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General Pediatric Board Review: General Pediatric Board Review:

General Pediatric Board Review: - PowerPoint Presentation

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General Pediatric Board Review: - PPT Presentation

Pediatric Cardiology Daniela Rafii MD Associate Director Pediatric Cardiology Maimonides Infants and Childrens Hospital of Brooklyn Innocent Heart Murmurs Over 50 of children have an innocent heart murmur ID: 616997

murmur heart left treatment heart murmur treatment left cyanosis infant vsd ekg chf pda babies coarc surgical chd pulmonary shunt diagnostic failure

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Slide1

General Pediatric Board Review:Pediatric Cardiology

Daniela Rafii, M.D.Associate Director, Pediatric CardiologyMaimonides Infants and Children’s Hospital of BrooklynSlide2
Slide3
Slide4
Slide5

Innocent Heart Murmurs

Over 50% of children have an innocent heart murmur  no intervention, require reassuranceMost common innocent heart murmurs:Still’s murmur AKA function heart:Musical/twangy/vibratory systolic ejection murmurLouder while supineUsually located over the LLSBVenous hum: Continuous murmurSofter/resolve while supine or w/ pressure to jugular vein or w/ turning the head

Usually RUSB or LUSBSlide6

Pathologic Murmurs

AS  Harsh crescendo-decrescendo SEMRUSB

Radiates to the neck

PS 

Harsh

crescendo-decrescendo

SEM

LUSB

Radiates to the back

PDA  continuous machinery murmur

Diastolic murmurs are never innocent Slide7

Upon routine 3 year physical, a 3 yo female who is new to the practice is noted to have a continuous grade 2/6 continuous murmur at the RUSB while sitting. When

supint, she is noted to have a 3/6 vibratory systolic ejection murmur at the LLSB only. The remainder of the PE is norma. Of the following, the MOST appropriate next step is:Reassure the parents about the benign prognosis

Request a cardiology consult

Order a chest X ray

Request a transthoracic echocardiogram

Request an EKGSlide8

Acyanotic Congenital Heart Disease (CHD)

Acyanotic (pink) CHD ASD (left to right shunt)VSD (left to right shunt)PDA (left to right shunt)ASPSCoarcSlide9

Atrial Septal Defect (ASD)Slide10

ASD

Acyanotic CHD3 typesPrimum

Secundum

(most common)

Sinus

Venosus

Asymptomatic in childhood

SEM loudest at LUSB

 relative PS murmur w/ fixed widely split S2Slide11

ASDEKG w/ right ventricular conduction delaySlide12

ASDDx

 echoTreatment  surgical vs percutaneous closureNatural history in the 3rd-4th decade of life: Arrhythmias

Pulmonary HTN

Paradoxical emboli Slide13

Ventricular Septal Defect (VSD)Slide14

VSD

Acyanotic CHDMost common CHD4 main types of VSD

Perimembranous

Muscular

Inlet

Outlet

Small VSDs

 no

sx

only loud harsh 3/6

holosystolic

murmur loudest at LLSB

Large VSDs  congestive heart failure (CHF)Slide15

VSD & CHF

CHF x typically start at 6-8 weeks old from a VSDBabies present with:Some degree of resp distress: tachypnea, retractions, abd

breathing,

etc

Irritability

Hepatomegaly

Cardiomegaly

NO peripheral edema

NO cyanosis

Poor

po

intake, easy fatiguing w/ feeds, prolonged feeding times

Poor weight gain: weight drops off before height

Excessive sweating

Sinus tachycardiaLoud harsh

holosystolic murmur loudest at the LLSBSlide16

CXR

CardiomegalyInc lung markingsPulm edemaSlide17

VSDEKG:

NormalSinus tachyLVHEcho: diagnostic Slide18

VSD TreatmentIf CHF treat with:

Digoxin 5mcg/kg/dose bidLasix 1mg/kg/dose bidEnalapril  afterload reductionIncrease caloric intake  24kcal/oz formulaSurgical closure of large VSDs ~ 6 mo old  sooner if FTT despite medical trt CHFSlide19

VSD Natural HistorySmall VSDs:

80% close within the 1st year of lifeLarge VSDs:CHF  40% of babies will die w/o treatment in 1st year of lifeUnrepaired large VSDs past 1yo  develop Eisenmenger’s syndrome in teen yearsProgressive pulmonary HTN w/ progressive cyanosis

Right to left shunt

Mean survival mid-20’sSlide20

Atrioventricular Canal

AKA: AV Canalendocardial cushion defectAV septal defectSlide21

AV Canal

~50% of children w/ T21 have CHD~40% of those with T21 and CHD have AVCEKG: often has left superior axis

Echo: diagnostic

Treatment:

Medically manage CHF (same as w/ large VSD)

Surgical repair 4-6 months oldSlide22

AVC EKGSlide23

Patent Ductus Arteriosus (PDA)Slide24

PDA

All babies are born with a PDAUsually closes within 1st 2 wks of life in full term babies

Persists longer in preterm babies

Small PDAs

 no symptoms

Large PDAs  CHF

sx

similar to large VSDs

In preemies 

resp

distress can be seen in the 1

st

few days of lifeSlide25

PDAClassic murmur

 continuous machinery murmur secondary to continuous shunt in systole and diastole loudest under L clavicleMurmur is not heard in newborns secondary to elevated pulm vascular resistance (PVR)Murmur develops only after PVR sropsSlide26

PDA Treatment

If significant sx in preterm babies IndomethacinMay try CHF rx

Surgical PDA ligation

If CHF

sx

in term babies

Medically treat CHF

If babies can reach 10kg  percutaneous PDA closure

If

cannot gain weight

 surgical PDA ligation Slide27

A 3 month old female with T21 presents with 1 month history of poor weight gain and tachypnea. She has an active precordium and soft systolic murmur on exam. Chest X-ray demonstrates cardiomegaly and increased lung markings. EKG demonstrates a left superior axis. Of the following, the MOST likely diagnosis is:

Coarctation of the aortaAtrioventricular canal defect

Patent ductus arteriosus

Large ventricular septal defect

Secundum

atrial septal defectSlide28

Patient is a 5 day old infant born at 31 weeks gestation on

ventilatory support in the NICU. He has a hyperdynamic precordium, bounding peripheral pulses, and a continuous heart murmur. His Hgb, electrolytes, and creatinine are all normal. Of the following, the most appropriate INITIAL management is to:

Administer furosemide intravenously

Administer indomethacin intravenously

Perform an echocardiogram

Defer interventions since spontaneous closure is likely

Obtain a surgical consult for PDA ligationSlide29

You are called to evaluate a 6 hour old female. Labor and delivery were uncomplicated, however amniocentesis at 20 weeks revealed T21. The infant is sleeping, has facial features consistent with T21, he is well perfused, heart rate is 140 bpm, and there are no audible murmurs. Of the following, the MOST appropriate diagnostic study to perform is:

Barium swallowCervical spine radiography

Echocardiogram

Head ultrasound

Radiography of the abdomen Slide30

Coarctation of the AortaSlide31

Coarctation

More common in malesAlmost always juxtaductal [A]Preductal

[B]

 present earlier

Postductal

[C]  present later

85% of children with

coarc

have a BAV

Frequently

seen in Turners syndrome {45, XO}

10% have severe

coarc

30% have bicuspid aortic valve (BAV)Slide32

CoarcVariable presentation

Infant with cardiogenic shockChild or adolescent with systemic hypertensionChild with a heart murmurSlide33

Neonatal Coarctation

Severe coarc in a neonate Often present before 2 weeks oldCardiogenic shock: resp distress/failure, poor perfusion, altered metal status Multisystem organ failure: NEC, renal failure, intracranial bleed**Diminished lower extremity pulsesModerate coarc in a neonate CHF: resp distress, poor feeding, poor growthMild coarc in a neonate 

No symptoms +/- murmurSlide34

Older Children with Coarc

Older children and adolescents do NOT present with heart failure sxCan have upper extremity hypertension refractory to antihypertensive rx

Can have diminished lower extremity pulses

Can have claudication

Can just have a murmurSlide35

Coarc

EKG:Often normalCan have LVH with strain pattern (ST elevation +/- T wave inversion in precordial leads)

CXR: often nonspecific findings, normal or:

Cardiomegaly

Increase

pulm

vascular markings

Rib notching (not seen in infants, uncommon prior to 5 years old)Slide36

Coarc

Echo: diagnosticTreatment:Infant in shock  immediate PGE + aggressively trt shockSurgical repair for children or for complex coarcPercutaneous stent placement in adult sized patients Slide37

Hypoplastic Left Heart Syndrome (HLHS)Slide38

HLHSVarying degrees of left heart hypoplasia

Babies present in cardiogenic shock when PDA closesImmediate treatment with PGE IV infusionSlide39

HLHS

Surgical treatment:Norwood palliation  1st week of lifeBidirectional Glenn  4-6 months oldFontan palliation  2-4 years oldSlide40

A 7 month old female has undergone the second stage of surgical palliation (Glenn) for

hypoplastic left heart syndrome. She was discharged from the hospital 1 week ago. Her mother brings her to the office because of irritability that began in the morning. On physical exam, she is awake and irritable, heart rate 150 bpm and respiratory rate of 50 bpm. She has cyanosis of the face and mucosal surfaces and welling of the arms and head. Of the following, the BEST explanation for this patient’s clinical presentation is:

Polycythemia

Postpericardiotomy

syndrome

Protein-losing enteropathy

Superior vena cava syndrome

Thoracic duct injurySlide41

Pulmonary Stenosis (PS) and Aortic Stenosis (AS)Slide42

PS AS

Stenosis may be valvular, subvalvar, or supravalvarMild-mod PS  no

sx

Severe or critical PS  cyanosis

Murmur  harsh SEM at LUSB

Echo –> diagnostic

Treatment: ballooning or surgical

Stenosis may

valvular

,

subvalvar

, or

supravalvar

More common in males

More significant lesion compared to PS, no cyanosis, (+) heart failure/cardiogenic shock

Valvar AS is often associated w/ BAV

Murmur

 harsh SEM at

RUSB

Echo –> diagnostic

Treatment: ballooning or

surgicalSlide43

Hypertrophic Cardiomyopathy (HCM)Mild to severe usually asymmetric thickening of the myocardium

Often autosomal dominantIncidence 1:500Symptoms: Sudden death (on exertion)ArrhythmiasSyncopeChest painSlide44

A 3 day old infant is found unconscious in her crib and brought to the ED. Findings include: tachypnea, tachycardia, pallor, poor cap refill, hepatomegaly, cardiomegaly with increased pulmonary vascular markings,

hgb 17 gm/dl, hematocrit 51%. Of the following, the cardiogenic shock in the girl is most likely due to :Critical aortic stenosis

Erythroblastosis

fetalis

Patent ducts arteriosus

Severe hypovolemia

Ventricular septal defectSlide45

A 6 hour old infant has increasing pallor, tachypnea and

resp distress. Pysical exam reveals an enlarged liver, gallop, poor pulses in the upper extremities and absent pulses in the lower extremities. In addition to treating the infant for sepsis, the most appropriate INITIAL management is to administer:

Dopamine infusion

Loading dose of digoxin

25% glucose and water solution

Furosemide

Prostaglandin E1Slide46

Cyanotic CHD

Cyanotic (blue) CHD  Often have cyanosis with NO resp distress

Need

some sort of L to Right shunt to have cyanosis

TOF (right to left shunt)

TGA

Tricuspid

atresia (right to left shunt)

Truncus arteriosus

TAPVR

Ebstein’s

Anomaly

(right to left shunt)

Single VentriclesSlide47

Tetralogy of Fallot (TOF)Slide48

TOF

PSVSD

Overriding aorta

Right ventricular hypertrophy (RVH)Slide49

TOF

Most common cyanotic heart lesionSigns/symptoms:CyanosisLoud harsh SEM at the LUSB (PS murmur)Squatting in older kidsTet spells  cyanosis often worsened or caused by cryingLose PS murmur b/c less blood across pulm

valve

Increase right to left shunt across VSD

Trt

 calm kid down, knees to chest, morphine, oxygen, general anesthesiaSlide50

Transposition of the Great Arteries (TGA)Slide51

TGA

Aorta arises from the RV and the pulmonary artery arises from the LVMixing of blood occurs at the PFO/ASD, PDA, +/-VSDSx: Cyanosis

+/- murmur

May have higher

sats

in the lower

e

xtremities vs the upper extremities

Echo: diagnostic Slide52

TGASlide53

Tricuspid Atresia (TA)Slide54

TATA

 left superior axis on EKG (like AVC)Slide55

Truncus ArteriosusSlide56

Total Anomalous Pulmonary Venous Return (TAPVR) Slide57

TAPVRSx occur when pulm veins are obstructed

Can occur soon after birthBabies present with severe cyanosis (unresponsive to O2) and resp distressNo murmurTreatment  emergent surgerySlide58

TAPVRSlide59

Ebstein AnomalySlide60

Ebstein

AnomalyApical displacement of the tricuspid valveCan present with:Severe TRR to L shunting  neonatal cyanosis25% can have SVT or WPW

Neonatal treatment  decrease PVR

iNO

Oxygen

CXR  severe cardiomegalySlide61

The mother of a 5 month old reports that following a feeding, the child began to breath deeply, became increasingly blue and then lost consciousness. After being held briefly, the infant regained her usual color and became alert. Physical examination reveals a harsh murmur. Of the following the MOST likely diagnosis is:

Aortic stenosisCoarctation

of the aorta

Myocarditis

Tetralogy of Fallot

Ventricular

s

eptal defectSlide62

You are called at 3am from the nursery about a 36 hour old BB Bleu who was noted to be cyanotic . The nurse reported that he had been feeding well and appeared well prior to the episode.

Apgars were 9/9. Until this evening the baby appeared pink. They report no significant tachypnea. You order a chest X-ray and pulse oximetry to be done while you rush to the hospital. On arrival the pulse oximetry indicates a saturation of 55% and the X-ray shows no increase pulmonary vascular marking or infiltrate. The next MOST appropriate intervention is to:

Obtain a stat EKG to evaluate for SVT

Intubate the infant and place on 100% O2

Start IV PGE infusion at 0.05-0.2mcg/kg/min

Start

iNO

at 40ppm inspired to reduce PVR

Arrange for transfer to a facility capable of ECMOSlide63

Following an uncomplicated delivery, a 3.7kg term infant develops cyanosis in the first hour of life. Physical exam reveals: HR 140 bpm, RR 56 bpm, no heart murmur, on room air the right arm saturation is 70% and the right leg saturation in 75%, on 100% O2 the right had saturation increased to 75 and the right foot increases to 90%. Chest X-Ray is normal. These findings are MOST consistent with:

Primary pulmonary hypertension of the newborn

Pulmonary valve atresia

Transient tachypnea of the newborn

Transposition of the great arteries

Truncus

arteriosus Slide64

At 1 hour old at full term 3.5kg infant appears cyanotic but otherwise well. Oxygen saturation in the upper and lower extremities is 79%, there is a soft systolic murmur heard across the precordium. The remainder of the physical exam is within normal limits. After placing the baby on O2,there is no change in saturations. Of the following, the MOST likely cause of this child’s findings is:

Anemia

Hypoplastic

left heart syndrome

Neonatal sepsis

Retained fetal lung liquid syndrome

Tracheoesophageal

fistulaSlide65

Arrhythmias Slide66

Supraventricular Tachycardia (SVT)

Most common arrhythmia in childhoodBabies present with poor feeding, pallor, irritabilityOlder kids present w/ palp, dizziness, fatigue

EKG diagnostic

 n

arrow complex regular

tachy

>220bpm

Can be associated with WPW

If patient hemodynamically stable

 vagal maneuvers:

Ice to face

Valsalva

If doesn’t work  adenosine

If patient hemodynamically unstable  synchronized cardioversionSlide67

Valsalva ManeuversIce to face

“Bearing down”Blowing against and occluded strawGagCoughSlide68

SVTSlide69

Atrial Flutter/Fib

Atrial flutter: saw tooth p wavesAtrial fib: irregularly irregularAdenosine is diagnostic not therapeuticTreat both with synchronized cardioversionSlide70

Heart Block

1st degree HB: prolonged PR intervalRheumatic fever, myocarditis, KD, congenital2nd degree HB:Type I: Wenkebach, progressive prolongation of PR interval then dropped beat  high vagal tone, benignType II: dropped (

nonconducted

beats)  can progress to CHB  pacemaker

3

rd

degree: complete HB

Postop,

lyme

disease, myocarditis

Congenital CHB  maternal SLESlide71

Long QT Syndrome

QTc >0.45 secGenetic  often family hx sudden deathArrhythmia  torsades de pointes (type of VT)Be suspicious if:Family hx SCDHx seizure d/oCongenital deafnessSyncope following loud noises, being startled Acute treatment: magnesium

Long term treatment: beta blockersSlide72

Torsades de PointesSlide73

Commotio Cordis

Sudden, blunt, non-penetrating trauma to the chest  V fib  sudden deathTypical story: healthy kid gets hit with a baseball to the chest and dropsTreatment  stat defibSlide74

Syncope

Benign causes of syncope:Vasovagal: during blood draws, site of bloodOrthostatic hypotension: standing up quickly, standing up for too long, especially if hot Hyperventilation

Breath holding spells: 6-18mo, associated w crying, +/- cyanosis

Concerning, potentially life-threatening syncope:

Associated w/ exertion (VT, LQTS, HCM, other cardiomyopathy)

Associated w/ excitement/startle (LQTS)

Family

hx

sudden deathSlide75

Sudden Death in Young AthletesSlide76

Carditis

Carditis refers to inflammation of any of the 3 layers of the heart, occur in isolation or conjunction with one anotherEndocarditis  inflammation of the cardiac valves  valvar dysfunctionMyocarditis  inflammation of the muscular walls of the heart  myocardial dysfunction, conduction abnormalities (heart block, arrhythmias)Pericarditis  inflammation of the pericardium  pericardial effusionSlide77

Infective Endocarditis (IE)

Diagnosis  Modified Duke Criteria 2 major1 major + 3 minor5 minorTreatment  IV abx, type and length of treatment varySlide78

Major Criteria

Positive Bcx w/ typical IE microorganism: Typical microorganism consistent w/ IE from 2 separate Bcx: Viridans-grp strep, or Strept bovis including nutritional variant strains, or HACEK group (Haemophilus spp, Actinobacillus, Cardiobacteriom hominis, Eikenella spp, Kingella), or Staph aureus, or

Community-acquired Enterococci, in the absence of a primary focus

Microorganisms consistent w/ IE from persistently (+) Bcx:

2 positive Bcx drawn >12 hours apart, or

All of 3 or a majority of 4 or more separate Bcx (w/ first and last sample drawn at least 1 hour apart)

Coxiella burnetii on at least 1 (+) Bcx or antiphase I IgG antibody titer >1:800

Evidence of endocardial involvement w/ (+) echo:

Oscillating intracardiac mass on valve or supporting structure, or in the path of regurgitant jet, or on implanted material, or

Abscess, or

New partial dehiscence of prosthetic valve or new valve regurgitationSlide79

Minor Criteria

Predisposing factor: CHD, recreational IV drug use Fever >38°C Evidence of embolism: arterial emboli, pulmonary infarct,

Janeway

lesions, conjunctival hemorrhage, mycotic aneurysm,

intracarnial

hemorrhage

Immunological

problems: glomerulonephritis, Osler’s nodes, Roth spots, rheumatoid factor

Positive

Bcx

(that doesn't meet a major criterion) or serologic evidence of infection w/ organism consistent with IE but not satisfying major criterionSlide80

Minor Criterea

Janeway lesions: small, erythematous, non-tender, macular or nodular lesion on soles/palms  septic emboli

microbscesses

Osler’s nodes: painful

, red, raised lesions on

hands/feet

immune

complex

deposition

Roth spots: retinal

hemorrhages w/ white or pale centers composed of coagulated fibrin  immune complex mediated vasculitis Slide81

Subacute Bacterial Endocarditis Prophylaxis

2007 AHA guidelines:Prosthetic valvesPrevious IE

Unrepaired cyanotic heart disease

Repaired CHD <6 months after surgery

Repaired CHD >6mo if residual lesion near prosthetic material

Cardiac transplant with

valvulopathy

Slide82

Myocarditis

Myocarditis  inflammation of the muscular walls of the heart  myocardial dysfunction/failure, heart block, arrhythmiasPresentation in babies  irritability, poor feeding, pallor, shock, cardiomegaly, hepatomegaly, pulm edemaPresentation in older kids  fatigue, dyspnea, chest pain, palpitations, pallor, hypotension, cardiomegaly, hepatomegaly,

pulm

edemaSlide83

Myocarditis

Infectious vs noninfectious vs idiopathicViral  most common cause in US: coxsackie, adenovirus, parvovirus B19, enterovirus, EBV, CMV, HSV 6Other infectious  Lyme dz and other spirochetes, fungal, bacterial

Toxic/

hypersenstivity

reactions 

chemotherapeutic agents,

abx

, amphetamines

Systemic

dz

Giant-cell myocarditis, sarcoidosis, KD, Crohn’s, UC, SLE, thyrotoxicosisSlide84

Myocarditis

No single clinical or imaging test to confirm dxDx made by:Clinical sxSerologic: CKMB, troponin, BNP, CRP

Noninvasive: EKG, echo, MRI

Invasive: myocardial

biopsy

Treatment: depends on severity of

sx

, mostly supportive: ionotropic support, mechanical vent, ECMO,

trt

CHF

,

antiarrhythmics

,

temporary pacing, IVIG or steroids

Long term: fully recover (50%), chronic heart failure, need for transplant Slide85

Pericarditis

Viral pericarditis most commonPreceded by a viral URISx: sharp chest pain, improves with leaning forward

PE: friction rub, pulses paradoxus (exaggeration of normal finding of

dec

in systolic BP w/ inspiration)

EKG diagnostic: diffuse ST segment elevation

Echo: pericardial effusion

Risk

 cardiac tamponade

Treatment  NSAID

,

pericardiocentesis

if severe Slide86

In addition to irritability, sweating and difficulty breathing with feeding, the symptom that is MOST indicative of congestive heart failure is a 3 week old infant is:

AscitesCough

Cyanosis

Diminished feeding volume

Pretibial edemaSlide87

A term infant was born with a large ventricular septal defect. At what age is the infant MOST likely to first demonstrate clinical findings of congestive heart failure?

2 days2 weeks

2 months

6 months

12 monthsSlide88

Rheumatic Fever

ARF results from a complex interaction btw GAS and a susceptible hostAbnormal immune response leads to acute inflammation of joints, brain, heart, and or skinAll organ systems recover w/o sequelae except the heartARF can lead to chronic RHDDiagnosis

modified Jones CriteriaSlide89

Rheumatic Fever

2 major or 1 major + 2 minor

And evidence of a preceding streptococcal infection

Except w/ presence of chorea or indolent carditis

Major criteria:

Polyarthritis

Carditis

Sydenham’s chorea

Erythema marginatum

Subcutaneous nodules—extensor surf

Minor criteria

Fever

Polyarthralgia

Elevated acute phase reactants (ESR, CRP)

Prolonged PR intervalSlide90

RF Cardiac Involvement

Initial cardiac involvement is a pancarditisMost prominent feature is valve involvement #1 mitral (MR)#2 mitral + aortic (MR + AI)#3 aortic (AI)Treatment:

Primary

ppx

: Treat strep infection

High dose aspirin or NSAIDs or steroids (4-6 weeks)

Secondary

ppx

: monthly IM PCN

Long term RHD: MR + AI

 MR/MS + AI/ASSlide91

Kawasaki DiseaseArteritis of medium size arteries

AKA mucocutaneous lymph node syndromeDiagnosis  5 days of fever + 4/5 of the following: B/l nonexudative conjunctivitisErythema of the lips or oral mucosa

Changes in the extremities: swelling, erythema

Rash

Cervical LAN, >1.5cmSlide92

Typical Kawasaki Disease

Supplemental laboratory criteria:

Albumin </=3.0 g/

dL

Anemia for age

Elevation of ALT

Plt

after 7d >/=450

WBC >/= 15

Urine 10 WBC/high-power field

Newburger, J. W.

et al

. Circulation 2004;110:2747-2771Slide93

Kawasaki DiseaseTreatment for typical and atypical KD is the same

IVIG w/i 10 days of fever, may repeat if still febrileHigh dose aspirin W/o treatment  20% of children will develop coronary artery aneurysmsW/ treatment w/ IVIG and ASA5% of pts will develop

aneurysms

Aneurysms develop usually w/

i

2 weeks

Can have an associated mild myocarditis Slide94

Syndromes

T21: CHD incidence 50%, think AV canal defectsTurner’s: 10% coarc, 30% BAV

Pompe’s

:

Hypertrophic

cardiomyopathy (HCM)

Alagille

: Peripheral pulmonic stenosis (PPS

)

Williams’s:

Supravalvar

aortic stenosis, PPS

Noonan:

PPS and (HCM)Marfan’s: Aortic root dilatation, MVP

DiGeorge (22q11 del): TOF, truncus arteriosus, interrupted Ao

arch, right aortic arch

Kartagener:

dextrocardia

, situs

inversus

totalis

,

immotile cilia

Holt-

Oram

: Limb

abnormalities, ASD

Ellis-van

Creveld

:

ASDSlide95

Teratogens

Lithium: Ebstein’s anomalyEthanol: ASD,VSD (fetal alcohol syndrome)Anticonvulsants: PS, AS, TOFRetinoic Acid: TGARubella: PDA, PPSCoxsachie B: Neonatal myocarditisMaternal Diabetes: HCM, TGAMaternal Lupus: Complete heart blockPKU: VSD, ASD, complex CHDSlide96

A 5 year old girl is very excited following a ride on a

ferris wheel. In the midst of her excitement she suddenly loses consciousness and falls to the ground. Paramedics on the scene document a ventricular tachycardia. Family history reveals a maternal uncle who died suddenly at 16 years old. Following treatment of her ventricular tachycardia, an EKG is most likely to demonstrate:

Corrected QT interval of 0.52 sec

P wave axis of -30 degrees

PR interval of 0.81 sec

QRS axis of -15 degrees

QRS interval of 0.12 secSlide97

Julie, an otherwise healthy 9 year old comes to the ED because she “passed out”. After asking questions and examining the patient, all but one of the following reassures you that she has vasovagal syncope, a relatively benign cause of syncope in children:

She was standing in line waiting to see “The Hunger Games: Mockingjay part 2” when she passed out

She fainted once before when she had a blood test

After falling to the ground she came to quickly and remembered feeling warm and dizzy

She was lying on the sofa watching TV when a door slammed and she suddenly became unresponsive

S1 and S2 were normal and no murmurs were notedSlide98

A 13 year old boy wishes to participate in competitive sports. His father died suddenly at age 28 years, and hypertrophic cardiomyopathy was found on autopsy. Of the following , the MOST helpful test for assessing the boy’s risk is:

EchocardiographyElectrocardiography

Exercise

myocardial perfusion scintigraphy

Genetic

testing for myosin chain mutations

Genetic

testing for troponin

mutationsSlide99

Two weeks after a nonspecific upper respiratory infection, a previously healthy , 3 year-old boy is noted to have a resp. rate of 40 breaths/min, a HR of 140 beats/min, hepatomegaly and a gallop rhythm. No heart murmurs are detected. Of the following, the MOST likely diagnosis is:

Acute rheumatic feverInfective

endocarditis

Myocarditis

Paroxysmal

atrial tachycardia

PericarditisSlide100

A 14 year old boy complains of dull chest pain over the precordium. It began 4 days ago and occurs intermittently. It is not associated with activity, but it does increase when he is in a supine position and decreases when he is leaning forward. The frequency, duration, and the intensity of the pain has been increasing. Among the following, the MOST likely explanation for these findings is:

Acute rheumatic fever

Arrhythmia

Costochondritis

Myocardial

ischemia

PericarditisSlide101

GOOD LUCK!!!