Step 2 Peds

• Spiral Fxrs
• bucket fxrs
• post. rib fxrs

• phenytoin
• alcohol
• lithium
• thalidomide

• ASD
• VSD
• PDA

• Rt-->Lft shunt
• "12345"

• Truncus arterioss
• Transposition of great vessels
• Tricuspid atresia
• Tetralogy of fallot
• Total Anomalous Pulmonay Venus Retun

VSD

• Apert's Syndrome
• cranial deformity
• fusion of fingers/toes

• Down's
• Fetal Alcohol Syndrome
• TORCHES
• Cri du Chat
• Trisomies 13, 18

• harsh holosystolic murmer
• L sternal border

Dx with Echo

• CXR: poss LVH w/ small defects
• LVH+RVH w/ larger defects
• ^pulm vascular markings

small defects close spontaneously

• Surgery if:
• pts fail medical management
• pts <1yo + pulm HTN
• older kids w/ large VSD that hasnt shrunk

• Holt-Oram Syndrome
• absent radii
• ASD
• 1st deg heart block

• FAS
• Downs

• 1: si/sx in early childhood
• 2: si/sx in late chldhood, or early adulthood

2 is most common

• RT ventricular heave
• Systolic Ejection murmur
• Upper Left Sternal border
• **Wide and Fixed, Split S₂**

may also have mid-diastolic rumble at left lower sternal border

• Echo: shows blood flow btwn atria
• EKG: R axis deviation and RVH, PR prolongation is common
• CXR: cardiomegaly ^ pulm vascular markings

most are small and resolve spontaneously

• Surgery:
• infants w/ CHF
• >2:1 pulmonary to systemic blood flow

• L-to-R shunt --> Pulm HTN
• Pulm HTN--> shunt reversal

• 1st trimester Rubella infxn
• prematurity
• female

• Usually no symptoms
• Large defects:
• FTT
• recurrent lower RI's
• Lower extremity clubbing
• CHF

• Wide Pulse Pressure
• continuous "machinary murmur"
• 2nd L IC space at sternal border
• loud S2
• bounding Periph pulses

TOC: Color flow doppler

• EKG: poss LVH
• CXR: poss cardiomegaly w/ large lesions

• Tx: Indomethacin
• CI: cyanotic heard dz

• Tricuspid Valve displacement into Right Vent
• **associated with mom taking LITHIUM**

child >6-8 yo and indomethacin fails

• Bicuspid aortic valve (2/3 of patients)
• Turner's
• Berry Aneurysms
• males

• SPB upper extremities > lower
• Different PB in L and R arms
• **Differential Cyanosis
• Weak femoral pulses
• Childhood HTN
• LE claudication
• syncope
• epistaxis
• HA

• short systolic murmur
• in left axilla
• forceful apical impulse

• Dx with Echo and color flow doppler
• CXR: cardiomegaly and pulm congestion
• ECG: "3" sign d/t pre/post ductal dilation, rib notching

• Severe coarctation needs PDA kept open with...
• PGE₁
• Surgery or alloon angioplasty
• Monitor for:
• restenosis
• aneurysm
• aortic dissection

Transposition GV (per 1st Aid)

• Tachypnea
• progressive hypoxemia
• extreme cyanosis
• Poss signs of CHF
• Single loud S2

• Dx: Echo
• CXR: narrow base heart, no main pulm artery segment
• "egg-shaped heart"
• ^ pulm vascular markings

• IV PGE₁ to keep PDA open
• Balloon atrial septostomy to creat/enlarge ASD if PGE₁ fails

• "PROV"
• Pulmonary stenosis
• RVH (R vent. outflow obstruction)
• Overriding arota
• VSE

**MC cyanotic congenital heard dz in children**

• no cyanosis at birth, develops over 1st 2 yrs
• degree of cyanosis reflects extent of pulm stenosis

• Infants often no symptoms 'til 4-6 mos
• 4-6 mos when CHF poss and manifests as sweating w/ fedding or tachypnea
• Hypoxemia may -->FTT
• "Tet spells"

• Murmur: syst. ejection @ L upper sternal border
• R vent heave, single S2

• CXR: boot shaped heart, v pulm vasc markings
• EKG: R-axis dev and RVH

• if severe pulm stenosis: immediate PGE1
• tet spells:
• Morphine
• O2
• Phenylephrine
• propanolol
• squatting
• "tx tet spills w/ MOPPS"

MR, flat facial profile, Prominent epicanthal folds, simian crease

• Assoc: ^maternal age, duodenal atresia, Hirschsprung's,
• endocardial cushion (ASD, VSD, Mitral/Triscuspid valve abnormalities)

Malignancy: ALL

Other: hypothyroidism, early Alzheimer's

• Trisomy 18
• Rocker-bottom ears