
Q

What is a specific sign for radiation associated heart disease?

A

Aorto-mitral curtain thickening

  • thickening or calcification (junction between the base of the anterior mitral leaflet and the aortic root)
    • typically this region is spared from most degenerative or acquired conditions, with the exception of endocarditis or aortitis
  • Additional finding (highly suggestive in conjunction with aorto-mitral curtain thickening) –> aortic or mitral valve disease
    • calcification of the aortic sinuses (atypical location for atherosclerosis)
  • MR is most common reason for surgery
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14
Q

What are HCM features associated with increased risk of SCD?

A
  • Restrictive diastolic filling pattern
  • Increased LV wall thickness
    • > 30 mm –> increased risk + indication for ICD
  • Increased LA volume index
  • LV dysfunction (burn-out stage)
  • LV apical aneurysm
  • LVOT obstruction
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15
Q

What is the most typical echocardiographic appearance of hereditary hemochromatosis?

A

Mildly dilated LV cavity with global LV dysfunction

and

normal or mildly increased LV wall thickness

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16
Q

Describe the stages of cardiac involvement in hereditary hemochromatosis

A
  • Early stages
    • diastolic dysfunction - may be the first manifestation
  • Progressive decrease in LV systolic function, LV cavity dilation, and biatrial enlargement
  • Later stages
    • LV wall thickness is usually normal or mildly increased even in the later stages
    • Restrictive filling pattern is a late manifestation when there is LV dysfunction
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17
Q

What is the diagnosis in this young athlete:

  • LVEDD - 5.9 cm
  • LVESD - 2.6 cm
  • LVEF - 60%
  • IVSd - 1.3 cm
  • PWT - 1.3 cm
  • LAVI 37 mL/m2
  • Latera e’ 18 cm/s
A

Athlete’s Heart​

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18
Q

What is the diagnosis in this young athlete:

  • LVEDD - 5.3 cm
  • LVEF - 65%
  • IVSd - 1.6 cm
  • PWT - 1.2 cm
  • Latera e’ 6 cm/s
A

Hypertrophic Cardiomyopathy

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19
Q

What is the diagnosis in this young athlete:

  • LVEDD - 5.2 cm
  • LVEF - 55%
  • RVOT measurement (PSAX) in diastole - 3.6 cm
A

Arrhythmogenic RV dysplasia due to RVOT dilation

normal size RVOT in PSAX = < 3.6 cm

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20
Q

What is the diagnosis in this young athlete:

  • LVEDD - 5.3 cm
  • LVEF - 60%
  • MVP with moderate MR
  • Aortic diameter
    • Sinuse of Valsalva - 4.3 cm
    • ST junction - 4.1 cm
    • mid-ascending aorta - 3.8 cm
A

Marfan syndrome

  • due to the presence of aortic root dilation and MVP
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21
Q

Differentiate Athletes heart from HCM

A
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22
Q

What is the most specific Echocardiographic feature in Chagas cardiomyopathy?

A

Normal LV cavity size with an apical aneurysm and LV thrombus

  • may occur with or without thrombus
  • RV aneurysms or biventricular aneurysms have also been reported
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23
Q

Describe findings of Chagas Cardiomyopathy

A
  • Acute and chronic myocarditis (appears years to decades later)
    • T. Cruzi from Reduviid bug
  • LV apical aneurysms with or without thrombus (most specific)
  • Late stages of disease –> LV dysfunction and heart failure
  • EKG findings:
    • RBBB
    • LAFB
    • AV block
    • PAB’s (multifocal)
  • EKG abnormalities –> wall motion abnormalities, apical aneurysms, LV dysfunction
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24
Q

What is the diagnosis?

Diagnostic criteria?

A
  • Apical HCM
  • LV apex > 15 mm
  • Apical to posterior wall thickness ratio > 1.5
  • Dimensions:
    • LV linear dimension in diastole is usually normal
    • LV cavity may be reduced
    • LA increased
    • Abnormal diastolic function
  • Absence of other etiologies of LVH

****Two forms: isolated (normal wall thickness except the apex) or mixed (increased wall thickness of nonapical segments, especially the septum)

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25
Q

What is one setting in HCM in which provocative maneuvers (with amyl nitrate) does not lead to LVOTO?

A

Isolated apical HCM

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26
Q

What are typical Echo and Doppler findings in Restrictive Cardiomyopathy?

A
  • Vp < 50 cm/s
  • Pulmonary vein systolic/diastolic velocity ratio < 0.5
  • DT < 160 ms
  • LAVI > 34 mL/m2
  • E/a ratio ≥ 2
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27
Q

What is the most important predictor of mortality in primary amyloidosis (AL) with cardiac involvement?

A

Increased LV wall thickness

  • Direct relationship between LV wall thickness > 15 mm and heart failure / mortality has been established
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28
Q

What are diastolic parameters associated with worse survival in primary Amyloidosis?

A
  • Mitral DT < 150 ms
  • Increased E/a and E/’e ratio
  • Increased LA volume
  • Abnormal Tei (MIP) index
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29
Q

Describe the findings and diagnosis

A
  • Hypereosinophilic syndrome
    • restrictive cardiomyopathy with endomyocardial involvement due to hypereosinophilia

****RV involvement in uncommon

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30
Q

What are the three stages of disease in hypereosinophilic syndome?

A
  • Toxic Endomyocardial Damage
    • Necrotic
    • Thrombotic
    • Fibrotic
      • if treated prior to this stage, reversibility may occur
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31
Q

What are the Echo findings in Hypereosniphilic syndrome?

A
  • obliteration of the LV and RV apex with thrombus/fibrosis
  • normal or small LV and RV cavity size
  • MR / TR due to papillary muscle involvement
  • Biatrial enlargement
  • Elevated right and left sided filling pressures (with restrictive filling pattern)
  • Merlon sign –> hypercontractile basal segment in contrast to the hypokinetic apical segment
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32
Q

Describe the pathophysiology in Fabry’s disease

A
  • X-linked lysosomal enzyme deficiency (alpha-galactosidase A)
  • results in progressive intracellular glycosphingolipid accumulation affective several organs including the heart
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33
Q

What are the characteristic findings in Fabry’s disease?

A
  • LV hypertrophy (generally symmetric)
  • Preserved LV systolic function
  • Normal LV dimensions
  • Impaired diastolic function
  • MR
  • Fibrosis and thinning of the basal inferolateral wall
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34
Q

What is a specific Echo finding that may help to differentiate Fabry’s disease?

A

Fibrosis and thinning of the basal inferolateral wall

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35
Q

Describe the findings and diagnosis

A

Hypertrophic Cardiomyopathy (suggestive)

  • crypt in the basal segment of the inferior wall
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36
Q

Describe the findings and diagnosis

A

ARVD

isolated RV dilatation of the RV apex (in the “triangle of dysplasia”)

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37
Q

What are common features of ARVD?

A

Fibrofatty ventricular replacement

Ventricular arrhtyhmias

Risk of SCD

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38
Q

Describe Echo features of ARVD

A
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39
Q

Describe the major and minor diagnostic criteria for ARVD

A
  • Major
    • RV akinesia, dyskinesis, or aneurysm
    • PLAX or PSAX RVOT dilatation
    • Reduced RV FAC (< 33%)
  • Minor
    • RV akinesis or dyskinesis
    • PLAX or PSAX RVOT dilatation
    • Reduced RV FAC (33% - 40%)
40
Q

Describe the findings and diagnosis

A

LV function is hyperdynamic and symptoms are due to diastolic dysfunction (on PW and CW doppler)

  • PW Doppler
    • Grade 1 diastolic dysfunction or impaired relaxation
  • CW Doppler
    • three distinct waves in diastole
      • first - intraventricular flow seen during the isovolumic relaxation period (IVRP)
      • second - low velocity early diastolic flow
      • third - higher velocity late-diastolic flow during atrial contraction
41
Q

What do findings of intraventricular flow seen during the isovolumic relaxation period (IVRP) indicate?

A
  • ​associated with normal or hyperdynamic systolic function seen in HCM, usually with cavity obliteration in the basal or midcavity at the level o fthe papillary muscles
  • Mechanism –> thought to be due to asynchronous relaxation of the midwall segments in the area of cavity obliteration

****First arrow

42
Q

What type of amyloidosis carries a more favorable prognosis than the AL subtype?

A

SSA or senile

43
Q

What subtype of Amyloidosis are associated with cardiac involvement?

A
  • AL (primary)
  • TTR (familial)
  • SSA (senile)
44
Q

Describe the findings and presentations associated with Amyloidosis subtypes

A
45
Q

Describe the findings

  • Ichemic cardiomyopathy
  • LVEF 20%
  • Bi-V PPM
A
  • CW Doppler demonstrating diastolic MR
  • Diastolic MR may occur during AV pacing when AV delay is too long
    • ​atrial contraction is completed befor the onset of ventricular contraction and therefore the mitral valve remains open but with a LV pressure >> LA pressure
  • Shortening AV delay –> improvement in symptoms related to diastolic MR
46
Q

Describe the findings and diagnosis

A

Hyertrophic Cardiomyopathy with LVOT obstruction

  • PW doppler
    • “lobster claw pattern” with midsystolic dip in velocity (white arrow)
    • sample volume is placed just apical to the point of LVOTO (SAM/MR)
      • initial high velocity followed by midsystolic drop in velocity during SAM/MR-septal contact when aortic afterload exceeds LV contractility
      • finding consistent with LVOTO, usually with PG > 60 mmHg
47
Q

Describe the findings and diagnosis

A

Cardiac Sarcoidosis

  • thinning of the basal anteroseptal and basal inferoseptal walls with normal mid anteroseptal and mid inferosptal thickness
48
Q

What is a relatively specific finding for cardiac sarcoidosis?

A

thinning of the basal anteroseptal wall

49
Q

Describe the findings and diagnosis

A

HCM (elderly patient)

  • more often have a localized proximal septal hypertrophy (septal bulge)
  • due in part to geometric changes in the heart with aging
  • This form is associated with sarcomeric mutations (~8% of patients)
  • More likely to present with symptoms due to LVOTO, though SCD is uncommon
50
Q

What other conditions may demonstrate thinning of the basal inferolateral wall?

A
  • Sarcoidosis
  • Muscular dystrophies
  • Fabry’s disease
  • Chagas disease
  • Freidrich ataxia
51
Q

Describe the findings and diagnosis

A

HCM in a young patient

  • asymmetric hypertrophy involving the septum with reversed septal curvature and RV hypertrophy
    *
52
Q

What is the most sensitive and specific finding for discriminating athlete’s heart from HCM in idividuals within the “gray zone” of LVH?

A

RWT < 0.6

  • most commonly used when patient’s fall in the gray zone
  • used since LV cavity is usually dilated in athlete’s heart
53
Q

What Echo modality can be very helpful in distinguishing between etiologies of increased LV wall thickness?

A

Speckle-tracking Longitudinal strain

  • characterizes increased wall thickness based on global and regional pattern
54
Q

Describe the findings and diagnosis in this patient with HCM

A

Stage 3 diastolic dysfunction

  • Restrictive filling pattern
    • E/a > 2
    • Mitral DT < 150 ms
    • E/e’ 40
    • LAVI 52 mL/m2
55
Q

What is the prevalence of a restrictive filling pattern (Grade 3 diastolic dysfunction) in HCM patients?

A

1-2%

  • restrictive phenotype of HCM (associated with specific genetic mutations)
56
Q

What is one way to differentiate HCM from other conditions presenting with SAM/MR?

A

CHF

  • unless there is LV dysfunction, AF or primary causes of severe MR most patients with HCM do not develop clinical signs of CHF
57
Q

Structures found in the right atrium that can be misinterpreted as a pathologic mass?

A
  • eustachian valve
  • chiari network (whip-like movement)
  • crista terminalis
58
Q

A structure in the left atrium that can be misinterpreted as a pathologic mass?

A

suture line following transplant

59
Q

Define Rhabdomyoma

A
  • benign cardiac tumor
  • usually small and lobulated, with diameters 2mm - 2cm in size
  • most often multiple
  • strong association: Tuberous Sclerosis
60
Q

What tumors are know to metastasize to the heart?

A
  • Renal cell carcinoma
  • Breast
  • Carcinoid
  • Melanoma
  • Lung
  • Lymphoma
61
Q

This tumor is unlikely to metastasize to the heart

A

Thyroid

62
Q

What cardiac tumor locations are associated with the greatest risk of embolization?

A

Aortic valve

and

Left atrial tumors

63
Q

What is the incidence of primary cardiac tumors?

A

< 1% (based on autopsy studies)

64
Q

What are the mechanisms by which cardiac tumors cause symptoms?

A
  • Embolism
  • Obstruction
  • Vavlular interference –> regurgitation
  • Direct invasion of the myocardium
    • leading to impaired contractility, arrhythmias, heart block, pericardial effusion
  • Constitutional / Systemic symptoms
65
Q

Describe syndromic relation with specific cardiac tumors

A
66
Q

What information can TEE provide with regards to valvular vegetations?

A

Predictive of embolic risk

  • Vegetation diameter > 10 mm –> significantly higher incidence of embolic events
    • association is strongest in those with mitral valve endocarditis
67
Q

What side of the valve are vegetations typically located?

A

“Upstream” side of the valve

  • characteristically prolapse into the upstream chamber
68
Q

Describe the findings of saline contrast (in assessment of PFO)

A

Timing of contrast can help distinguish intracardiac shunting

  • PFO
    • Early contrast appearance in the left heart ( < 3 beats of contrast appearance in the right heart) –> intracardiac shunting
  • Pulmonary AV shunting
    • Late shunting (after 3-5 beats) –> extracardiac shunting
69
Q

Describe common features of PFO

A
  • Asymptomatic, usually
  • 25% of population
  • Size and prevalence similar in males/females
70
Q

What are common symptoms associated with cardiac myxoma?

A

Dyspnea and fever

  • Most common symptom –> dyspnea
  • Common symptoms: embolization, obstruction, constitutional symptoms
  • Fever related to IL-6 production
  • Syncope and palpitations can also be seen
71
Q

Describe findings of Tuberous sclerosis

A
  • Autosomal-dominant hamartoma syndrome
  • Causative genes (TSC-1 and TSC-2) are tumor suppressor genes that encode a protein that regulates cell size
  • Rhabdomyomas are actually myocardial hamartomas (or malformations) that are composed of myocytes rather than true neoplasms
  • 80% of pateints with cardiac rhabdomyomas –> TS
  • 50% of rhabdomyomas regress spontaneously after infancy
72
Q

Describe common findings of papillary fibroelastomas

A
  • 90% are single
  • Median diameter ~ 8 mm with short pedicle 50% of time
  • Can occur on any valve
    • Valve dysfunction is rare
  • Aortic and mitral valves are most commonly involved in adults
  • Occur less commonly on:
    • papillary muscle, chordae tendinae, atria
73
Q

What is the most common malignant tumor of the heart?

A

Metastatic disease

  • 40 times more likely than primary malignant tumors
74
Q

What is a characteristic feature of a cardiac myxoma on 2D Echo?

A

A narrow stalk connected to the fossa ovalis

  • 75% of cardiac myxomas occur in the left atrium
    • 15-20% occur in RA
  • Site of attachment is almost always the region of the fossa ovalis of the interatrial septum
75
Q

This cardiac mass will appear as an intramural hyperechoic mass?

A

Lipoma

76
Q

Cardiac tumor commonly associated with AIDS?

A

Kaposi Sarcoma

77
Q

The majority of malignant tumors occur in this side of the heart?

What is the exception?

A

Right side

Leiomyosarcoma (left atrium)

78
Q

Describe Leiomyosarcoma findings

A
  • Presentation in 30’s
    • a decade earlier than other types of sarcomas
  • Only left sided cardiac malignant tumor
  • Smooth muscle cell proliferation
    • may originate from pulmonary veins
  • Poor prognosis ~ 7 months
    • even with surgery, chemotherapy and radiation
79
Q

Describe features of Angiosarcoma

A
  • Large or have metastasized at the time of diagnosis
  • Most commonly found in RA
  • Commonly associated with pericardial effusion
  • 3:1 male-to-femal ratio
  • Very poor prognosis
  • Often not amenable to complete resection
80
Q

Describe diagnosis

A
  • Synovial sarcoma
    • Malignant primary cardiac sarcoma
  • Caused by translocation between chromosome 18 and X chromosome
  • Extremelty rare with poor prognosis
81
Q

Describe the findings and diagnosis

A

Cystic structure

  • Lack of opacification (or uptake) –> lack of vascularity
    • Benign tumors (myxoma, papillary fiboelastoma) –> partial enhancement
82
Q

Describe features of Chiari network

A
  • Congenintal remnant of the right valve of the sinus venosus
    • network of fibers in the RA that originates from the region of the eustachian valve at the orifice of the IVC with attachements to the upper wall of the RA or atrial septum
  • Present in 2-3% of normal hearts
  • Not clinically significant
83
Q

Describe findings of pericardial cyst

A
  • Diagnosis can sometimes be suggested on CXR –> identification of a rounded mass along the right heart border
  • Rare to cause compressive symptoms and hymodynamic alterations
84
Q

Describe the findings and diagnosis

A

Myxoma - Left atrium (atrial septum attachment)

  • Most common primary cardiac tumor
  • Accounts for 20-30% of cardiac tumors
  • May be found as part of multisystem disease - Carney Complex
85
Q

What is the most common valve associated tumor?

A

Papillary fibroelastoma (accounts for 85%)

  • not easily distinguishable from vegetations (often confused)
  • do not typically result in vavluar regurgitation
  • most common presentation –> embolization (CVA or TIA)
86
Q

Where are papillary fibroelastoma’s most commonly attached?

A
  • Semilunar valves - arterial side
  • AV valves - atrial surface
87
Q

What are the treatment recommendations for papillary fibroelastoma’s?

A
  • Symptomatic –> Surgery
    • embolic events (CVA/TIA)
    • tumor mobility complications (coronary ostial occlusion)
    • highly mobile or large ( > 1 cm) tumors
  • Asymptomatic
    • immobile, small ( < 0.5cm) –> close follow up
    • highly mobile tumor –> consider surgery

***** tumor mobility is an independent predictor of death or nonfatal embolization

88
Q

When are papillary fibroelastomas not visible by TTE?

A

< 0.2cm in size

89
Q

What must be present for a patient to develop right sided carcinoid heart disease?

A

liver metastases

90
Q

What percentage of patients does left sided carcinoid heart disease occur?

What conditions makes this possible?

A
  • 10-15% of cases
  • Conditions leading to left sided carcinoid heart disease:
    • PFO
    • Bronchial carcinoid
    • High levels of circulating vasoactive substances
91
Q

What percentage of malignant melanoma cases involve the heart?

A

50%

92
Q

Describe the development of malignant melanoma with cardiac metastasis

A

Can be diagnosed and initially treated y ears prior to the development and discovery (of cardiac metastases)

93
Q

What is the most common cardiac extension of melanoma?

A

“Charcoal” heart

  • tumor studding the pericardial surface
94
Q

What is the sensitivity and specificity of TEE for detecting left atrial / LAA thrombus?

A

100% sensitivity

99% specificity

95
Q

What percentage of strokes are related to intracardiac sources?

A

15-20%

96
Q

What percentage of LAA thrombii demonstrate spontaneous echo contrast?

A

80%

97
Q

Describe Fibroma

A
  • Location: left ventricle free wall, septum or apex.
  • Well demarcated; often with elements of calcium deposits inside the mass.
  • Grows into cavity and may interfere with ventricular filling, causing heart failure or arrhythmias.
  • Often multiple.

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