Sudden Cardiac Arrest in Children and Adolescents Coronary Artery Abnormalities

43 Pages·2011·1.71 MB·English

Sudden Cardiac Arrest in
Children and Adolescents
OOtthher causes off SSCCAA
Coronary artery abnormalities
Myocarditis
Commotio Cordis
SCA Symposium
CHOC/UCI
January 14, 2011
Coronary Artery
Abnormalities
January 14-15, 2011 SCA Conference 1
Maron BJ, et al. Sudden death in young competitive athletes:
clinical demographic and pathological profiles. JAMA.
1996;276:199-204.
Coronary artery abnormalities
•• AAnnoommaalloouuss oorriiggiinn ooff tthhee lleefftt ccoorroonnaarryy aarrtteerryy
from the pulmonary artery (ALCAPA)
• Origin of the coronary artery from the opposite
sinus of Valsalva
• Coronary artery fistulae
• KKawasakkii ddiisease
• Atherosclerotic coronary artery disease
January 14-15, 2011 SCA Conference 2
Coronary artery abnormalities
•• AAnnoommaalloouuss oorriiggiinn ooff tthhee lleefftt ccoorroonnaarryy aarrtteerryy
from the pulmonary artery
• Origin of the coronary artery from the opposite
sinus of Valsalva
• Coronary artery fistulae
• KKawasakkii ddiisease
• Atherosclerotic coronary artery disease
Coronary artery from the opposite
sinus of Valsalva
•• WWhhiicchh ccoorroonnaarryy aarrtteerryy hhaass tthhee aannoommaalloouuss
origin?
•Right or left
• What is it’s course?
•Intramural, intrarterial
••IInnttrraammyyooccaarrddiiaall
• What are the patient’s symptoms if any?
• What to do and when?
January 14-15, 2011 SCA Conference 3
Coronary artery anomalies
• Byyron H
•“Healthy” 14 year old
•March 2001, sudden cardiac arrest while
playing basketball at community center
•CPR performed, defibrillation 15 minutes
llaatteerr ((ppaarraammeeddiiccss))
•Transfer to CHW, severe DCM
Coronary artery anomalies
•• BByyrroonn’ss sseevveerree LLVV ddyyssffuunnccttiioonn::
•Primary?
•History suggests that he was asymptomatic
•Secondary to the prolonged arrest and
fibrillation?
•Most likely scenario
January 14-15, 2011 SCA Conference 4
Coronary artery anomalies
•• BByyrroonn HH.
•Balloon pump then mechanical cardiac
support with ECMO for 72 hours
•Past history: This was the 4th episode of
syncope in the last 2 years
•CCaarrddiiaacc wwoorrkk-uupp iinn RRaacciinnee 11 yyeeaarr pprreevviioouuss
with echocardiogram, tilt, EP – all “normal”
Coronary artery anomalies
•• EEcchhooccaarrddiiooggrraamm
•Transthoracic
•TEE
January 14-15, 2011 SCA Conference 5
Coronary artery anomalies
Ao
Anomalous origin of
LCA from right sinus of
Valsalva
January 14-15, 2011 SCA Conference 6
Coronary artery anomalies
•• BByyrroonn HH.
•Weaned from ECMO after 72 hours with full
recovery of LV function
•Taken to OR for ECMO decannulation and
“unroofing” of LCA into left sinus of Valsalva
•FFuullll nneeuurroollooggiicc rreeccoovveerryy, ddiisscchhaarrggeedd 33
weeks after admission
January 14-15, 2011 SCA Conference 7
AOCA from the Opposite Sinus with an Interarterial
Course—Anatomic Features
Aorta
Cx
R L Cx R L
LMCA RCA
LAD
LAD
Pulmonary trunk
LCA from right sinus RCA from left sinus
AOCA: Sudden Death Risk
(cid:131) LCA from right sinus of Valsalva is rare (0.03%) but clearly
associated with sudden cardiac death
(cid:131)2299//338811 andd 3366//449922 carddiiac ddeatthh
(cid:131)23/23 <20 yrs died suddenly with exercise1
(cid:131)Majority of exercise-induced SD were
asymptomatic 1,3
(cid:131) RCA from the left sinus of Valsalva is more common (0.1%) but also
associated with sudden cardiac death
15/52 cardiac death; 13 sudden cardiac death2
(cid:132)
All 13 SD were asymptomatic
(cid:132)
8/25 sudden cardiac death; 6/8 were 16-33 yrs1
(cid:132)
From:1Roberts WC, Am Heart J 1986; 111
2Taylor AJ et al , J Am Coll Cardiol 1992; 20
3Frescura C et al, Hum Pathol 1998; 29
January 14-15, 2011 SCA Conference 8
AOCA: Which is Riskiest?
(cid:131) Largest retrospective review of SCD in military recruits
ffrroomm 11997777--22000011
(cid:131)64 sudden deaths classified as cardiac among 6.3
million recruits
(cid:131)39/64 died with coronary pathology at autopsy
(cid:131)54% had AOCA (left from right sinus)
(cid:131)26% had obstructive atherosclerotic CAD
(cid:131)(cid:131)00%% hhaadd AAOOCCAA ((rriigghhtt ffrroomm lleefftt ssiinnuuss))
(cid:131)50% had symptoms
Eckhart RE et al, Cardiology in Review 2006; 14
Anatomy of CCAA Associated with SCD
(cid:131)Certain anatomic subtypes of anomalous coronary
oriigiin carry hhiighher riiskk off acutte myocarddiiall
complications
(cid:131)Anomalous origin of a coronary artery (AOCA) from the
opposite sinus with an interarterial course
Interarterial:
(cid:131)coursing between the great arteries
IInnttrraammuurraall::
(cid:131)coursing within the wall of the aorta
Intramyocardial:
(cid:131)coursing through the myocardium
January 14-15, 2011 SCA Conference 9
AAnnoommaalloouuss oorriiggiinn ooff tthhee LLMMCCAA
ffrroomm tthhee rriigghhtt ssiinnuuss ccaann hhaavvee aa
vvaarriiaabbllee ccoouurrssee
AAAA))AAntteriiorttoPPAA
BB)BetweenAoandPA AA BB
-interarterial
CC)Inventricularseptum
-intramyocardial
DD)PosteriortoAo
CC DD
From Roberts and Shirani, Am J Cardiol 1992; 70
AOCA with an intramural course: runs within
the wall of the aorta
Features
-separate orifice
-acuteangle take-off
-may have ostial stenosis
-exits aortic wall from appropriate
sinus then courses normally
-surgiicall unrooffiing ttechhniique iiddeall
LCA from right sinus
January 14-15, 2011 SCA Conference 10