Download Cardiac CT and MRI Findings of Structural Heart Diseases

Cardiac CT and MRI Findings of Structural Heart Diseases causing Sudden Cardiac Death in Adults
1Department
Sung-Min Ko1, Jeong-Geun Yi1,Woo-Chul Kwon2
of Radiology, Konkuk University Hospital, Seoul, Korea
Introduction
Coronary
y artery
y disease with angina
g
or MI
Ischemic Heart Disease
CAD with MI or angina
Coronary artery embolism
Nonatherogenic CAD
Coronary artery spasm
C
D
E
No structural Heart Disease
Primary electrical disease
Prolonged QT syndrome
Preexcitation syndrome
Complete heart block
Chest wall trauma
Nonischemic heart disease
CAD without MI or angina
Cardiomyopathy
– hypertrophic, dilated, restictive
Valvular heart disease
Congenital heart disease
Myocarditis
Acute pericardial tamponade
Acute myocardial rupture
Acute aortic dissection
Arrhythmogenic RV dysplasia
- 80% of patients who experience SCD at all ages have
atherosclerotic coronary artery disease
- At post mortem acute occlusive coronary thrombus was
found in 15% to 64% due to plaque fissuring, hemorrhage or
thrombosis
- No difference in artery involved.
- More risk of SCD with abrupt proximal obstruction rather
than progressive diffuse disease when collateral can form
- Patient prognosis with AMI depends on the extent of
myocardial necrosis, the presence of microvascular
obstruction, and the degree of contractile dysfunction
A
of Radiology, Wonju Christian Hospital
Causes of SCD
Sudden cardiac death (SCD) is defined as an
unexpected sudden death due to cardiac cause and
occurring within 1 hour of symptoms and is a major
public health problem. SCD accounts for 30% of
sudden deaths between 14 and 21 years of age.
There is an increased risk of SCD in athletic young
adults. There are a number of underlying cardiac
conditions that increase the risk of SCD even though
acute myocardial infarction as a correlate of
coronary artery disease is mostly common.
Different underlying causes can be diagnosed and
differentiated by contrast-enhanced CT and MRI
non-invasively.
B
2Department
Fig. 2. 72-year-old man with
reperfused AMI.
(A) CT angiography of RCA
– Massive thrombotic occlusion
of m-dRCA
(B-E) Two-phase perfusion
images with CT (B, C) and MR
((D,E)
, ) - Early
y subendocardial
perfusion defect (B&D, arrows)
and subendocardial residual PD
with transmural late
enhancement (C&E, arrows) in
the mid inferior myocardium of
LV wall.
A
A
Noncardiac Disease
Drowning
Pickwickian syndrome
Pulmonary embolism
Drug-induced
Airway obstruction
B
C
Fig. 1. 62-year-old man who presented with anterior chest pain for 10 days.
(A, B) CT angiography
-Significant stenoses (arrows) with noncalcified plaques in the proximal
segment of LAD
(C) Conventional angiography – 85% stenosis in the same segment (arrow).
Fig. 3. 68-yearold man with AMI.
(A, B) Multiplanar
reformations in
4-chamber
4
chamber (A)
and short-axis (B)
views
– Rupture of
interventricular
septum
A
B
B
Fig. 6. 58-year-old woman who presented with dyspnea on exertion.
(A) Inverse angiographic emulsion image- Left anterior descending coronary
artery (arrow) originating right coronary artery (arrowhead, single coronary
artery without left circumflex artery).
(B) Oblique coronary thick slap MIP image – Interarterial and intramyocardial
course of LAD (arrow) originating from the right coronary (arrowhead)
Myocardial bridging
A
Coronary artery fistula
- An anomalous communication between a coronary artery
and either a cardiac chamber, coronary sinus or pulmonary
artery
- More commonly involving the RCA (60% of cases) than the
LCA (40%)
- The commonest sites of drainage are the right ventricle
(45%), right atrium (25%) or pulmonary artery (15%),
producing left to-right shunt.
- If fistula flow is high, the distal perfusion of the
originating coronary artery may be compromised.
B
A
Hypertrophic cardiomyopathy
A
B
Fig. 4. 59-year-old man who presented with chest pain after blunt trauma.
(A, B) Multiplanar reformations in 4-chamber (A) and short-axis (B) views
– Intramural hematoma (arrows) extending from the left main bifurcation to
the proximal LAD.
(C) Volume
Volume-rendered
rendered image – Excellent depiction of luminal narrowing of pLAD.
pLAD
B
A
- The most common of cardiomyopathy with an incidence of 1 in 500
- The commonest cause of SCD in the US (70% of patient suddenly die)
- Most common inherited cardiac disease (autosomal dominant)
- Basal anterior and anterior septal are most commonly affected
- Wall thickness > 30 mm infers an increased risk of SCD
- Systolic anterior motion (SAM) of the mitral leaflet with associated
mitral regurgitation is frequent finding.
finding
- With increasing wall thickness, there is increased percentage of
fibrosis. As fibrosis increases, wall function decreases.
B
C
Fig. 8. 66-year-old woman
who presented with chest pain
on exercise.
(A, B) Curved MPR image (A)
and short-axis MIP image of
LAD (B)
- Intramyocardial course of
mLAD (arrows) and
asymmetric wall thickening of
LV myocardium (B),
corresponding to hypertrophic
cardiomyopathy.
C
A
B
C
D
H
F
C
B
E
A
A
B
Fig. 7. 50-year-old man who presented with angina.
(A) Short-axis MPR image – Deep type of myocardial bridging (arrow)
(B, C) Conventional angiography – Systolic compression of the
tunneled segment (B, arrow) but recovery its normal diameter on
di t l (C,
diastole
(C arrow).
)
Fig. 9. Two cases of coronary artery-pulmonary artery fistula.
(A, B) Volume-rendered images – Anomalous connection (arrows) arising from right
and left coronary arteries and draining into the pulmonary trunk. The fistulas
contain aneurysms (arrowheads).
Anomalous coronary artery from the opposite sinus
- Risk of adverse events is highest in anomalous arteries with
interarterial course
: RCA from left coronary sinus - >90%
: LCA from right coronary sinus – 60%
: LAD from right coronary sinus – 50%
- The cause of 5-35% of SCD in young athletes
- Mechanism not entirely clear
: Abberant artery arises at an acute angle, often from a slitlike hypoplastic ostium, traverses the aortic wall obliquely,
emerges between the aorta and the right ventricular outflow
tract, and then proceeds to its usual area of distribution.
: With exertion, proximal angulation or compression obstructs
the blood flow , leading to distal ischemia, ventricular
tachycardia, and fibrillation.
A
- Coronary artery dives into the substance of the
myocardium
di
(i
(intramyocardial
t
di l course))
- Commonly middle segment of LAD
- Clinically silent in most cases. Rarely, myocardial ischemia
and infarction, arrhythmia, SCD
- Incidence: angiography 0.5-4.5%, CTA 3.5-30.5%
- Atherosclerotic plaque proximal to tunneled segment
- Angiography: milking effect and step down-step up
phenomena induced by systolic compression of MB segment
- MDCT: superficial and deep type, systolic compression
Æ Alternative noninvasive imaging tool that allows for easy
and accurate evaluation of myocardial bridging.
Coronary artery dissection after blunt trauma
- Coronary artery injuries after blunt chest trauma
: Lacerations,
L
ti s thrombosis,
th mb sis intimal
i tim l dissection,
diss ti
arteriovenous fistula, pseudoaneurysm formation
- Coronary dissection consists of about 16% of traumatic
coronary injuries.
- MDCT
: Ideal imaging technique to delineate intramural
hematoma, assess luminal narrowing and follow up the
appearance
: Useful to decide to do coronary angiography or not in
patients with borderline probability of coronary injury.
B
Fig. 5. 54-year-old man who presented with chest pain during exercise.
(A) Volume-rendered image – Left coronary artery (arrow) originating
from the right sinus of Valsalva and traveling between the aorta and
pulmonary artery
(B) Curved multiplanar reformation image of LAD
- Intramyocardial course of proximal segment of LAD (arrow)
D
Fig. 5. 29-year-old man who presented with chest pain during exercise.
(A) Volume-rendered image – Right coronary artery (RCA) originating from
the left sinus of Valsalva and traveling between the aorta and pulmonary
artery
(B, C) Oblique axial thick slap maximum intensity projection images of a RCA
- Slit-like narrowing of RCA ostium on end-systole (B) compared with enddiastole (C).
(D) Volume-rendered image obtained after coronary artery bypass graft
surgery – Patent right internal mammary artery graft to pRCA
B
D
G
I
J
Fig. 10. 64-year-old woman who presented with severe chest discomfort on exertion.
(A, B) 2- (A) and 4-chamber (B) SSFP cine images of MR – Concentric hypertrophy of LV wall.
(C, D) First pass myocardial perfusion cardiac MR images at stress (C) and rest (D)
– Partially reversible myocardial perfusion defect in anterior, septal and inferior LV wall.
(E-G) Delayed contrast-enhanced cardiac MR images – Diffuse enhancement except lateral wall
(H, I) Dual-energy CT iodine map images – Myocardial perfusion deficit in anterior LV wall
(J) Myocardial perfusion SPECT – No myocardial perfusion defect at stress and rest.
Fig. 11. 62-year-old man who presented with chest pain for
more than 10 year.
(A, B) LV outflow tract SSFP cine images of cardiac MR
- Hypertrophied LV septum (arrows)
– Narrow LVOT (arrowheads) on diastole (A) and systole (B)
- SAM with associated subvalvular stenosis and stenotic jet
flow (B, arrowhead) on systole and mitral regurgitation
(C) Short-axis SSFP cine image of cardiac MR – Asymmetric
hypertrophy of LV septum and anterior myocardium.
(D) Delayed contrast-enhanced cardiac MR image
- Extensive delayed hyperenhancement (arrows) of LV wall.
Non-ischemic dilated cardiomyopathy (NIDCM)
- Dilatation of all four chambers of the heart, but primarily
the left ventricle, with associated systolic dysfunction.
- 18% of symptomatic patients with ejection fraction less
than 30% were diagnosed with NIDCM.
- Patients with NIDCM suffer from heart failure mortality
and SCD in near equal numbers.
- SCD may well be the first manifestation of NIDCM,
NIDCM and
idiopathic NIDCM is responsible for 10% of all SCDs in
adults
- It is often preceded by infectious myocarditis but 2030% is familial. Other causes include autoimmune, toxic and
metabolic diseases.
- A mid-myocardial stripe on delayed contrast-enhanced
cardiac MR imaging is the “classic” imaging pattern seen in
about 30% of patients. 60% of patients will have no
enhancement
Aortic stenosis (AS)
B
A
- Most common valve disease resulting in valve replacement
- Causes: Congenital (bicuspid aortic valve), degenerative,
rheumatic heart disease
- Pathophysiologic effects of AS
: LV hypertrophy, LV systolic dysfunction, myocardial
fibrosis, abnormal coronary blood flow, pulmonary
hypertension
- Incidence of SCD in asymptomatic severe AS : 0-4.1%
- Mechanism of SCD: Unclear, and both malignant
ventricular arrhythmia and bradyarrhythmia have been
documented.
-Timing of surgery for AS
: Symptomatic moderate to severe AS
: Asymptomatic severe AS
- Progressive increase in AV velocity and decrease in AVA
grad > 65 mmHg,
g, AVA 0.7 cm2)
- Critical AS ((mean g
- LV systolic dysfunction
- Marked LVH
- Associated severe coronary artery disease
C
Fig. 12. 62-year-old man with
congestive heart failure
(A,B) 4-chamber SSFP cine images of
cardiac MR – Severely dilated LV
without thinned myocardium (A,
diastole). Global severe hypokinesia of
LV wall and mild MR (B, systole)
(C,D) Delayed contrast-enhanced
cardiac MR images – A mid-wall stripe
of delayed hyperenhancement in the
myocardium of anterior and septal LV
wall (arrows).
D
Amyloidosis cardiomyopathy
- Almost all cases of primary amyloidosis and approximately
¼ of familial amyloidosis
- Amyloid deposition occurs with the myocardium of the
ventricle and atria, valve leaflets and within the coronary
arteries.
ymp m occur from
f m disruption
p
in contractile function,
f
,
- Symptoms
conduction and influences coronary artery flow.
- Left ventricular wall thickness - prognostic importance
(median survival of only 6 months)
- Cardiac MR imaging: diffuse hyperenhancement pattern on
delayed-enhancement MRI, typically more prominent in the
subendocardium and the basal segments.
A
B
C
Fig. 13. 56-year-old woman who presented with dyspnea on exertion.
(A) Short-axis MPR image of cardiac CT – Concentric hypertrophy of LV
myocardial wall.
(B) First pass myocardial perfusion cardiac MR images at rest
– Concentric perfusion defect (arrows) of LV myocardial wall
(C) Delayed contrast-enhanced cardiac MR images – Diffuse myocardial
enhancement involving both ventricles (arrows).
A
B
C
Fig. 14. 67-year-old man with heart failure.
(A) Axial double inversion recovery T1WI – High signal intensity of thick
p
layer
y ( )and
)
iso-signal
g
intensity
y of thin compacted
p
layer
y ( )of
)
noncompacted
LV myocardium. Ratio noncompacted/compated layer = 4.8
(B, C) Short-axis (B) and 2-chamber (C) end-diastolic SSFP cine image of
cardiac MR – Excessive trabeculations of the LV wall, particularly severe in
apex and apical wall. LV dilatation and severe, global hypokinesia in the LV wall.
LV ejection fraction 15%
Fig. 15. 47-year-old
woman with arrhythmia.
(A, B) Axial SSFP cine
image of cardiac MR –
Prominent epicardial
fat (A,
(A arrow).
arrow)
- Aneurysm formation
in RV wall (B, arrows)
on end-systole.
Arrhythmogenic right ventricular dysplasia
- Myocardial
y
cell loss with partial
p
or total replacement
p
of
right ventricular muscle by adipose and fibrous tissue
- A familial disorder in 30% of cases, with autosomal
dominant inheritance.
- The most commonest cause of SCD in an Italian screening
program.
- Unexplained syncope or SCD is the first clinical
manifestation of the ARVD.
- Onset in adolescence or early childhood.
- CT: Abundant epicardial adipose tissue, prominent
t b
trabeculations
l ti s with
ith low
l attenuation,
tt
ti
scalloped
s ll
d appearance of
f
RV free wall, intramyocardial fat deposits
- MRI : Intramyocardial fat, RV wall thinning or
hypertrophy, trabecular disarray of RV, enlargement of
RVOT, global and regional RV dysfunction, myocardial
fibrosis with delayed contrast enhancement
- “billowing” or prolapse of one or both of the mitral
leaflets into the left atrium
prevalent condition more associated with non- MVP is a p
lethal arrhythmias.
- Whether simple MVP causes SCD in unclear as it is such a
prevalent condition that it may be coincidental finding and
not causally related to SCD.
- Patients who also have mitral regurgitation and LV
dysfunction or myxomatous degeneration of the valve are at
higher risk of SCD.
- M/C cause of severe non-ischemic MR in the USA
- Overall prognosis – excellent, but serious complications
i f ti endocarditis,
infective
d
diti SCD
SCD, severe MR
- Pathophysiological basis
: Leaflet thickening and redundancy
(myxomatous degeneration)
- Echocardiography
: single or bileaflet prolapse at least 2 mm
- Association with heritable connective tissue disorders
: Marfan syndrome, Ehlers-Danlos syndrome, osteogenesis
imperfecta, and pseudoxanthoma elasticum
Marfan’s syndrome
- Autosomal dominant inherited connective disorder
occurring in about 1 in 10000 people
- Defects in fibrillin-1 gene (FBN1) on chromosome 15
- Involves CV, skeletal, ocular systems
- Cystic medial necrosis in the wall of the aorta which
weakens it
g
dilatation of
f aortic root and sinus of
f Valsalva
- Progressive
and may lead to aortic regurgitation and complete dissection
or rupture of the aorta with subsequent death, pericardial
tamponade or coronary artery dissection
C
A
B
A
Fig. 16. 29-year-old
woman with
arrhythmia
(A, B) Axial (A) and
oblique coronal MIP
images – RV wall
thinning (A, arrow)
and scalloped
appearance of RV
free wall
(B, arrows)
B
A
B
Fig. 17. 27-year-old woman who presented with acute atypical chest pain (A)
Delayed contrast-enhanced cardiac MR – Mainly subepicardial enhancement
involving the mid anteroseptal and inferior LV wall (arrows).
(B) Short-axis triple inversion recovery T2WI – Ill defined high signal
intensity in the same areas (arrows). Courtesy of Chun EJ (BSNU)
- Longitudinal cleavage of the aortic media by dissecting
column of blood
- Almost always accompanied by a transverse intimal and
medial tear
- Intimal tear sites
:Type I and II: 1 ~ 2 cm above the sino-tubular junction
involving right lateral wall
:Type III: Distal to the origin of the left subclavian
artery
-False lumen in classic dissection
: Within the media
: Intimomedial flap = inner 2/3 of media + intima
- Complication : rupture,
rupture leakage,
leakage side branch
involvement, aortic occlusion, dissection aneurysm
Conclusion
C
D
Fig. 19. 57-year-old woman with known aortic stenosis.
(A, B) CT images with thick slab 4D reconstruction with blood pool inversion
technique – Pure bicuspid aortic valve with thickened, calcified cusps (A, arrow)
and reduced systolic opening (B
(B, arrow)
(C) CT planimetry of bicuspid AV – Moderate to severe degree of AS
(D) Oblique sagittal MPR image of cardiac CT – Aneurysmal dilatation of aorta
A
B
C
D
E
Fig. 20. 49-year-old
F
ld man with
h known
k
mitrall regurgitation
(A-C) CT images with thick slab 4D reconstruction with blood pool inversion
technique
– Prolapsed posterior mitral leaflet (P1 portion, arrows) during systole
(D, E) Short axis (D) and 3-chamber (E) SSFP cine images of cardiac MR
- Eccentric regurgitant jet flow across mitral valve into LA in systole (arrows)
Aortic dissection
B
D
Fig. 18. 81-year-old man who presented with chest pain.
(A, B) Double oblique axial MPR images of cardiac CT images
– Degenerative aortic valve disease with severely reduced systolic opening of
aortic valve (CT planimetry 0.72 mm2), corresponding to severe AS
(C) Oblique sagittal SSFP cine image of cardiac MR – Stenotic jet flow across
aortic valve into ascending aorta in systole (peak velocity 4.2 m/sec)
(D) Short-axis MPR image of cardiac CT – Old MI in the mid inferolateral wall
A
A
Myocarditis
- Most
M t cases are subclinical.
b li i l
- Most common cause: group B Coxsackie virus
- SCD is a recognized complication and is often associated
with exercise due to poor contraction of a dilated LV or an
arrhythmogenic focus from acutely inflamed or healed
myocardium.
- DE-MRI : Multiple patchy enhancement predominantly in
subepicardial, inferolateral portion.
: follow-up Æ decreased extent of enhancement
B
Mitral valve prolapse (MVP)
Non-compaction cardiomyopathy
- Rare cardiomyopathy which is believed to be a result of an
arrest in development of the myocardium.
- Gross anatomical appearance: numerous, excessively
prominent trabeculations and deep intertrabecular recesses
in communication with the ventricular cavity.
- Predominantly (>80%) found in the apical and midventricular areas of both the inferior and the lateral wall.
- Natural history: LV systolic dysfunction and heart failure
(most common), arrhythmias, thromboembolism, SCD
- Non-compacted to compacted ration > 2.3 in end-diastole
is considered diagnostic by MRI criteria.
- Differential diagnosis: prominent normal ventricular
trabeculations, ARVD, apical HCMP, thrombus,
endomyocardial fibrosis
A
B
C
Fig. 21. 33-year-old man with Marfan’s syndrome.
(A, B) Oblique sagittal (A) and axial MPR (B) images of cardiac CT
- Dilatation of aortic root and redundant non-coronary cusp (arrows)
with coaptation defect in diastole.
(C) Cine echocardiographic image - Severe eccentric aortic
regurgitation (arrow) due to prolapse of noncoronary cusp.
A
B
C
Fig. 22. 35-year-old man with Marfan’s syndrome.
(A C) Oblique
(A-C)
Obli
sagittal
itt l (A)
(A), axial
i l (B)
(B), and
d oblique
bli
coronall MPR (C) iimages of
f
cardiac CT - Aortic dissection (arrows) involving aortic root, particularly right
sinus of Valsalva with RCA ostial mild stenosis.
- Dilatation of aortic root and redundant non-coronary cusp (arrowhead) with
coaptation failure.
1. SCD is not common in the general population. However, the incidence is significantly higher in the cohort of
patients who has congenital cardiac disease. The causes of SCD in adults vary with age.
2. It is very important for the radiologists to be familiar with radiological findings of the predisposing underlying
cardiac abnormalities may allow preventive management.