Cardiac Flashcards
(330 cards)
1
Q
What is MC anomalous branching anatomy of aortic arch?
A
Common origin of the brachiocephalic artery and left common carotid artery?
13%
“Bovine aortic arch” - true bovine arch in cattle features a single great vessel arising from the aortic arch.
2
Q
What are the anomalous branching patterns of the aortic arch?
A
Common origin of the brachiocephalic artery and left common carotid artery - 13%
Aberrant right subclavian - 1%
Left vertebral origin off the aorta - 6%
3
Q
What is an aberrant right subclavian?
A
1% of patients
Right subclavian artery arises directly from the aortic arch distal to the left subclavian and loops behind the esophagus on its way into the right arm.
Uncommon to cause symptoms, but may cause dysphagia via esophageal compression, called dysphagia lusoria. Posterior indentation of the esophagus on esophagram.
Recurrent laryngeal nerve will not be in the usual location.
Diverticulum of Kommerel = small bulge at the origin of the aberrant subclavian artery.
4
Q
Posterior indentation of the esophagus on esophagram?
A
Aberrant right subclavian artery - can cause symptoms - dysphagia lusoria.
5
Q
What is Dysphagia Lusoria?
A
Esophageal compression due to an aberrant right subclavian artery.
6
Q
What is a Diverticulum of Kommerel?
A
Small bulge at the origin of the aberrant subclavian artery.
7
Q
What is acute aortic syndrome?
A
Clinical spectrum of three related diseases - damage to at least one component of the aortic wall - presents as severe chest pain.
Penetrating Atherosclerotic Ulcer - defect in the intima
Intramural Hematoma - Defect in the media only
Aortic Dissection - Defect in the intima extending to the media.
Defect in all 3 layers (aortic transection) is almost always due to trauma.
8
Q
What can cause disruption of the aortic intima?
A
Ulcerative plaque, trauma, or aneurysm.
9
Q
What is the key feature of an aortic dissection?
A
Disruption of the intima, which allows high-pressure blood to infiltrate and expand the media.
10
Q
Aortic dissection secondary to atherosclerosis is more common in Type
A
B
11
Q
What is an Intramural Hematoma?
A
Variant of dissection where blood collects w/in the media, without intimal flap to connect the intramural hematoma with the aortic lumen.
Thought to the due to rupture of the vasa vasorum, which are small blood vessels that supply the aortic wall.
HTN or trauma.
Clinically identical to aortic dissection.
Same treatment recommendations
Faint peripheral hyperattenuating (45-50 HU) crescent w/in the aorta - best seen on noncontrast CT - only done if suspicion of IMH.
12
Q
Cause of an intramural hematoma?
A
Rupture of vasa vasorum within the media - blood collects w/in the media - without intimal flap to connect the intramural hematoma with the aortic lumen.
13
Q
What is a Penetrating Atherosclerotic Ulcer?
A
Focal defect in the intima that occurs at the site of an atherosclerotic plaque - may cause media to enlarge, leading to anerysm formation.
May lead to saccular aneurysm formation.
In contrast to dissection and intramural hematoma, penetrating ulcers tend to be caused by atherosclerosis rather than HTN. Can lead to dissection.
Contrast ulcerating beyond the expected contour of the aortic wall. Different than simple ulcerated atherosclerotic plaque - which would not extend beyond the expected contour of the aortic wall.
14
Q
Cause of penetrating atherosclerotic ulcers?
A
Tend to be caused by atherosclerosis rather than HTN, incontrast to dissection and intramural hematoma.
15
Q
Difference between a penetrating atherosclerotic ulcer and simple ulcerated plaque?
A
PAU - Contrast ulcerating beyond the expected contour of the aortic wall.
Simple ulcerated atherosclerotic plaque - would not extend beyond the expected contour of the aortic wall
16
Q
What are the relatively fixed levels of the aorta where traumatic aortic injuries occur secondary to deceleration injury?
A
Aortic root
Isthmus - 95%
Hiatus
17
Q
What are the direct CT signs of traumatic aortic injury?
A
Dissection flap, pseudoaneurysm, and intramural hematoma.
Mediastinal hemorrhage that is separated from an intact aorta by a fat plane can be presumed to be venous - conservative treatment.
Hemorrhage in contact with the aortic wall is suggestive of aortic injury - surgical treatment.
18
Q
What is a thoracic aortic aneurysm?
A
Ascending aortic diameter >4 cm or descending thoracic aorta >3 cm. May also be normalized to BSA and compared to reference values.
Most are caused by atherosclerosis - the descending TAA more commonly affected. Almost 1/3 will have associated abdominal aortic aneurysm.
Non-atherosclerotic causes - connective tissue disorders (Marfan and Ehlers-Danlos syndromes), bicuspid aortic valve associated aortopathy, vasculitis (including Takayasu arteritis, giant cell arteritis, ankylosing spondylitis, and relapsing polychrondritis), cystic medial necrosis, and infectious aortitis.
19
Q
MC cause of thoracic aortic aneurysm?
A
Atherosclerosis.
Non-atherosclerotic causes - connective tissue disorders (Marfan and Ehlers-Danlos syndromes), bicuspid aortic valve associated aortopathy, vasculitis (including Takayasu arteritis, giant cell arteritis, ankylosing spondylitis, and relapsing polychrondritis), cystic medial necrosis, and infectious aortitis.
20
Q
Non-atherosclerotic causes of thoracic aortic aneurysm?
A
Non-atherosclerotic causes - connective tissue disorders (Marfan and Ehlers-Danlos syndromes), bicuspid aortic valve associated aortopathy, vasculitis (including Takayasu arteritis, giant cell arteritis, ankylosing spondylitis, and relapsing polychrondritis), cystic medial necrosis, and infectious aortitis.
MC cause is atherosclerosis.
21
Q
What is Annuloaortic Ectasia?
A
Dilated sinuses of Valsalva and ascending aorta with effacement of the sinotubular junction, resulting in a tulip bulb-shaped aorta.
Associated with Marfan and Ehlers-Danlos syndromes.
22
Q
Dilated sinuses of Valsalva and ascending aorta with effacement of the sinotubular junction?
A
Annuloaortic Ectasa - results in tulip bulb-shaped aorta.
23
Q
When is surgical treatment recommended for thoracic aortic aneurysms?
A
Ascending TAA >5.5 cm
Descending TAA >6 cm
However, patients with connective tissue disorders and bicuspid aortopathy (meeting criteria for valve replacement) have a lower surgical threshold of 4.5 cm.
Annual growth rate >1cm/year (or >5 mm/6months)
24
Q
What is the draped aorta sign?
A
Drooping of the posterior aorta against the spine on an axial image - sign of impending rupture.
25
Complications of thoracic aortic aneurysm?
Rupture, dissection, infection, endoleak, and paraplegia (caused by artery of Adamkiewicz occlusion)
26
What is an abdominal aortic aneurysm?
Aortic diameter >3 cm.
27
What is the annual risk of rupture of a AAA based on size?
5.5 - 5.9 cm is 9.4% annual risk
6.0 - 6.5 cm is 10.2%
6.5 - 6.9 cm is 19.2%
>7 cm is 32.5%
28
Follow-up recommendations for AAA
<4 cm - f/u in 6 months, if no change - annual surveillance
4-4.5 cm - f/u in 6 months, if no change - 6-month suveillance
5-5.5 cm - consider surgery
>5.5 cm - surgery recommended
29
When is surgery recommended for a AAA?
>5.5 cm and expanding at rapid rate (>5.5 mm/year) or symptomatic.
5-5.5 cm - consider surgery
Mortality of elective open repair is >3%
Urgent repair is 19%
Ruptured repair is at least 50%.
30
What are the complications of AAA endovascular repair?
Rupture, dissection, infection, endoleak, and aorto-enteric fistula
31
What is an endoleak?
Persistent flow into an excluded aneurysm sac after endovascular treatment with a stent graft.
32
What is a Type I endoleak?
Inadequate graft seal
IA is proximal
IB is distal
33
What is a Type II endoleak?
Persistent collateral flow to the excluded aneurysm sac - typically arises from the lumbar arteries or the inferior mesenteric artery.
34
What is a Type III endoleak?
Device failure causing leakage through graft fabric or segments of a modular graft.
Defect in the graft.
35
What is a Type IV Endoleak?
Caused by a porous graft and is typically transient and seen intra-procedurally.
Usually resolves w/in 1 month after withdrawl of anticoagulation. Rarely seen in modern grafts.
36
What is a Type V endoleak?
Also called endotension - continued expansion of the aneurysm w/o any other endoleak present - thought to be due to endoleak below the resolution of imaging.
37
What is the complication of infectious aortitis?
Development of a mycotic aneurysm.
38
Causes of inflammatory aortitis?
Takayasu arteritis, giant cell arteritis, ankylosing spondylitis, polyarteritis nodosa, rheumatoid arthritis, and immune complex disease.
39
MRI findings of active aortitis?
Aortic wall thickness >2 mm and enhancement of the aortic wall.
Circumferential mural thickening and enhancement. In contrast to intramural hematoma, aortitis tends to cause circumferential thickening than the eccentric, crescentic thickening of IMH.
Chronic phase will cause long segment stenosis and/or aneurysms.
40
What is Takayasu Arteritis?
Idiopathic, inflammatory, large-vessel vasculitis - involves the thoracic and abdominal aorta, subclavian arteries, carotid arteries, pulmonary arteries, and large mesenteric arteries.
Pulseless disease.
Young to midle-aged women.
Long smooth stenoses are classic. Imaging is often indistinguishable from giant cell arteritis - patients age is the main distinguishing factor.
TA is younger patients
Giant cell arteritis is rare in patients under 50
41
How to tell difference between Takayasu Arteritis and Giant Cell Arteritis?
Often indistinguishable based on imaging. Both cause long smooth stenoses.
Age is main distinguishing factor:
TA is younger patients
Giant Cell Arteritis is rare in patients under 50.
42
What is the difference between the adult and infant forms of coarctation?
Adult - juxtaductal (at the junction of the ductus arteriosus) - leading to upper extremity hypertension.
Infant - Presenting with CHF due to coarctation is usually due to a preductal variant, which functions as a left ventricular obstructive lesion.
43
What is pseudocoarctation of the aorta?
Focal narrowing of the aorta, similar in morphology to true coarctation, but no pressure differential and thus no collaterals.
44
How much stenosis is coronary CT sensitive for?
>50% luminal narrowing.
Stenosis found on CT may be overcalled, especially if calcified plaque, which can cause a blooming artifact.
45
How do you estimate radiation dose for coronary CT?
Multiply the dose-length product (DLP) by a conversion factor of 0.017 to arrive at the dose in millisieverts
46
What is retrospective coronary gating?
Continuous CT scanning is performed throughout the cardiac cycle and images are correlated to the ECG cycle afterwards.
Main advantage - ability to create cine reconstructions to evaluate cardiac and valvular function.
Main disadvantage - significant increase in radiation exposure compared to a prospectively gated study.
47
What is prospective coronary gating?
ECG is used to time image acquisition at a specific phase of the cardiac cycle - exposing patient to radiation only during this segment of the cardiac cycle.
Main advantage- decreased radiation exposure
No cine reconstructions.
48
What is the resolution of coronary CT vs coronary artery luminal diameter?
Resolution of 0.35 to 0.5 mm
Coronary arteries have an average luminal diameter of about 3 mm.
Only have resolution to grade a stenosis with accuracy greater than about 20% of the diameter.
49
What categories can coronary artery stenosis be classified as?
<20%
20-50%
50-70%
>70%
>50% is considered potentially hemodynamically significant.
<50% is considered not hemodynamically significant.
50
How can you increase temporal resolution with coronary CT?
Need slightly more than 180 degrees of rotation for image acquisition.
Dual source CT decreases temporal resolution.
51
What drugs are given for coronary CT?
PO Metoprolol for HR <60.
Sublingual nitroglycerin (0.5 - 0.8 mg) to dilate coronary arteries.
52
What are the three coronary sinuses?
Right (anterior)
Left (lateral posterior)
Non-coronary (right posterior)
53
Where does the left main coronary artery course?
Between the pulmonary artery and the left atrial appendage.
Branches to LAD, L circumflex, and sometimes Ramus
54
What are the branches of the left main coronary artery?
LAD and L circumflex
Ramus branch may be present to form a trifurcation.
55
What is the course of the LAD?
In the anterior interventricular groove.
Gives off diagonal branches (LAD - Diagonal) and septal branches.
56
What are the branches of the LAD?
Diagonal (LAD - Diagonal) and septal branches
57
What is the course of the Left Circumflex coronary artery?
Courses underneath the L atrial appendage (LAA) in the left AV groove (between the left ventricle and left atrium).
Gives off the Obtuse Marginal (OM; circOMflex) branch - which supplies the posterolateral wall of the LV. The angle of the lateral wall of the obtuse marginal artery is obtuse, hence the name
Uncommonly (~7%) supplies the posterior descending artery (PDA) - left-dominant system
58
What are the branches of the Left Main Coronary Artery distally?
Left main -
LAD, Circumflex, and Ramus
LAD -
Diagonal (LAD - Diagonal) and septal branche
Left Circumflex -
Obtuse Marginal
Posterior Descending Artery (~7%)
59
What is the course of the right coronary artery?
Within the right atrioventricular groove - mirrors the LCs in course.
60
Branches of the right coronary artery?
Conal (supplies the RVOFT)
SA nodal branch (supplies the SA node)
Acute Marginal Branch - courses anterior to the RV free wall and muscular branches to supply the RV fee wall
AV nodal branch - branches as the crux (junction of the four chambers)
Posterior descending artery (85%) - to supply the posterior half of the ventricular septum.
Posterolateral artery - supplies the posterior left ventricle
61
What is the terminal branch of the RCA and what does it do?
Posterolateral artery - supplies the posterior left ventricle.
62
What does the obtuse marginal branch supply?
Branch of the left circumflex artery - supplies the posterior lateral wall of the LV.
63
What does the acute marginal branch supply?
Branch of the RCA - supplies the RV free wall
64
What does the posterior descending artery supply?
Branch of the RCA - supplies the posterior half of the ventricular septum
65
What does the posterolateral artery supply?
Terminal branch of the RCA - Supplies the posterior left ventricle.
66
What is a malignant coronary artery anomaly?
Carries increased risk of sudden death (in up to 40%), often associated with exercise.
Can be malignant (if arising from the pulmonary artery) or potentially malignant (depending on course)
67
What determines if a coronary artery anomaly is malignant or potentially malignant?
Malignant - arising from the pulmonary artery
Potentially Malignant - depending on course
68
What are the malignant coronary artery anomalies?
CA arising from the pulmonary artery = malignant
ALCAPA - anomalous left coronary artery from the PA
RCAPA - anomalous right coronary artery from the PA
69
What are the potentially malignant coronary artery anomalies?
RCA arising from left coronary sinus
Left main arising from the right coronary sinus
Left circumflex or LAD arising from right coronary sinus
Any artery arising from the noncoronary sinus.
```
Interarterial coure (between the aorta and PA) of an anomalous CA is malignant
Retroaortic, prepulmonic, and septal coronary artery course are all considered benign.
```
70
What is an intramural course of a coronary artery?
When the vessel courses through the wall of the aorta for a short segment. Is associated with sudden death.
Slit-like configuration on CCTA.
Tx is bypass, reimplantation, or unroofing procedure when opens and enlarges the ostium from inside the aorta.
71
What is Bland-White-Garland Syndrome?
ALCAPA - Anomaous Left Coronary from the Pulmonary Artery
| 90% mortality
72
What is myocardial bridging?
Band of myocardium overlying a segment of a coronary artery - MC seen in the mid LAD.
Usually asymptomatic - may cause angina, MI, or even death.
If present and thought to be cause of patients symptoms, further evaluation is recommended with exercise myocardial perfusion
73
Sequences used in cardiac MRI
Steady-sate free precession (SSFP) and/or gradient echo cine sequences
Tissue characterization is typically performed with double or triple inversion fast spin echo sequences - fat suppression
SSFP = "white blood" - excellent contrast between myocardium and blood pool. High temporal resolution and excellent contrast.
74
What is the difference between first-pass and delayed contrast-enhanced MRI?
First-pass contrast-ehanced perfusion MRI is performed pre and post vasodilator stress to evaluate myocardial perfusion. Normal myocardium enhances, while areas of decreased perfusion will be relatively hypoenhancing.
Delayed contrast-enhanced MRI is used to image changes in myocyte to interstitial space ratio. This ratio decreases most commonly after myocardial infarction - myocytes replaced with scar tissue. Any delayed enhancement in DE-MR is abnormal and represents an increase in extracellular volume fraction.
75
What does delayed contrast-enhanced MRI look for?
Used to image changes in myocyte to interstitial space ratio.
Ratio decreases most commonly after myocardial infarction - myocytes replaced with scar tissue. Any delayed enhancement in DE-MR is abnormal and represents an increase in extracellular volume fraction.
76
What are the nonischemic types of delayed enhancement?
Mid-myocardial delayed enhancement - Dilated cardiomyopathy, sarcoidosis, chagas disease, and hypertrophic cardiomyopathy.
Epicardial/subepicardial delayed enhancement - Myocarditis, Chagas, Sarcoidosis
Circumferential subendocardial delayed enhancement - Amyloidosis and cardiac transplant
77
What are the causes of mid-myocardial delayed enhancement?
Dilated cardiomyopathy
Sarcoidosis - nodular or patchy pattern
Chagas Disease
Hypertrophic Cardiomyopathy - at junction of IV septum and RV free wall.
78
What is dilated cardiomyopathy and what type of delayed enhancement does it cause?
Most common nonischemic cardiomyopathy - most commonly idiopathic, but may be caused by alcohol abuse, myocarditis, or drug toxicity.
Mid-myocardial delayed enhancement.
Will see diffuse chamber enlargement and reduced EF.
79
What is Sarcoidosis and what type of delayed cardiac enhancement does it cause?
Systemic disease of noncaseating granulomas with cardiac manifestations of arrhythmias, LV dysfunction, and restrictive cardiomyopathy. Usually see cardiac findings in conjunction with other manifestations of sarcoid - lung disease and adenopathy.
Mid-myocardial or subepicardial delayed enhancement in a nodular or patchy pattern.
80
What is Chagas Disease and what type of delayed cardiac enhancement does it cause?
Caused by protozoan Trypanosoma cruzi - can lead to cardiomyopathy.
Epicardial or mid-myocardial delayed enhancement.
81
What is hypertrophic cardiomyopathy and what type of delayed enhancement does it cause?
Abnormal LV myocardial thickening w/o dilation. Pathologic thickening may be diffuse or focal. Can cause sudden death.
Mid-myocardial delayed enhancement in regions of hypertrophied myocardium and at the junctions of the IV septum and the RV wall - due to myofibril disarray.
Will see reduced diastolic filling of the LV on cine clips.
82
What are the causes of Epicardial/subepicardial delayed enhancement?
Myocarditis
Chagas Disease
Sarcoidosis
83
What diseases can cause both epicardial/subepicardial and mid-myocardial delayed enhancement?
Chagas Disease
| Sarcoidosis
84
What is myocarditis and what type of delayed enhancement does it cause?
Inflammation of the myocardium - multiple causes, viral infection is the most common, followed by autoimmune disorders and drug toxicity.
Subepicardial delayed enhancement. Will also see wall motion abnormalities in the affected regions.
85
What are the causes of circumferential subendocardial delayed enhancement?
Amyloidosis
| Cardiac Transplant
86
What is Amyloidosis and what type of delayed cardiac enhancement does it cause?
Disorder of glycoprotein deposition throughout the extracellular spaces.
Can cause biventricular myocardial thickening, leads to diffuse ventricular subendocardial delayed enhancement.
87
What type of delayed cardiac enhancement can be seen after cardiac transplant?
Circumferential subendocardial delayed pathological enhancement, thought to correlate with the presence of myocardial fibrosis pathologically.
88
What are the cardiovascular diseases with an enlarged cardiac silhouette?
Cardiomyopathy 2/2 congestive heart failure, valvular regurgitation (aortic, mitral, tricuspid regurgitation), high output or volume overload states, dilated cardiomyopathy, pericardial effusion, and paracardiac mass.
89
What are the cardiovascular diseases with a normal-sized cardiac silhouette?
Valvular stenosis (aortic or mitral), pulmonary artery HTN, hypertrophic cardiomyopathy, restrictive physiology, and acute MI.
90
What are the key structures to look at to distingush between the various causes of enlarged heart cardiac disease?
Left atrium and Aorta.
Enlarged left atrium - suggests mitral regurgitation
Enlarged aorta - suggests aortic regurgitation.
If neither LA or aorta is enlarged - suggests one of the other etiologies.
91
What are the key structures to look at to distingush between the various causes of normal sized heart cardiac disease?
Left atrium and aorta
Enlarged left atrium and cardiac silhouette is normal - suggests mitral stenosis.
Enlarged aorta and cardiac silhouette is normal - suggests aortic stenosis or aortic aneurysm.
If neither the LA or aorta is enlarged - suggests one of the other etiologies.
92
What is the most anterior cardiac chamber?
RV
Enlargement causes displacement of the cardiac apex in a leftward direction (in contrast to LV enlargement - causes displacement in a left-inferior direction).
Can cause opacification of the retrosternal clear space on the lateral radiograph.
93
What chamber forms the right heart border?
RA
RA enlargement causes lateral bulging or elongation of the right heart border.
94
What chamber forms the left heart border?
LV
LV enlargement causes displacement of the cardiac apex in the left-inferior direction.
Hypertrophic cardiomyopathy does not cause enlargement of the external contour of the ventricle.
95
What is the most posterior cardiac chamber?
LA
Enlargement may be caused by mitral regurgitation (with an enlarged cardiac silhouette) or mitral stenosis (with a normal cardiac silhouette)
Enlarged LA may splay the carina, seen on the frontal view. On lateral radiograph, an enlarged LA can elevate the left upper lobe bronchus.
Double density - right aspect of the enlarged LA visualized through the RA.
96
Difference between a true LV aneurysm and a pseudoaneurysm?
True LV aneurysm is focal outpouching of ventricular wall with all layers of the muscular wall affected. True aneurysms are associated with occlusion of the LAD.
False aneurysm (pseudoaneurysm) - contained ventricular rupture with only pericardial adhesions preventing a complete rupture - no myocardium in the wall of a false aneurysm. Associated with LCx and RCA occlusion. Surgical treatment.
97
Occlusion of which vessels causes LV aneurysm vs pseudoaneurysm?
Aneurysm - LAD
Pseudoaneurysm - LCx and RCA.
98
What is Dressler Syndrome?
Autoimmune pericarditis - often associated with pericardial and pleural effusions.
99
What are Mitral Annulus Calcification associated with?
Mitral regurgitation - can be associated with increased risk of stroke, adverse cardiovascular events, and atrial fibrillation. Also considered a risk marker for CV disease.
Unlike mitral valve calcifications, MAC is not associated with mitral stenosis.
100
What is Takotsuba Cardiomyopathy?
Catecholamine Induced Cardiomyopathy - broken heart syndrome.
Older women in the setting of acute emotional stress- can present as chest pain, abnormal ECG, and elevation of cardiac enzymes. Cardiac cath is normal. Self-limited
Characteristic ballooning of the cardiac apex. No abnormal delayed enhancement on MRI.
101
What causes ballooning of the cardiac apex?
Takotsuba/Catecholamine cardiomyopathy.
102
What is Arrhythmogenic Cardiomyopathy?
Previoiusly called arrhythmogenic RV dysplasia
Fibrofatty replacement of the ventricular myocytes, causing focal contraction abnormalities and/or aneurysm formation.
Diagnosis is difficult - imaging plays a supportive role - look for RV enlargement or presence of focal aneurysm. Presence of focal fat is no longer in the criteria as fat can be seen in normal individuals with aging.q
103
What is Myocardial Noncompaction?
Developmental defect in embryologic formation of the LV - due to failure of part of the LV to form a solid myocardium.
Have increased risk of adverse cardiac events - arrhythmias, thrombus formation, stroke, and cardiomyopathy.
LV appears heavily trabeculated as the RV with relatively thin LV.
104
What is hypertrophic cardiomyopathy?
AD cardiomyopathy with hypertrophic LV myocardium. MC cardiomyopathy.
The asymmetric septal hypertrophy variant - idiopathic hypertrophic subaortic stenosis - may cause LVOT obstruction.
Wall thickness of >15mm and a ratio of >1.5 compared to the lateral wall. Wall thickness of >30mm is an indication for ICD placement
Systolic anterior motion of the anterior leaflet of the mitral valve can cause mitral regurgitation and resultant LA enlargement.
105
What is Idiopathic Hypertrophic Subaortic Stenosis?
Asymmetric septal hypertrophy variant - may cause LVOT obstruction.
Wall thickness >15 mm and a ratio of >1.5 compared to the lateral wall. A wall thickness >30 mm is an indication for ICD placement.
106
What is restrictive cardiomyopathy?
Small, stiff, thickened ventricles that impair diastolic filling. Results in dilated atria and ultimately a dilated IVC.
May be idiopathic or due to sarcoidosis, hemochromatosis, or myocardial deposition diseases (e.g. amyloidosis).
Restrictive cardiomyopathy and constrictive physiology are different entities.
Role of imaging is to exclude constrictive pericarditis (can be treated surgically) as the etiology of the diastolic dysfunction.
107
What is Dilaated Cardiomyopathy?
Concentric ventricular chamber enlargement with impaired systolic function. Typically both ventricles enlarged.
Can be ischemic or idiopathic in etiology - MRI or CT is useful to determine etiology.
108
What is Lipomatous Hypertrophy of the interatrial septum?
Proliferation of fatty deposits w/in the interatrial septum - typically along the lateral right heart border.
Incidental finding - no treatment.
109
Where is lipomatous hypertrophy of the interatrial septum occur?
lateral right heart border
110
What is abnormal thickness of the pericardium?
<2 mm = normal
| >4 mm = abnormal.
111
What are common causes of pericardial calcification?
Prior pericarditis - MC viral or uremic etiology.
112
What is congenital absence of the pericardium?
Ranging from focal defect to complete absence of the R and L pericardium. Total absence is rare.
MC involves the L pericardium in the region of the LA appendage and adjacent PA.
Partial absence = at risk for herniation of a portion of the heart through the defect.
Defects in the L pericardium can cause leftward displacement of the heart, which may in some cases be the only imaging finding.
113
What is the difference between a vascular sling and ring?
Complete encircling of the trachea and esophagus by the aortic arch or great vessels = vascular ring.
A sling refers to an anomalous course of the left pulmonary artery - which arises aberrantly from the right pulmonary artery and traps the trachea in a "sling" on three sides.
114
What is a vascular ring?
Complete encircling of the trachea and esophagus by the aortic arch or great vessels
115
What is a vascular sling?
Anomalous course of the left pulmonary artery which arises aberrantly from the right pulmonary artery and traps the trachea in a "sling" on three sides.
116
What is an important clue to a potential vascular cause of stridor?
Right sided aortic arch.
The PA sling is the only vascular anomaly that causes stridor in a patient with a normal (left) aortic arch.
117
What is the only vascular anomaly that causes stridor with a normal (left) aortic arch?
PA sling
118
What are the three most important vascular causes of stridor?
Double aortic arch, right arch with aberrant left subclavian, and pulmonary sling.
Each will show abnormality on the lateral radiograph or esophagram - Double arch and right arch with aberrant left subclavian look the same on radiography/esophagram - each producing a posterior impression on the esophagus.
119
What is the most common vascular ring?
Double aortic arch.
120
What is a double aortic arch?
MC vascular ring.
The arches encircle both the trachea and esophagus, and may cause stridor.
Right arch is usually superior and larger in caliber than the left - imaging to determine which is dominant - surgery will ligate the non-dominant arch.
Will see posterior indentation of the esophagus (by the right arch) and anterior indentation of the trachea (by the left arch)
121
What is a right arch with an aberrant left subclavian?
2nd MC vascular ring.
The right arch indents the anterior trachea while the aberrant left subclavian wraps posteriorly around the esophagus.
The ring is completed by ligamentum arteriosum.
122
What makes up the vascular ring with a double aortic arch?
Right arch indents the posterior esophagus.
Left arch indents the anterior trachea.
123
What makes up the vascular ring with a right arch with an aberrant left subclavian?
The right arch indents the anteiror trachea.
Aberrant left subclavian wraps posteriorly around the esophagus.
The ring is completed by the ligamentum arteriosum.
LEFT ARCH WITH ABERRANT RIGHT SUBCLAVIAN IS USUALLY ASYMPTOMATIC AND NOT A VASCULAR RING.
124
What is a Pulmonary Sling?
Anomalous left pulmonary artery arising from the right pulmonary artery - forms a sling by coursing in between the trachea and esophagus.
Usually only the trachea is trapped in the sling, but occasionally the bronchus intermedius may also be compressed.
Pulmonary artery sling is the only vascular cause of stridor in a patient with a left arch. Aortic branching pattern is normal.
Associated with tracheal anomalies including tracheomalacia and bronchus suis (RUL bronchus originating from the trachea).
125
What is the only vascular cause of stridor in a patient with a left arch?
Pulmonary artery sling
126
What is Bronchus Suis?
RUL bronchus originating from the tracha.
Associated with pulmonary artery sling.
127
What indents the posterior esophagus and anterior trachea with a double aortic arch?
Posterior esophagus - right arch
| Anterior trachea - left arch
128
What indents the posterior esophagus and anterior trachea with a right arch with aberrant left subclavian artery?
Posterior esophagus - aberrant left subclavian
| Anterior trachea - right arch
129
Does a left arch with aberrant right subclavian cause stridor?
No. Passes posterior to the esophagus. Not a ring.
Dysphagia Lusoria - aberrant right subclavian passes posterior to esophagus.
130
What is Innominate Artery Syndrome?
In infants, the large thymus can occasionally cause the normal innominate artery to press against the anterior trachea.
Not a vascular ring.
131
Cause of posterior impression on esophagus with anterior tracheal impression?
Double aortic arch and Right arch with aberrant left subclavian - appear identical on lateral radiograph/esophagram.
132
Cause of posterior impression on esophagus with no anterior tracheal impression? Can be asymptomatic or have dysphagia?
Left arch with aberrant right subclavian.
133
What is the only vascular ring or sling to produce an anterior impression on the esophagus?
Pulmonary artery sling.
Aberrant PA runs between the trachea and esophagus - it also produces a posterior tracheal impression.
Only congenital vascular cause of stridor with a left aortic arch.
134
What piece of history is needed with evaluating pediatric congenital heart disease?
Cyanotic or acyanotic.
Then look at the pulmonary vasculatiry - increased venous flow, increased arterial flow, or decreased arterial flow.
135
What category of pediatric heart conditions causes increased pulmonary venous flow (pulmonary edema)?
When the LVOT can't keep up with the venous return
Left heart insufficiency, an obstructive lesion of the left heart, or CHF.
Peripheral 1/3 of the lungs shows indistinct vessels and septal markings.
136
What category of pediatric heart conditions causes increased pulmonary arterial flow ("shunt vascularity")?
Increased arterial flow - left --> right shunt - right side of the heart is pumping too much blood.
Peripheral 1/3 of the lungs shows distinct, large-caliber vessels.
137
What category of pediatric heart conditions causes decreased pulmonary arterial flow?
RVOT insufficiency
Decreased pulmonary arterial flow is ALWAYS cyanotic b/c not enough blood is being oxygenated.
Peripheral 1/3 of the lungs shows decreased vasculature.
138
What can be other causes of an enlarged heart with abnormal pulmonary vascularity, besides congenital heart disease?
Intracardiac tumor, such as rhabdomyoma
CHF due to peripheral shunt (e.g. vein of Galen malformation or hepatic hemangioendothelioma)
Mediastinal mass
Congenital diaphragmatic hernia with unaerated bowel.
139
What are the Acyanotic congenital heart diseases?
With increased pulmonary venous flow (pulmonary edema):
Hypoplastic left heart, aortic coarctation, CHF, Neonatal sepsis
Increased pulmonary arterial flow (shunt vascularity):
ASD, VSD, PDA, and endocardial cushion defect.
140
What are the acyanotic congenital heart diseases with cause increased pulmonary venous flow (pulmonary edema)?
Hypoplastic left heart - similar physiology to CHF (LV cannot keep up with venous return)
Aortic coarctation
CHF - may be due to a primary cardiac anomaly or an extra-cardiac AV shunt, such as vein of Galen malformation or hepatic hemangioendothelioma.
Neonatal Sepsis - typically seen in the setting of neonatal pneumonia.
141
What are the causes of acyanotic congenital heart disease with increased pulmonary arterial flow (shunt vascularity)
ASD
VSD
PDA
Endocardial cushion defect - also known as an AV canal defect - has a strong association with Down syndrome.
142
What are the cyanotic congenital heart diseases?
With decreased pulmonary vascularity with cardiomegaly:
Ebstein anomaly
With decreased pulmonary vascularity without cardiomegaly:
Tetralogy of Fallot
With usually increased pulmonary vascularity without cardiomegaly: "T lesions"
Transposition of great arteries
Truncus arteriosus
Tricuspid atresia
Total anomalous pulmonary venous return
Single "Tingle" Ventricle and variants including double outlet LV and double outlet RV.
143
What is hypoplastic left heart syndrome?
Spectrum of congenital heart anomalies - underdevelopment of any part of the left heart including the LA, mitral valve, LV, aortic valve, or aorta.
Survival depends on a PDA with right to left shunting to supply the systemic circulation.
Heart is normal size - not small
Surgical treatment is with the Norwood procedure.
144
What is survival with hypoplastic left heart syndrome dependent on?
PDA
145
What is the surgical procedure used to fix hypoplastic left heart syndrome?
Norwood Procedure - performed in three sequential stages - necessary b/c high pulmonary vascular resistance in the neonatal period precludes immediate definitive repair.
Stage 1:
RV is redirected to supply the systemic circulation - reconstruct the aorta using the main PA (Damus-Kaye-Stansel Procedure)
The atrial septum is excised and the PDA is ligated to redirect the pulmonary venous return to the right heart via the atrial septal defect.
Pulmonary arterial circulation is provided by a modified Blalock-Taussig shunt - connects the right subclavian to the right PA via a prosthetic graft.
Stage 2:
The Blalock-Taussig shunt is replaced with a bidirectional Glenn shunt, where the SVC is connected to the right PA (done once PA resistance has fallen to normal levels.
Stage 3:
Modified Fontan - performed once the patient cannot supply adequate oxygenated blood to the systemic circulation - A tunneled conduit connects the IVC to the PA. The SVC continues to empty into the right PA via the bidirectional Glenn from stage 2.
146
In a Norwood procedure to repair hypoplastic left heart syndrome, what is the procedure to reconstruct the aorta using the main PA?
Damus-Kaye-Stansel Procedure
147
In a Norwood procedure to repair hypoplastic left heart syndrome, what is the procedure to connect the right subclavian artery to the right PA?
Blalock-Taussig shunt
148
What is used to replace a Blalock-Taussig shunt in a Norwood procedure to repair hypoplastic left heart syndrome?
Bidirectional Glenn - SVC is connected to the right PA.
149
In a Norwood procedure to repair hypoplastic left heart syndrome, what procedure connects the IVC to the pulmonary artery?
Modified Fontan Procedure
150
What is a Damus-Kaye-Stansel Procedure?
Reconstruct the aorta using the main PA - redirect the RV to supply the systemic circulation.
Used in a Norwood procedure to repair hypoplastic left heart syndrome
151
What is a Blalock-Taussig Shunt?
Connects the right subclavian artery to the right PA to supply pulmonary arterial circulation via a prosthetic graft.
Used in a Norwood procedure to repair hypoplastic left heart syndrome
152
What is a Bidirectional Glenn Procedure?
SVC is connected to the right PA.
Used in a Norwood procedure to repair hypoplastic left heart syndrome
153
What is a Modified Fontan Procedure?
A tunneled conduit connects the IVC to the PA.
Used in a Norwood procedure to repair hypoplastic left heart syndrome
154
What are the two most common causes of extracardiac shunt causing CHF?
Vein of Galen malformation and hepatic hemangioendothelioma.
155
What are ASDs rarely associated with?
Holt-Oram syndrome - ASD with upper extremity bone deformities, including absence or hypoplasia of the thumb.
156
What is Holt-Oram Syndrome?
ASD with upper extremity bond deformities, including absence of hypoplasia of the thumb.
157
What is the MC type of ASD?
Ostium secundum ASD - 75% - incomplete covering of the ostium secundum by the septum secundum.
158
What causes an ostium secundum ASD?
MC type of ASD - 75%.
Incomplete covering of the ostium secundum by the septum secundum.
159
What causes an ostium primum ASD?
Second most common type of ASD - 15%
Incomplete fusion of the septum primum to the endocardial cushion
160
What is the two types of VSD - which is most common?
Membranous and Muscular
Membranous is MC.
161
What makes up endocardial cushion defects?
Spectrum of abnormalities including ostium primum ASD, VSD, and mitral or tricuspid anomalies.
Strongly associated with trisomy 21.
162
What is the primitive endocardial cushion responsible for forming?
Posterior and membranous ventricular septum
Anterior leaflet of the mitral valve
Septal leaflet of the tricuspid valve
Responsible for closure of the ostium primum
163
What is Ebstein Anomaly?
Severe malformation of the tricuspid valve characterized by apical displacement of the septal and posteroinferior leaflets, resulting in obstruction of the pulmonic valve and RVOT obstruction.
"Atrialization" of the RV.
ASD is always present.
Huge heart with massive RA enlargement with decreased pulmonary vascularity - Box-shaped heart.
164
What causes a "box-shaped heart"?
Ebstein Anomaly
Huge heart with massive RA enlargement with decreased pulmonary vascularity.
165
What are the cyanotic heart diseases with decreased pulmonary vascularity with cardiomegaly?
Ebstein Anomaly
| Pulmonary Atresia with intact ventricular septum
166
What is Pulmonary Atresia with Intact Ventricular Septum?
Appears identical to Ebstein Anomaly on radiography with massive RA enlargement with decreased pulmonary blood flow.
Lack of VSD markedly reduced blood flow to the lungs, pulmonary blood flow depends on the L to R shunt, such as patent ductus.
167
What are the cyanotic heart diseases with decreased pulmonary vascularity without cardiomegaly?
Tetralogy of Fallot (ToF)
168
What are the four components of Tetralogy of Fallot?
1. RVOT obstruction
2. RV hypertrophy
3. VSD
4. Aorta is "over-riding" the VSD.
Pentalogy of Fallot - which also has an ASD.
169
What is Pentalogy of Fallot?
Tetralogy plus an ASD.
170
What determines the degree of cyanosis with ToF?
Degree of RVOT obstruction
171
What is ToF associated with?
DiGeorge syndrome (absence of thymus and parathyroids, causing hypocalcemia), VACTERL, and trisomy 21.
172
What is Tetralogy of Fallot?
1. RVOT obstruction
2. RV hypertrophy
3. VSD
4. Aorta is "over-riding" the VSD.
Degree of stenosis depends on degree of RVOT obstruction.
MC cyanotic heart disease of children and adults.
Associated with DiGeorge syndrome (absence of thymus and parathyroids, causing hypocalcemia), VACTERL, and trisomy 21.
Boot-shaped heart (cardiac apex uplifted by RV hypertrophy, but normal size) and decreased pulmonary vascularity.
25% have right-sided aortic arch
173
25% of ToF patients have what?
Right-sided aortic arch
174
What is the MC cause of cyanotic heart disease in newborns?
Transposition of great arteries.
175
Findings of transposition of great arteries on imaging?
US shows aorta anterior to the heart and great vessels
"Egg-on-a-string" appearance with a narrow mediastinal waist - caused by configuration of the great vessels and thymic involution secondary to stress, lack of main PA bulge, and parallel course of the aorta and PA
176
Surgical procedure used to fix transposition of great arteries?
Jatene procedure - swaps the aorta and PA, relocates the coronary arteries.
177
What is a Jatene Procedure?
Used to fix transposition of great vessels - swaps the aorta and PA, relocates the coronary arteries.
178
What is Tricuspid Atresia and what does it look like?
Absence of Tricuspid valve
Variable appearance depending on size of the associated VSD.
Small VSD: Normal size heart with decreased pulmonary vasculature.
Large VSD: Enlarged heart with increased pulmonary flow
179
Causes of cyanotic heart diseases with increased pulmonary vascularity?
```
Transposition of Great Arteries
Tricuspid Atresia
Truncus Arteriosus
Total Anomalous Pulmonary Venous Return
Single "Tingle" Ventricle
```
180
What is Truncus Arteriosus?
A single great artery arises from the base of the heart to supply the systemic, pulmonary, and coronary circulations.
The single great artery usually over-rides a VSD.
MC type of congenital heart disease to have a right arch (21-36% of cases)
Cardiomegaly, narrow mediastinum, and pulmonary edema.
181
What is the MC type of congenital heart disease to have a right arch?
Truncus Arteriosus
182
What is Total Anomalous Pulmonary Venous Return?
All pulmonary veins connect anomalously to the systemic venous circulation instead of draining into the LA.
Has an obligate R-to-L communication enabling blood to reach the left heart, typically an ASD.
50% supracardiac - at or above the level of the SVC. Snowman sign - common confluence of the pulmonary veins drains into the L inominate vein via a vertical vein. The dilated vertical vein making up the left border of the snowman.
Cardiac - 20% - Anomalous drainage into the coronary sinus or RA.
Infracardiac - 20% - Anomalous drainage passes through the diaphragm via the esophageal hiatus and then drains into the hepatic IVC, hepatic vein, or portal venous system.
Mixed - 20% - anomalous venous drainage is a combination.
183
What are the types of Total Anomalous Pulmonary Venous Return?
50% supracardiac - at or above the level of the SVC. Snowman sign - common confluence of the pulmonary veins drains into the L inominate vein via a vertical vein. The dilated vertical vein making up the left border of the snowman.
Cardiac - 20% - Anomalous drainage into the coronary sinus or RA.
Infracardiac - 20% - Anomalous drainage passes through the diaphragm via the esophageal hiatus and then drains into the hepatic IVC, hepatic vein, or portal venous system.
Mixed - 20% - anomalous venous drainage is a combination.
184
What is Aortic Coarctation associated with?
Bicuspid aortic valve - may become stenotic and cause post-stenotic aortic dilatation proximal to the site of coarctation
Turner Syndrome (XO)
May produce acyanotic CHF in infants if preductal
185
What is the MC cardiac tumor?
Rhabdomyoma - primarily babies under 1 with tuberous sclerosis.
186
What is a Cardiac Rhabdomyoma?
MC cardiac tumor - primarily in babies under 1 with tuberous sclerosis.
Earliest sign of TS that can be diagnosed in utero.
Varied clinical presentation - arrhythmia and obstruction. Cardiomegaly. Pulmonary vascularity is variable.
187
What is the earliest sign of tuberous sclerosis that can be diagnosed in utero?
Cardiac Rhabdomyoma
188
What is the second MC cardiac tumor detected in utero?
Cardiac or pericardial teratoma.
Commonly attached to the root of the PA or Aorta.
Can cause pleural effusion when arising from the pericardium. One of the primary causes of massive perinatal pericardial effusion, with potential for tamponade.
189
What is a Cardiac Teratoma.
Cardiac or pericardial teratoma.
Commonly attached to the root of the PA or Aorta.
Can cause pleural effusion when arising from the pericardium. One of the primary causes of massive perinatal pericardial effusion, with potential for tamponade.
190
What is a Cardiac Fibroma?
Rare, benign cardiac tumor of infancy and early childhood, arising from fibroblasts and myofibroblasts.
Similar variable clinical presentation to rhabdomyoma, with rhythm disturbances and outflow obstruction. The IV septum is MC site of origin - cause arrhythmia due to disruption of the conduction system.
Cardiomegaly is MC imaging finding.
191
MC site for cardiac teratoma?
Commonly attached to the root of the PA or Aorta.
Can cause pleural effusion when arising from the pericardium. One of the primary causes of massive perinatal pericardial effusion, with potential for tamponade.
192
MC site for cardiac fibroma?
IV septum - cause arrhythmia due to disruption of conduction system.
193
What is the Crista Terminalis?
Normal muscular ridge in the right atrium that runs from the entrance of the superior to that of the inferior vena cava.
194
What is a Chiari Network?
When the IVC or Eustachian valve has more trabeculated appearance.
195
What defines the right ventricle?
Moderator Band
Tricuspid papillary muscles insert on the septum.
No fibrous connection between the AV valve/outflow tract - Pulmonary valve separated from the tricuspid valve by an infundibulum - thick muscle called the crista supraventricularis.
In LVOT the mitral and aortic valves lie side by side.
196
Where do tricuspid papillary muscle insert?
On the septum.
197
What is the crista supraventricularis?
No fibrous connection between the RV AV valve/outflow tract - Pulmonary valve separated from the tricuspid valve by an infundibulum - thick muscle called the crista supraventricularis.
198
What is the walking man sign?
Indirect sign of LA enlargement.
Posterior displacement of the L main bronchus on the lateral radiograph. Creates an upside down "V" shape with the intersection of the right bronchus (looks like a man walking).
199
What connects the leaflets of the mitral valve to the papillary muscles?
Cordae Tendinae.
Papillary muscles insert into the lateral and posterior walls as well as the apex of the LV (not the septum, as in the RV)
200
Where do the papillary muscles insert in the LV?
Insert in the lateral and posterior walls as well as the apex of the LV.
Not the septum as in the RV.
201
What is an echogenic focus in the LV?
Calcified papillary muscle - associated with increased incidence of Downs (13%) - usually goes away by third trimester.
202
Difference between Lipomatous hypertrophy of the intra-atrial septum and a lipoma?
Lipomatous hypertrophy = dumbbell (bilobed) appearance, sparing of the fossa ovalis. Can be HOT on PET b/c it's made of brown fat.
When it doesn't spare = lipoma.
203
The obtuse marginal artery arises from which coronary artery?
LCx - supplies the lateral margin
204
The Diagonal branches come off which coronary artery?
LAD
205
The acute marginal branch comes off which coronary artery?
Right main
206
What does "co-dominant" circulation mean?
The posterior descending artery arises from the RCA and the posterior LV branches arise from the LCx.
207
What is Steal Syndrome associated with ALCAPA?
Reversal of flow in the anomalous left coronary arising from the pulmonary artery as pressure decreases in the pulmonary circulation.
208
MC cause of coronary artery aneurysms in children and adults?
Adults - atherosclerosis, iatrogenic
Child - Kawasaki (spontaneously resolve in 50%)
Can be associated with/caused by coronary fistulas.
209
What is a coronary fistula?
Connection between a coronary artery and cardiac chamber or great vessels.
Usually the RCA, with drainage into the right cardiac chambers.
Are associated/result in coronary artery aneurysms. Think about this if you see big crazy dilation of coronaries.
210
Contraindications to giving a BB?
Asthma, heart block, acute chest pain, or recent cocaine.
If can't give BB, have to use retrospective gating- can't do prospective.
211
Contraindications to Nitroglycerine?
Hypotension (SBP <100)
Severe aortic stenosis
Hypertrophic obstructive cardiomyopathy
Phosphodiesterase (Viagra-Sildenafil) use
212
Supra-valvular Aortic Stenosis =
Williams Syndrome
213
Bicuspid Aortic Valve and Coarctation =
Turners Syndrome
214
MC cause of mitral stenosis?
Rheumatic heart disease
215
MC cause of acute mitral regurgitation?
Endocarditis or papillary muslce/chordal rupture post MI
Chronic - primary (myxomatous degeneration) or secondary (dilated cardiomyopathy leading to mitral annular dilation)
216
Cause of idolated RUL pulmonary edema?
Mitral regurgitation
217
What is pulmonary valvular stenosis associated with?
Noonan's Syndrome - Male version of Turners
218
What is "peripheral pulmonary stenosis" seen in?
Alagille syndrome - kids with absent bile ducts
219
Cause of Supra-valvular aortic stenosis?
William's Syndrome
220
Cause of sub-valvular pulmonary stenosis?
TOF
221
MC cause of pulmonary regurgitation?
S/p congenital valve disease after surgery - TOF patient that has been repaired.
222
Causes of tricuspid regurgitation?
Weak annulus - endocarditis (IVDU) or carcinoid syndrome (serotonin degrades the valve).
MC cause in adults in pulmonary arterial HTN.
223
What is Ebstein Anomaly?
Children who's moms used Lithium.
Tricuspid valve is hypoplastic and the posterior leaf is displaced apically (downward). Large RA, decreased RV ("atrialized"), and tricuspid regurgitation.
Massive "box-shaped" heart on CXR.
224
What is Tricuspid Atresia?
Congenital anomaly that occurs with RV hypoplasia. Almost always has an ASD or PFO.
Recognized association with asplenia.
Can have right arch.
Usually has pulmonary stenosis, therefore will have decreased vascularity. If no PS is present, there will be increased vascularity.
225
What congenital heart abnormality is associated with asplenia?
Tricuspid atresia.
226
What is the MC arch branching variant?
"Bovine Arch" - brachiocephalic artery and R common carotid arise from a common origin.
227
What are the two types of right arches?
Mirror branching and right arch with aberrant left subclavian.
228
What is a right arch with mirror branching?
Often asymptomatic, strongly associated with congenital heart disease.
MC associated with TOF, but most closely associated with Trucus.
If there is mirror image arch, then 90% will have TOF (6% truncus).
If the person has truncus, then they will have a mirror image right arch 33% (TOF 25%)
229
If a person has a right arch with mirror branching, what else are they likely to have?
TOF - 90% (6% truncus)
230
If a person has Truncus, what else are they likely to have?
Right arch with mirror imaging - 33% (TOF 25%)
231
What is the Diverticulum of Kommerell?
L arch with aberrant R subclavian - the origin of the R subclavian may be dilated.
232
What is the MC vascular ring?
Double aortic arch - symptoms begin at birth and include tracheal compression and/or difficulty swallowing.
R arch is larger and higher, the left arch is smaller and lower.
Arches are posterior to esophagus and anterior to trachea.
233
Vessels passing between esophagus and trachea?
Pulmonary sling
234
Vessel compressing anterior to trachea and posterior to esophagus?
Double aortic arch
235
Vessel compressing anterior to trachea only?
Innominate Artery Compression
236
Vessels compressing posterior esophagus only?
R arch with aberrant L
| L arch with aberrant R
237
Causes of subclavian steal?
Atherosclerosis (98% - in adult) - Takayasu Arteritis (teenager/20 year old), Radiation, Preductal aortic coarctation, and Blalock-Taussig Shunt.
238
Egg on a string heart?
Transposition
239
Snow man heart?
Total anomalous pulmonary venous return (supracardiac)
240
Boot-shaped heart?
TOF
241
Figure 3?
Coarctation
242
Box-shaped heart?
Ebstein Anomaly
243
Scimitar Sword?
Partial anomalous pulmonary venous return with hypoplasia
244
What are the associations with right arch?
Truncus (more closely) - increased pulmonary vasculature
| TOF (more common) - decreased pulmonary vasculature
245
How to tell if a right arch is associated with TOF or Truncus?
Look at pulmonary vasculature
```
Increased = truncus
Decreased = TOF
```
246
What congenital heart abnormalities are associated with normal sized heart with decreased or normal pulmonary blood flow?
TOF
Ebsteins
Tricuspid Atresia
247
What congenital heart abnormalities are associated with normal sized heart with increased pulmonary blood flow?
TAPVR (especially type 3)
D-transposition
Truncus (look for R arch)
"Tingle Ventricle"
248
What are the cyanotic congenital heart conditions?
The Ts
```
TOF
TAPVR
Transposition
Truncus
Tricuspid Atresia
```
249
What are the noncyanotic congenital heart conditions?
```
ASD
VSD
PDA
PAPVR
Aortic Coarctation (adult type - post ductal)
```
250
Causes of CHF in newborn?
```
TAPVR (infracardiac type "III")
Congenital Aortic or Mitral Stenosis
Left-sided hypoplastic heart
Cor Triatriatum
Infantile (pre-ductal) coarctation
```
251
Cyanotic congenital cardiac heart conditions with survival dependent on admixture?
```
TAPVR (has PFO)
Transposition
TOF (has VSD)
Tricuspid Atresia (has VSD)
Hypoplastic left
```
252
Differential of small heart without congential cardiac disease?
Adrenal insufficiency (Addisons)
Cachectic state
Constrictive pericarditis
253
What is the MC congenital heart disease?
VSD - membranous is most common (70%).
254
What types of VSDs need to be repaired?
Outlet subtypes (infundibulum) - the right coronary cusp prolapses into the defect.
255
What should you think of with a PDA?
Prematurity, Maternal Rubella, and Cyanotic heart disease
256
What is the MC type of ASD?
Secundum (50-70%)
Primum is larger (results from an endocardial cushion defect) - more likely to be symptomatic.
Only secundum types will close w/o treatment (Primum, AV canal, sinus venosus will not)
257
Hand/thumb defects + ASD =
Holt Oram
258
Ostium Primum ASD (or endocardial cushion defect) =
Downs
259
What is PAPVR?
Partial Anomalous Pulmonary Venous Return - one (or more) of the four pulmonary veins draining into the RA.
Often mild or no physiologic consequence.
Often associated with ASD (secundum and sinus venosus types).
260
Right-sided PAPVR =
Sinus Venosus ASD
RUL: SVC association with sinus venosus type ASD.
261
Right sided PAPVR + Pulmonary Hypoplasia =
Scimitar Syndrome.
262
What is TAPVR?
Total Anomalous Pulmonary Venous Return
Cyanotic heart disease - all of the pulmonary venous system draining to the right side of the heart.
Large PFO or less commonly ASD is required for survival
263
What is necessary for survival with TAPVR?
Large PFO or less commonly ASD is required for survival
264
What are the 3 types of TAPVR?
1. Supracardiac - most common type. Vein drains above the heart, gives a snowman appearance.
2. Cardiac - 2nd MC type.
3. Infracardiac - Vein drains below the diaphragm (hepatic veins or IVC) - obstruction on the way back through the diaphragm is common and causes pulmonary edema.
265
What will see in supracardiac TAPVR?
Snowman
266
What will see in infracardiac TAPVR?
Pulmonary edema in newborn
267
How many asplenia patients have congenital heart issues?
50% - of those nearly 100% include TAPVR (85% have additional endocardial cushion defects).
268
MC cause of cyanosis in the first 24 hours?
Transposition
269
Transposition is MC seen in what patient population?
Infants of diabetic mothers
270
What is necessary for survival with transposition?
ASD, VSD, or PDA - MC is VSD.
271
What are the two types of transposition?
D-Transposition: Discordance between the ventricles and vessels. Classic radiographic appearance - "egg on a string".
L-Transposition: "Lucky" - congenitally corrected - "double discordance" - the atrium hooks up with the wrong ventricle and the ventricle hooks up with the wrong vessel.
272
What do you use to fix D-Transposition?
Intra-arterial baffle (Mustard or Senning procedure)
273
What is the characteristic appearance of corrected D-transposition?
PA is draped overtop the aorta - LeCompte Maneuver
274
Associations with Truncus Arteriosus?
Almost always has a VSD, closelyi associated with right arch.
Associated with CATCH-22 genetics (DiGeorge Syndrome).
275
Associations with Coarctation?
Strong association with Turners Syndrome (15-20%).
Bicuspid Aortic Valve is MC associated efect (80%).
Have more berry aneurysms.
Rib notching of 4th - 8th ribs - does not involve 1st and 2nd b/c those are fed by the costocervical trunk.
276
What is cor Triatriatum Sinistrum?
Abnormal pulmonary vein draining into the LA (sinistrum meaning left) with an unnecessary fibromuscular membrane that causes a subdivision of the LA. Creates appearance of a tri-atrium heart.
Can be a cause of unexplained pulmonary HTN in the peds setting - acts like mitral stenosis, can cause pulmonary edema.
277
What is stunned myocardium?
After acute injury (ischemia or reperfusion injury), dysfunction of myocardium persists even after restoration of blood flow - can last days to weeks.
Perfusion study will be normal, but the contractility is crap.
278
What is Hibernating Myocardium?
More chronic process, result of severe CAD causing chronic hypoperfusion.
Will have decreased perfusion and decreased contractility even when resting - not an infarct.
On FDG-PET, this tissue will take up tracer more intensely than normal myocardium - will also demonstrate redistribution of Thallium.
Reversible with revascularization.
279
What sequence is used for delayed contrast imaging for MRI?
An inversion recovery technique to null normal myocardium, followed by a gradient echo.
280
What does Microvascular obstruction mean and when is it seen?
Islands of dark tissue in an ocean of late Gd enhancement.
Indicate microvascular obliteration in the setting of an acute infarct. Gd is unable to get to those regions even after the restoration of epicardial blood flow.
Poor prognostic finding.
NOT seen in chronic infarct.
281
Characteristics of a true ventricular aneurysm?
Mouth is wider than body. Myocardium is intact.
Usually anterior-lateral wall.
282
Characteristics of a false ventricular aneurysm?
Mouth is narrow compared to body. Myocardium is NOT intact (pericardial adhesions contain the rupture).
Usually posterior-lateral wall.
Higher risk of rupture.
283
Delayed enhancement in idiopathic dilated cardiomyopathy?
Linear midmyocardial enhancement
284
Causes of restrictive cardiomyopathy?
Anything that causes a decrease in diastolic function. Can be the result of myocardium replaced by fibrotic tissue (endocardial fibroelastosis), infiltration of the myocardium (amyloidosis), or damage by iron (hemochromatosis.
285
Findings in Amyloidosis?
Abnormal diastolic function with biatrial enlargement, concentric thickening of the LV and reduced systolic function of usually both ventricles.
Seen in 50% of cases of systemic amyloid.
Can sometimes see late Gd enhancement over entire subendocardial circumference.
Long TI is needed - "difficult to suppress myocardium"
286
"Difficult to suppress myocardium" with long TI =
Amyloidosis
287
Bilateral ventricular thrombus =
Eosinophilic Cardiomyopathy
288
What is "sigmoidization"?
Seen on SSFP cine imaging: the ventricular septum moves toward the LV in a wavy pattern during early diastole - due to constrictive pericarditis
"Diastolic Bounce"
289
What is "diastolic bounce"?
Seen on SSFP cine imaging: the ventricular septum moves toward the LV in a wavy pattern during early diastole - due to constrictive pericarditis
"Sigmoidization"
290
Enhancement of Myocarditis?
Late Gd enhancement follows a non-vascular distribution preferring the lateral free wall.
Epicardial or mid wall (NOT subendocardial).
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Findings of cardiac Sarcoidosis?
Signal in both T2 and early Gd (as well as late Gd) will be increased.
Late Gd pattern may be middle and epicardial in a non-coronary distribution.
Focal wall thickening from edema can mimic hypertrophic cardiomyopathy.
Often involves the septum.
RV and papillaries are RARELY affected.
292
What is Takotsubo Cardiomyopathy?
Transient akinesia or dyskinesia of the LV apex w/o coronary stenosis.
Ballooning of the LV apex is a buzzword. No delayed enhancement.
293
What is Arrhythmogenic RV Cardiomyopathy?
Fibrofatty degeneration of the RV leading to arrhythmia and sudden death.
Dilated RV with reduced function, fibrofatty replacement of the myocardium, and normal LV.
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What is Hypertrophic Cardiomyopathy?
Abnormal hypertrophy (from disarray of myofibrils) of the myocardium that compromises diastole.
Asymmetric hypertrophy of the intraventricular septum.
Ventrui forces may pull the anterior leaflet of the mitral valve into the LVOT (SAM - systolic anterior motion of the mitral valve).
Patchy midwall enhancement of the hypertrophied muscle may be seen.
295
What is Noncompaction?
Loosely packed myocardium. LV has spongy appearance with increased trabeculations and deep intertrabecular recesses.
Ratio of noncompacted end-diastolic myocardium to compacted end-diastolic myocardium of more than 2.3:1.
296
Kid with dilated heart and midwall enhancement?
Muscular Dystrophy - biventricular replacement of myocardium with connective tissue and fat - delayed Gd enhancement in the midwall.
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MC cardiac malignancy?
Mets
Pericardium is MC site affected.
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MC primary cardiac malignant tumor in adults?
Angiosarcoma.
Tend to like the RA and involve the pericardium. Bulky and heterogeneous.
"sun-ray" appearance - which describes enhancement appearance of the diffuse subtype as it grows along the perivascular spaces associated with epicardial vessels.
Often cause right-sided failure and/or tamponade.
299
MC location for cardiac angiosarcoma?
Tend to like the RA and involve the pericardium. Bulky and heterogeneous.
MC primary cardiac malignant tumor in adults
"sun-ray" appearance - which describes enhancement appearance of the diffuse subtype as it grows along the perivascular spaces associated with epicardial vessels.
Often cause right-sided failure and/or tamponade.
300
What is "sun-ray" appearance a buzz word for?
Angiosarcoma - describes enhancement appearance of the diffuse subtype as it grows along the perivascular spaces associated with epicardial vessels.
301
What is a cardiac myxoma?
MC primary cardiac tumor in adults (rare in children).
Associated with MEN syndromes and Blue Nevi (Carney Complex)
LA attached to the interatrial septum. May be calcified. May prolapse through the MV. Will enhance with Gd - important discriminator from a thrombus).
302
Location of cardiac myxoma?
LA attached to the interatrial septum. May be calcified. May prolapse through the MV. Will enhance with Gd - important discriminator from a thrombus).
303
What is a cardiac rhabdomyoma?
MC fetal cardiac tumor. It is a hamartoma.
Prefers the LV.
Associated with tuberous sclerosis.
Most will regress spontaneously (those NOT associated with TS are less likely to regress).
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MC fetal cardiac tumor
Rhabdomyoma - a hamartoma
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Where is a cardiac rhabdomyoma commonly found?
LV
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What is a cardiac rhabdomyoma associated with?
Tuberous Sclerosis
Most will regress spontaneously (those NOT associated with TS are less likely to regress).
307
What is a cardiac fibroma?
2nd MC cardiac tumor in childhood.
Likes the IV septum.
Dark/dark on T1/T2.
Enhance very brightly on perfusion and late Gd.
308
Where is a cardiac fibroma commonly found?
IV septum.
309
What does a cardiac fibroma look like on MRI?
Dark/dark on T1/T2
| Enhance very brightly on perfusion and late Gd.
310
What is a cardiac Fibroelastoma?
MC neoplasm to involve the cardiac valves (80% aortic or mitral).
Highly mobile on SSFP Cine.
Systemic emboli are common (especially if on the left side).
311
MC primary cardiac neoplasm to involve the cardiac valves?
Fibroelastoma (80% aortic or mitral)
312
Causes of pericardial effusion?
Renal failure (uremia) - Mc
Lupus
Dressler Syndrome.
313
ROI measuring water density along the R cardiophrnic sulcus =
Pericardial cyst
314
MC absent section of pericardium?
Over the LA and adjacent PA
LAA is most at risk to become strangulated.
315
What are the palliative surgeries for hypoplastic left heart?
Not curative - designed to extend life - 3 stage process:
1. Norwood or Sano - within days
2. Glenn - 3-6 months
3. Fontan - 1.5 to 5 years
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What is the Norwood Procedure?
Create an unobstructed outflow tract from the systemic ventricle. The tiny native aorta is anastomosed to the pulmonary trunk and the arch is augmented with a graft (or other methods).
ASD is enlarged to create non-restrictive atrial flow.
Blalock-Taussig Shunt is used between the right subclavian and right PA. Ductus is removed to prevent over shunting to the lungs.
317
What is a Sano Procedure?
Same as a Norwood, but instead of using a Blaylock-Taussig shunt - a conduit is made connecting the RV to the PA.
Disadvantage of the BT shunt undergoes a steal phenomenon (diverted to low pressure pulmonary system).
318
What is the Classic Glenn Procedure?
Shunt between the SVC and R PA (end-to-end), with the additional step of sewing the proximal end of the R PA closed to reduce the RV work, by diverting all venous return straight to the right lung
319
What is a Bi-Directional Glenn?
Shunt between the SVC and R PA (end-to-side). The RPA is left open, letting blood flow to both lungs.
Can be used to address R-sided heart problems in general and also step two in the palliative hypoplastic series. If used as step two, the previously placed Blaylock Taussig Shunt or Sano shunt will come down as the Glenn will be doint its job of putting blood in the lungs.
320
What is the Fontan Operation?
Used for hypoplastic hearts.
Classic consisted of a classic Glenn (SVC to RPA), closure of ASD, then placing a shunt between the RA and L PA.
Idea is to let blood return from systemic circuation to the lungs by passive flow (no pump), and turn the RV (the only one the kid has) into a functional LV.
321
What is a Classic Blalock Taussig Shunt?
Shunt between the subclavian artery and the pulmonary artery.
Constructed on the opposite side of the arch.
Glenn = Vein to artery (SVC to Artery)
Blalock Taussig = Artery to Artery (subclavian artery to pulmonary artery)
322
What is a Modified Blalock Taussig Shunt?
Gortex shunt between the subclavian artery and pulmonary artery - performed on the SAME SIDE as the arch - easier to do.
323
What is Pulmonary Artery Banding?
Done to reduce pulmonary artery pressure.
MC indication is CHF in infancy with anticipated delayed repair. Single ventricle is MC lesion requiring banding.
324
What is an atrial switch?
Mustard and Senning procedures - used to correct transposition of the great arteries by creating a baffle w/in the atria in order to switch back the blood flow at the level of in-flow. Result is the RV becomes the systemic ventricle and the LV pumps to the lungs.
Senning: Baffle is created from the RA wall and atrial septal tissue WITHOUT use of extrinsic material
Mustard: Involves the resection of the atrial septum and creation of a baffle using pericardium (or synthetic material)
325
What are the two atrial switch procedures and how are they different?
Senning: Baffle is created from the RA wall and atrial septal tissue WITHOUT use of extrinsic material
Mustard: Involves the resection of the atrial septum and creation of a baffle using pericardium (or synthetic material)
326
What is a Rastelli Operation?
Most frequently used operation for transposition, pulmonary outflow obstruction, and VSD.
Placement of a baffle w/in the RV diverting flow from the VSD to the aorta- essentially using the VSD as part of the LVOT.
The pulmonary valve is oversewn and the conduit is inserted between the RV and the PA.
Primary advantage - the LV becomes the system ventricle.
Primary limitation - requires multiple additional surgeries b/c the conduit wears out and must be replaced.
327
What is a Jatene Procedure?
Arterial switch method.
Involves transection of the aorta and pulmonary arteries about teh valve sinuses, including removal of the coronaries.
The great arteries are swtiched and the coronaries are sewn into the new aorta (Formerly the PA).
Difficult procedure, but no conduit to go bad, and the LV is the systemic vessel.
328
What is a Ross Procedure?
Performed for diseased aortic valves in children.
Replaces the aortic valve with the patient's pulmonic valve and replaces the PV with a cryopreserved PV homograft.
329
What is a Bentall Procedure?
Composite graft replacement of the aortic valve, aortic root, and ascending aorta, with re-implantation of the coronary arteries into the graft.
Used to treat combined aortic valve and ascending aorta disease, including lesions associated with Marfan syndrome.
330
Difference between orthotopic and heterotopic heart transplants?
Orthotopic - all of the heart is removed, except the circular part of the LA (the part wth the pulmonary veins). The donor heart is trimmed to fit into the LA.
Heterotopic- Recipient heart remains in place, and donor heart is added on top.
Gives native heart a chance to recover.
Gives you a backup if the donor is rejected.
