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
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General Pediatric Board Review:Pediatric Cardiology
Daniela Rafii, M.D.Associate Director, Pediatric CardiologyMaimonides Infants and Children’s Hospital of BrooklynSlide2Slide3Slide4Slide5
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 ASA5% 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!!!