UWorld Peds

Question 1

tetralogy of fallot murmur–describe, rxn to squatting

Answer 1

harsh, systolic crescendo-decrescendo ejection murmur, single S2; squatting increases intensity of murmur but improves cyanosis

Question 2

hypertrophic cardiomyopathy murmur–describe, rxn to squatting

Answer 2

systolic murmur best heard at apex and left lower sternal border; squatting decreases intensity of murmur

Question 3

isolated ASD murmur–describe

Answer 3

systolic ejection murmur, wide-splitting of S2

Question 4

VSD murmur–describe, rxn to squatting

Answer 4

holosystolic murmur at left sternal border; squatting increases intensity of murmur

Question 5

Kawasaki disease symptoms (6)

Answer 5

fever, bilateral nonexudative conjunctivitis, mucositis (injected/fissured lips, injected pharynx, strawberry tongue), cervical lymphadenopathy, rash, edema and erythema of palms and soles

Question 6

Kawasaki disease tx

Answer 6

aspirin + IVIG to prevent cardiac complications

Question 7

Kawasaki disease complications (2)

Answer 7

coronary artery aneurysms, myocardial infarction and ischemia

Question 8

what is reye syndrome?

Answer 8

hepatic encephalopathy that can develop in children using aspirin during influenza or varicella infections

Question 9

rash w/”sandpaper” texture that spares palms and soles–dx and tx

Answer 9

scarlet fever (complication of untreated strep pharyngitis); amoxicillin

Question 10

what study should be done for suspected Kawasaki disease to allow for monitoring throughout disease?

Answer 10

echo

Question 11

rocky mountain spotted fever sx and tx

Answer 11

rash, fever, headache, GI sx after tick bite; doxycycline

Question 12

endocardial cushion defect (i.e., atrioventricular septal defect) is associated w/ what condition?

Answer 12

down syndrome

Question 13

midsystolic click w/late systolic murmur

Answer 13

mitral valve prolapse

Question 14

systolic ejection murmur, wide-splitting of S2

Answer 14

ASD

Question 15

cardiac abnormalities associated w/williams syndrome (deletions in chromosome 7)

Answer 15

supravalvular aortic stenosis, pulmonary stenosis, septal defects

Question 16

list 3 congenital heart defects associated w/down syndrome in decreasing order of prevalence

Answer 16

complete AV septal defect, VSD, ASD

Question 17

GI complications associated with down syndrome

Answer 17

duodenal atresia, hirschprung disease

Question 18

endocrine complications associated with down syndrome

Answer 18

hypothyroidism, T1DM

Question 19

sx of infant heart failure

Answer 19

diaphoresis, dyspnea w/feeds, poor appetite, lethargy, hyperactive precordium

Question 20

complete atrioventricular septal defect tx before surgery

Answer 20

pharmacotherapy (diuretics, inotropic agents), nutritional supplementation to optimize cardiac fxn

Question 21

what congenital infection is associated w/PDA?

Answer 21

rubella

Question 22

what congenital heart defects are associated w/digeorge syndrome?

Answer 22

conotruncal abnormalities (truncus arteriosus, ToF, interrupted aortic arch)

Question 23

define cyanotic vs pallid breath-holding spells

Answer 23

cyanotic: crying followed by breath-holding in forced expiration, limpness, LOC, rapid return to baseline
pallid: minor trauma followed by breath-holding, pallor, diaphoresis, lasts

Question 24

work-up after dx of breath-holding spells in healthy infant

Answer 24

CBC, serum ferritin (IDA is often associated)

Question 25

handgrip decreases intensity of what 3 murmurs?

Answer 25

HCM, aortic stenosis, mitral regurg

Question 26

postpericardiotomy syndrome--sx in infants vs older children

Answer 26

infants: vomiting, decreased appetite | older children: pericardial friction rub, pleuritic CP exacerbated by inspiration or lying supine

Question 27

complication of postpercardiotomy syndrome

Answer 27

tamponade

Question 28

beck's triad of tamponade

Answer 28

distant heart sounds, JVD (or distention of scalp veins in infants), hypotension

Question 29

blunting of costophrenic angles on CXR

Answer 29

pleural effusions

Question 30

CXR in pulmonary embolism

Answer 30

normal

Question 31

micrognathia, microcephaly, rocker bottom feet, overlapping fingers, absent palmar creases

Answer 31

edwards syndrome (trisomy 18)

Question 32

most common congenital heart defect in edwards syndrome (trisomy 18)

Answer 32

VSD

Question 33

supravalvular aortic stenosis associated w/ what syndrome?

Answer 33

williams syndrome (deletions in chromosome 7)

Question 34

congenital heart block is associated w/ what maternal condition?

Answer 34

SLE

Question 35

coronary artery aneurysms

Answer 35

Kawasaki disease

Question 36

URI followed by worsening respiratory distress, syncope, N/V, hepatomegaly, holosystolic murmur, cardiomegaly/pulmonary edema on CXR in a child

Answer 36

pediatric viral myocarditis

Question 37

most common viruses causing pediatric viral myocarditis

Answer 37

adenovirus, coxsackie B virus

Question 38

appearance of endomyocardial biopsy for pediatric viral myocarditis

Answer 38

inflammatory infiltrate of myocardium w/myocyte necrosis

Question 39

pediatric viral myocarditis tx

Answer 39

diuretics and inotropes in ICU due to risk of shock and fatal arrhythmias

Question 40

acute rheumatic fever is less likely in children younger than what age? why?

Answer 40

3; they have fever epithelial cell attachment sites in throat

Question 41

"egg on a string" on CXR

Answer 41

transposition of great vessels

Question 42

cyanosis within first 24hrs of life

Answer 42

transposition of great vessels

Question 43

"snowman sign" on CXR

Answer 43

total anomalous pulmonary venous return w/obstruction

Question 44

"boot-shaped heart" on CXR

Answer 44

ToF

Question 45

rib-notching on CXR

Answer 45

coarctation of aorta

Question 46

murmur of coarctation of aorta--describe

Answer 46

mild, continuous murmur heard all over chest (collaterals between hypertensive and hypoperfused vessels), systolic ejection murmur at left paravertebral interscapular area

Question 47

CATCH mnemonic--what disease, what it stands for

Answer 47

digeorge syndrome; conotruncal cardiac defects, abnormal facies, thymic aplasia, cleft palate, hypocalcemia

Question 48

____penia is a feature of digeorge syndrome

Answer 48

lymphopenia (NOT neutropenia)

Question 49

list 2 congenital heart defects associated w/turner syndrome in decreasing order of prevalence

Answer 49

bicuspid aortic valve, coarctation of aorta

Question 50

mitral valve prolapse is more prevalent in pts w/__________

Answer 50

connective tissue disorders (i.e., ehlers-danlos, marfan, osteogenesis imperfect)

Question 51

characteristics of benign murmurs

Answer 51

no significant family hx, early or mid-systolic, grade I/II that decreases on standing and valsalva

Question 52

upper limits for QT intervals for males vs females

Answer 52

males:

Question 53

family hx of long QT, congenital sensorineural deafness

Answer 53

jervell and lange-neilsen syndrome

Question 54

jervell and lange-neilsen syndrome--inheritance, pathophysiology

Answer 54

autosomal recessive congenital long QT syndrome caused by molecular defects in K+ channels; associated w/congenital sensorineural deafness

Question 55

long QT syndrome tx for asymptomatic vs symptomatic (or hx of syncope)

Answer 55

asymptomatic: beta-blocker (except sotalol) | symptomatic or hx of syncope: beta blocker + long-term pacemaker

Question 56

pharmacotherapy for HCM

Answer 56

beta blockers or calcium channel blockers

Question 57

episodes of cyanosis in a child during feeding or crying

Answer 57

"tet" spells of ToF

Question 58

tx for tet spells

Answer 58

knee-chest positioning (increases SVR), inhaled O2

Question 59

central cyanosis (mucous membranes cyanotic) and heart failure in first few weeks of life, no murmurs

Answer 59

hypoplastic left heart syndrome

Question 60

ddx of new-onset HTN in children

Answer 60

fibromuscular dysplasia, autosomal polycystic kidney disease, wilms tumor

Question 61

systolic ejection murmur at left paravertebral interscapular area

Answer 61

coarctation of aorta

Question 62

tachypnea, poor feeding, lethargy, prolonged capillary refill, metabolic acidosis, and decreased urine output on 3rd day of life

Answer 62

coarctation of aorta (due to closure of ductus arteriosus)

Question 63

palpable abdominal mass, hepatomegaly, and blue nodules in an infant

Answer 63

neuroblastoma

Question 64

new-onset HTN, bilateral palpable abdominal masses in an infant

Answer 64

autosomal polycystic kidney disease (dilations of renal collecting ducts)

Question 65

new-onset HTN, unilateral palpable abdominal mass, microhematuria, fever in an infant

Answer 65

wilms tumor

Question 66

wilms tumor pathophysiology

Answer 66

proliferation of metanephric blastema

Question 67

___ axis deviation is never normal on neonatal ECG

Answer 67

left

Question 68

left axis deviation on ECG and decreased pulmonary markings

Answer 68

tricuspid valve atresia

Question 69

infant

Answer 69

vascular ring

Question 70

most common cause of stridor in infants/children between 6mo-6yrs

Answer 70

croup

Question 71

laryngomalacia causes ____ that improves in ____ position and worsens in ____ position; most commonly seen in age ____

Answer 71

laryngomalacia causes stridor that improves in prone position and worsens in supine position; most commonly seen in age 4-8mo

Question 72

stridor in infants/children that improves w/corticosteroids and racemic epi

Answer 72

croup

Question 73

PDA-dependent congenital heart diseases

Answer 73

coarctation of aorta, transposition of great arteries, hypoplastic left heart syndrome, TAPVR, tricuspid atresia