Sudden Cardiac Death in Children

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Coarctation of the Coarctation of the Aorta Aorta postoperative hypertension noted postoperative hypertension noted beyond the 10th postoperative yr: beyond the 10th postoperative yr: -- alive and well and -- alive and well and normotensive normotensive 70% at 10 yrs 70% at 10 yrs 65% at 15 yrs 65% at 15 yrs 20% at 25 yrs. 20% at 25 yrs. arm leg gradient with exercise arm leg gradient with exercise average is 80 mm Hg. average is 80 mm Hg.

Transcript of Sudden Cardiac Death in Children

Page 1: Sudden Cardiac Death in Children

Coarctation of the AortaCoarctation of the Aorta

postoperative hypertension noted beyond postoperative hypertension noted beyond the 10th postoperative yr:the 10th postoperative yr:

-- alive and well and normotensive-- alive and well and normotensive

70% at 10 yrs70% at 10 yrs

65% at 15 yrs65% at 15 yrs

20% at 25 yrs.20% at 25 yrs. arm leg gradient with exercise average is arm leg gradient with exercise average is

80 mm Hg.80 mm Hg.

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SUDDEN DEATH in YOUNG SUDDEN DEATH in YOUNG ATHLETESATHLETES

Maron, et al, Circ 1980Maron, et al, Circ 1980

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Clinical FindingsClinical Findings

asymptomatic 21/29asymptomatic 21/29 syncope 3/29syncope 3/29 presyncope 1/29presyncope 1/29 chest pain 2/29chest pain 2/29 mild fatigue 2/29mild fatigue 2/29

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Circumstances of DeathCircumstances of Death

death during or after severe exertion: death during or after severe exertion: 22/2922/29

death occurred during mild exertion: death occurred during mild exertion: 2/292/29

death occurred during sedentary death occurred during sedentary activity: 5/29activity: 5/29

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Causes of Sudden DeathCauses of Sudden Death

29292929

Probable CV DiseaseProbable CV Disease

66

55 11IdiopathicIdiopathicConcentric hypertrophyConcentric hypertrophy

(no fiber disarray)(no fiber disarray)

Hypoplastic coronariesHypoplastic coronaries

11

No CV diseaseNo CV disease

2222 Unequivocal CV dis.Unequivocal CV dis.

22Ruptured aortaRuptured aorta

33Atherosclero. CAAtherosclero. CA

33 ALCAPAALCAPA

1414

HOCM**HOCM**

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Magnitude of the ProblemMagnitude of the Problem

excluding trauma, cardiac death is the excluding trauma, cardiac death is the most frequent cause of sports related most frequent cause of sports related death.death.

5/100,000 have a condition which 5/100,000 have a condition which predisposes them to sudden death.predisposes them to sudden death.

1/200,000 athletes per yr have sudden 1/200,000 athletes per yr have sudden deathdeath

~12 high school ath. die/yr in U.S.~12 high school ath. die/yr in U.S.

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Types of SportsTypes of Sports

basketball 33%basketball 33% football 20%football 20% running 16%running 16% swimming 4.8%swimming 4.8% wrestling 3.8%wrestling 3.8% volleyball 2.9%volleyball 2.9% tennis 2.9%tennis 2.9% baseball 2.9%baseball 2.9%

GOLF<1%GOLF<1%

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Hypertrophic Cardiomyopathy Hypertrophic Cardiomyopathy and Sudden Deathand Sudden Death

Annual mortality rate 2-4%Annual mortality rate 2-4% Mechanism probably acute Mechanism probably acute

dysrhythmia(v.tach, v.fib., asystole)dysrhythmia(v.tach, v.fib., asystole) Sudden death most common 10-25 yrs.Sudden death most common 10-25 yrs. Peak age is 14 yrs.Peak age is 14 yrs. Approx. 40% occur during ahtleticsApprox. 40% occur during ahtletics If there is documented v. tach on If there is documented v. tach on

holter,death rate 8%. holter,death rate 8%.

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HCM and Sudden DeathHCM and Sudden Death

Increased risk of sudden death ass. with: Increased risk of sudden death ass. with: documented v. tach, family hx. of sudden documented v. tach, family hx. of sudden death, young age of onset of symptoms.death, young age of onset of symptoms.

Sudden death not related to presence or Sudden death not related to presence or degree of outflow gradient.degree of outflow gradient.

NO INTERVENTION(SURG,MEDICAL)NO INTERVENTION(SURG,MEDICAL)

HAS BEEN SHOWN TO DECREASE RISK HAS BEEN SHOWN TO DECREASE RISK OF SUDDEN DEATH.OF SUDDEN DEATH.

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Abnormal Origin of CA’s and Abnormal Origin of CA’s and Sudden DeathSudden Death

Left CA from right cusp is the most common Left CA from right cusp is the most common cause of sudden death.cause of sudden death.

Potential mechanisms: coronary comes off Potential mechanisms: coronary comes off tangentially from the aorta, ostium may be slit tangentially from the aorta, ostium may be slit like,ostium may be partially covered by flap like,ostium may be partially covered by flap valve, initial few mm’m may be in wall of aorta.valve, initial few mm’m may be in wall of aorta.

97% die at < 22 yrs of age97% die at < 22 yrs of age Rule out in pat with exercise chest pain or Rule out in pat with exercise chest pain or

syncope . Tx. surgicalsyncope . Tx. surgical

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OTHER CAUSES of SUDDEN OTHER CAUSES of SUDDEN DEATH in ATHLETESDEATH in ATHLETES

Marfan Syndrome: related to aortic Marfan Syndrome: related to aortic rupture.rupture.

Myocarditis: may be associated with Myocarditis: may be associated with acute inflammation and chronic acute inflammation and chronic multifocal scarring-- arrthymiasmultifocal scarring-- arrthymias

Drugs: anabolic steroids predispose to Drugs: anabolic steroids predispose to thrombotic MI, CVA, and thrombotic MI, CVA, and cardiomyopathy. cardiomyopathy. COCAINECOCAINE

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Other Causes,Other Causes,

Primary dysrhythmias:Primary dysrhythmias:

a. sudden death reported with SVT,long a. sudden death reported with SVT,long QT, SSS.QT, SSS.

b. exercise syncope most common b. exercise syncope most common presentation.presentation.

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SCREENING?SCREENING?

Scale: to identify 1000 atheletes at risk, Scale: to identify 1000 atheletes at risk, 200,000 would have to be screened to 200,000 would have to be screened to

prevent prevent 11 death.death. Routine screening by ECHO impracticalRoutine screening by ECHO impractical Routine EKG’s on all athletes probably Routine EKG’s on all athletes probably

impractical.impractical.

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SCREENING?SCREENING?

SMA 1: history and PESMA 1: history and PE

focused hx of syncope, chest focused hx of syncope, chest pain, or seizures in patient- always ask pain, or seizures in patient- always ask about sudden death in family membersabout sudden death in family members

focused PE looking for path. focused PE looking for path. murmur, gallop, or S4, obvious ectopymurmur, gallop, or S4, obvious ectopy

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LONG TERM EXPERIENCE LONG TERM EXPERIENCE AFTER CARDIAC SURGERYAFTER CARDIAC SURGERY 60% of important CHD:60% of important CHD:

VSDVSD

ASDASD

PSPS

PDAPDA

CoACoA

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Long Term Experience,Long Term Experience,

Surgery for uncommon lesions- has Surgery for uncommon lesions- has been available for 25 yrs.been available for 25 yrs.

TGATGA

TATA

Single ventricleSingle ventricle These patients are now showing up in These patients are now showing up in

adult clinics.adult clinics.

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RESIDUAE & SEQUELAE of RESIDUAE & SEQUELAE of CONGENITAL HEART CONGENITAL HEART

SURGERYSURGERYIt ain’t over, til it’s overIt ain’t over, til it’s over

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Surgical Residuae & SequelaeSurgical Residuae & Sequelae

Obstructive lesionsObstructive lesions HypertensionHypertension ShuntsShunts pulm. artery hypertension/ distortionpulm. artery hypertension/ distortion valve regurgvalve regurg

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Surgical Residuae & SequelaeSurgical Residuae & Sequelae

ArrhythmiasArrhythmias Systemic right ventricle- TGASystemic right ventricle- TGA

Mustard or SenningMustard or Senning Fontan physiology- physiologic Fontan physiology- physiologic

correction with single ventricle chambercorrection with single ventricle chamber

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Coarction of the AortaCoarction of the Aorta

50-85% incidence of bicuspid Ao valve.50-85% incidence of bicuspid Ao valve.

-- Late developement of stenosis/insuf--- Late developement of stenosis/insuf-

ficiency.ficiency.

Associated with calcific changesAssociated with calcific changes

midlife eventmidlife event

-- infective endocarditis-- infective endocarditis >50% have mitral abnormalities>50% have mitral abnormalities

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Coarctation of the AortaCoarctation of the Aorta

Associated abnormalities:Associated abnormalities:

-- intracranial aneurysms-- intracranial aneurysms

-- late aortic dissection-- late aortic dissection

-- intramural coronary artery disease-- intramural coronary artery disease

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Coarctation of the AortaCoarctation of the AortaAortic aneurysmsAortic aneurysms

With dacron onlay patchesWith dacron onlay patches

-- 38% incidence of aneurysms-- 38% incidence of aneurysms Aortic balloon angioplastyAortic balloon angioplasty

-- incidence of aneurysms unknown-- incidence of aneurysms unknown

native vs recoarc. For recoarctation,native vs recoarc. For recoarctation,

balloon is procedure of choiceballoon is procedure of choice

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Coarctation of the AortaCoarctation of the Aorta

Surgical results; aim for gradient < 10Surgical results; aim for gradient < 10

30-40% have recurrent gradient when 30-40% have recurrent gradient when surgery done at less than 1yr.surgery done at less than 1yr.

Significant late mortality-Significant late mortality-

--10-20% have resting hypertension--10-20% have resting hypertension

This is directly related to age at This is directly related to age at surgery.Exercise testing will provock surgery.Exercise testing will provock gradient.gradient.