Transcript Sudden Cardiac Death

Michael Liske, MD, FACC, FASE
East Tennessee Pediatric Cardiology
Knoxville, TN
www.etpc-hearts.com
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All time leading division I NCAA scorer with
44 PPG for LSU (would have been 57 PPG with
3 point rule)
"I don't want to play 10 years in the NBA and
then die of a heart attack at 40.“ (age 25)
Died at age 40 while playing a pick up game of
basketball with James Dobson, 1988
Dx?
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Single right coronary artery
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6 ft at 12 years, final height 6 ft, 5 in.
In 1984, led the US volleyball team to Olympic
silver
1986: collapsed on the sidelines watching her
team play in Japan
Dx?
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Aortic dissection from Marfan Syndrome
“Apart from her height, near-sightedness, very
long arms and large hands, she showed few
other physical symptoms. Hyman's sneaker
size was a USA size 12.” (Wikipedia)
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Sullivan South (Kingsport) Rebels co-captain
and linebacker
Died on the field while playing West High
School, Knoxville, August 2009
“Logue had been trailing the play, when he
dropped to the ground without being touched.
He lay on the field motionless. A fellow South
player tried to help him, but Logue was
unresponsive.” (News Sentinel)
Dx?
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“A heart problem with his bicuspid aortic
valve.” (News Sentinel)
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Sept 2009: “Collapsed on the practice field before
UT trainer Chad Newman and director of sports
medicine Jason McVeigh heroically brought
Negedu back to life with CPR and an automatic
external defibrillator.” (ESPN)
Dr. Stuart Bresee, UT cardiologist, said the initial
diagnosis was hypertrophic cardiomyopy,
however, “once Negedu quit training, his heart
was back to normal, . . the genetic testing was all
negative.” (ESPN)
He received an ICD and transferred to New
Mexico to continue competitive basketball.
Anyone know what happened to him next?
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Played 10 games for the U of NM Lobos, and
led the team in three games for rebounding
While playing The Citadel in a pre-Christmas
game, his defibrillator “produced a reading
that led doctors to keep him from finishing the
game. He never returned to competition.”
SportingNews NCAAB, Apr 2011
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Scope and demographics of the problem
Differential diagnoses, including new, in-vogue
conditions
Pre-participation screening
Practical EKG: normal or abnormal?
Rational public health approach
Please refer to the 10 questions of your handout!
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Maron, registry data of multiple sources (2009):
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News, LexisNexis, Internet, Center of Catastrophic
Sports Injury, NHLBI pathology archives, direct
submissions to registry, etc.
66 sudden cardiovascular deaths per year in USA in
athletes age < 39 years, on average.
0.6/100,000 participant-years (vs. 100/100,000 in
older adults)
Harmon, sources as above, plus NCAA
database (2011):
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2.3/100,000 participant-years (NCAA athletes only)
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In a patient with an unrecognized cardiac
condition, the risk of SCD is 2.5 times higher if
he is an athlete than a non-athlete,
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Yet, more non-athletes die per year of SCD because
there are more of them.
(eg., more non-athletes with HCM die, yet if you
have HCM, your individual chance of death
increases substantially if you are an athlete)
These are highly publicized events
NOT the most common cause of death in
children
Death in teens age 15-19 years of age
MVA 40%
Other accidents 12%
Homocide 14%
Suicide 11%
Neoplasia 5%
CV, non-SCD 1.5%
Athlete SCD 1.5%
All others 15%
National Vital Statistic Report 2005; Maron-Harmon data
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Males represent 89% of cases.
White = 55%, African American = 36%,
Hispanic = 3%, Asian = 1%
>50% of HCM deaths are in African Americans,
however, they are much less likely to be
identified before they die or have an arrest:
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Diminished access related to socioeconomic status?
80% occurred during or immediately after
exertion, 20% at rest.
Sport
(blunt trauma and
commotio cordis
excluded)
Age
Basketball
33%
High
school
59%
Football
25%
College
17%
Soccer 8%
All others
34%
Middle
school
11%
Other 13%
What is the most dangerous sport?
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#1 cause of SCD in competitive athletes
General mortality: 1%/yr (higher with some types)
Mutations of 11 genes affect cardiac muscle proteins
(sarcomeric proteins)
Some forms are inherited AD.
Potential Sxs: exertional syncope, palps, CP
The murmur is HCM may be soft, but tends to increase
with standing or Valsalva
In what other pathologic murmur does this occur?
The EKG is abnormal in 95% of cases: ST depression
and T inversion in inferior and lateral precordial leads;
prominent q’s, LVH
(EKG normal in 5% of patients)
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The degree of septal hypertrophy doesn’t
always correlate with the risk of sudden death
Doppler Tissue Imaging may be particularly
helpful in borderline cases.
Genetic testing is helpful in some cases.
Rx:
Exercise restrictions
 Meds: if symptoms: blockers and verapamil
 Septal myectomy: surgical or alcohol
 ICD in high risk cases (the other two haven’t been
shown to decrease the risk of SCD.)
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2nd most common cause
of SCD in athletes
Left coronary arising
from right sinus or
coronary and coursing
between aortic and PA
roots is the most
dangerous (in 0.15% of
pop)
Slit-like orifice and
acute angle take off are
mechanisms leading to
ischemia/arrhythmias.
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No prodrome in 50%
SCD more common in
the young
EKG and ETT are
almost always normal
Difficult echo diagnosis
in 140 kg linebacker
CT/MRI are accurate
Rx:
Exercise restrictions?
 Prophylactic surgery is
indicated in some cases.
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#2 cause of athlete SCD in
Italy, #4 cause in USA
Mutation of desmosomes
(adhesion proteins) between
ventricular myocytes
Secondary fibrosis and fatty
replacement of myocardium
LV is also involved late
Most variants are AD
Pathologic and EKG changes
increase with age
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EKG clues (difficult diagnosis!):
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Localized prolonged QRS
Epsilon waves in V1
T inversion, especially V2 and V3
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Most deaths occur in
adulthood (< 15%
prior to 18 years)
Difficult diagnosis in
childhood; MRI can
help
Some gene testing is
available
Rx:
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Exercise restrictions
Medical suppression
of arrhythmias
ICD
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Most famous “channelopathy”
Most LQTS types are due to Na channel “gain of
function” or K channel “loss of function.”
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QTc = QT/sq rt of the preceding RR
Top normal = 440 msec in males, up to 450-460 in
teen females
EKG normal in 5% of LQTS patients
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Channelopathies as a whole cause only 3% of SCD in
athletes
But, . . . , 10% of SIDS
Males particularly die early
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Multiple types, each with different genetics (12
so far), presentations, EKG’s and some with
varying treatments.
Romano Ward S: heterozygote LQT 1, 2, 3
(>90% of all cases)
Jervell Lange-Nielsen S: homozygote LQT 1, 5;
associated with severe sensorineural deafness
Timothy S: LQT8; syndactyly
Andersen-Tawil S: also periodic paralysis,
dysmorphisms,
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Torsades de Pointe
Prevention and Treatment:
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Exercise restrictions
Swimming precautions
Beta blockers, Na channel blockers
Pacemaker if associated with AV block
Defibrillation, isoproteronol if bradycardia
related
ICD
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Rx, con’t
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Avoid QT prolonging drugs
 www.sads.org
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What about all the fuss over Zofran?
 “Avoid Zofran in patients with congenital long QT syndrome.
ECG monitoring is recommended in patients with electrolyte
abnormalities (e.g., hypokalemia or hypomagnesemia), congestive
heart failure, bradyarrhythmias or patients taking other medicinal
products that lead to QT prolongation.” FDA
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Another
channelopathy
Gain of function of K
channels (early repol)
Peaked T’s and
shortened QTc,
typically < 300 msec
Familial syncope,
atrial and ventricular
fibrillation
“Impressively lethal”
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ICD’s problematic: with
sinus tachycardia, the
device interprets peaked
T wave as QRS
complexes and gives an
inappropriate shock
Quinidine has been
used
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Another
channelopathy
Loss of function of the
Na channel that
causes LTQ3
EKG (V1 findings)
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J point elevation
ST elevation with a
coved, downward
convex pattern.
AD inheritance
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Male:Female = 8:1
Asians
Sudden death in sleep and
with bradycardia
“Lai Tai” = sleep death, in
Thailand
Causes some SIDS
Arrhythmias precipitated
by fever in children
Rx:
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Exercise restrictions
(hyperthermia concern)
Quinidine
ICD if symptomatic
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WPW
CPVT: Catecholaminergic Polymorphic
Ventricular Tachycardia
Idiopathic Ventricular Fibrillation
Myocarditis
Dilated cardiomyopathy
Myocardial bridge over a coronary artery
Coronary artery disease
Aortic rupture
Aortic stenosis
MVP – on the list; real disease?
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3% of all deaths in athletes
Def: sudden death due to low energy trauma to
the chest wall
Impact to heart at a critical time of the cardiac
cycle
No pre-existing heart disease or genetic
condition, no subsequent rib fractures,
homothoraax, or significant myocardial
contusion
Primarily age 5-15 years.
Pig model, anesthetized animals:
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Wooden ball, 30 mph, 16 msec before T wave peak
Rhythm?
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Wooden ball, 30 mph, on QRS
Rhythm?
Implications for our Little Leaguers?
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13 year old hit
on chest by
inside pitch
Immediate
bystander CPR
Passing police
car “fortuitously” carried an
AED
Normal
neurologic
outcome
Implications?
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It begins with the PCP!
History, history, history! (and physical exam)
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EXERTIONAL SYMPTOMS ARE BAD
American Heart Association with American
College of Cardiology joint recommendations
contain 12 elements (Circ, 2007)
Any positive element should result in a
cardiology evaluation.
Tennessee Secondary School Athletic
Association form has all 12 elements! (only 7.5
in 2010) – Wonderful!!
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What about ancillary testing?
What one test will offer more insight at a lower
cost than any other?
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EKG
(Remember that H and P are critical)
When should an EKG be done?
On everyone? Just on those with a positive
AHA screen? Is a normal EKG enough to be
done with testing after a positive screen?
What about echocardiography?
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The EKG is abnormal in 95% of HCM and
LQTS cases; and most cases of WPW, SQTS,
pulmonary hypertension, myocarditis, dilated
cardiomyopathy, and severe AS. It is abnormal
in some cases of Brugada syndrome and
ARVC.
BUT, the EKG is typically normal with
congenital coronary anomalies, aortic dilation
Marfan, some dilated CM, commotio cordis,
etc. (altogether 30% of potential cases of SCD)
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In 1982, a mandatory, government funded, annual, preparticipation screening program was instituted for all
athletes in Italy, including an H & P with EKG.
Retrospective review of registry and newspapers, Veneto
region)
The results were seemingly impressive, with SCD rate
reduced to 0.4/100,000 patient-years:
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Some have argued that an EKG should be the part of
every pre-participation evaluation in the USA.
Barry Maron argues otherwise:
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US and Italian SCD rates are equal already (2011 Harmon data
would disagree)
Can the American sedentary population not be screened? (most
sudden deaths from genetic cardiac disease occurs in nonathletes!)
Cost considerations:
 False positive cases are much more frequent than true positive
cases, and are COSTLY.
 The annual cost would be $2 billion (just for the competitive
athlete population)
 Cost per prevented death = $3.4 million.
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The US does not have the physician resources for this program
(excess medical students in Italy)
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Israeli data, 2011
Mandatory screening, including EKG (yearly) and ETT
(q4 yrs) of all athletes, since 1997.
 Systematic search of newspaper events (felt to be
inclusive)
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So what should we do with conflicting data in
the setting of limited resources?
Recall that >50% of HCM SCD’s occur in the
African American population, BUT, this
population is under-represented in those
followed clinically with that condition.
Recall that most SCD’s occur in non-athletes
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(bigger denominator)
Recall that SCD of athletes represent 1.5% of
deaths in teens
For perspective, . . . . . . ,
Death in teens age 15-19 years of age
MVA, other
accidents, homocides,
suicides 77%
Athlete SCD 1.5%
All others 22.5%
National Vital Statistic Report 2005, Maron-Harmon Data
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Do NOW what we know works
 AHA/ACC H and P screening of all athletes: “Adherence
to uniform guidelines would result in the identification of many
more athletes with cardiac disease….” (AHA recs, 2007)
 Pediatricians should consider including the elements of
the 12 point screen in all well child exams.
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Allocate our limited health care funds where we
know they will have the greatest impact! The heart
is important, but many more kids are dying in
accidents, by homicide, and of suicide.
Definitively determine if EKG screening works
better than the AHA pre-participation H and P.
And if it does, . . . ,
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SCD is a huge problem for the individual, and
so must be taken seriously.
HCM and congenital coronary anomalies are
the most common causes of SCD.
The PCP is the key to identifying high risk
kids, and a good H & P, including the 12
elements of the AHA screen, are critical.
Screening EKG’s may play a role.
Let’s save some lives!
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Please see your hand-out for a complete list
These are highly recommended:
Sudden Deaths in Young Competitive Athletes,
Maron et al, Circulation, 2009.
ACC/AHA: Recommendations and
Considerations Related to Pre-participation
Screening for Cardiovascular Abnormalities in
Competitve Athletes: 2007 Update. Maron et al,
Circulation, 2007.
Recommendations for Interpretation of 12-lead
Electrocardiogram in the Athlete, Corrado et al,
European Heart Journal, 2010.