What is Eisenmenger’s syndrome?
complication of longstanding/uncorrected congenital heart anomalies that produce L→R shunting
What is the tx for Eisenmenger’s syndrome?
supportive, heart/lung transplant, endocarditis prophylaxis
What are sx of PPHN?
tachypnea, resp. distress, cyanosis, poor cardiac funciton/perfusion, hypotension, shock, loud single S2
What is the GOLD standard for dx Pulm HTN?
cardiac cath
What is the tx for Pulm HTN?
no cure; can use medications to improve sx and life expectancy
*Warfarin, Digoxin, Vasodilators
What heart defects are associated w/ Downs Syndrome?
AV canal/septal defects, ASD, VSD
What heart defects are associated w/ Marfan Syndrome?
aortic root dilation/dissection, MVP
What heart defect is associated w/ maternal Lithium ingestion?
Ebstein’s Anomaly of the Tricuspid Valve
What is Marfan Syndrome caused by?
mutation in FBN1 gene → effects fxn of fibrillin-1 in connective tissues
Which heart murmurs are innocent?
Still’s, Pulm ejection, Pulm flow, Venous hum, Carotid bruit
What is a key characteristic of Still’s murmur?
systolic, vibratory or musical nature; usually 3-6 yo
What are some characteristics of a Pulm Ejection murmur?
low grade, blowing, dec w/ inspiration, sitting up, standing up; 8-14 yo
What is a Venous Hum caused by?
turbulence in the SVC
What are the characteristics of a Venous Hum murmur?
loudest @ infra-clavicular area (especially w/ sitting/standing), disappears while lying supine, intensity changes w/ rotation of head, disappears w/ jugular compression; 3-6 yo
What are the 8 S’s of innocent murmurs?
Soft, Systolic, Short, Sounds (S1/2), Symptomless, Special tests normal, Standing/sitting variation, Sternal depression
What acute tx can be used to stop a tachyarrhythmia?
Vagal (ice to face, valsalva, gag reflex), IV adenosine or verapamil, DC cardioversion
What causes vasovagal syncope?
venous pooling → dec venous return → hypotension/bradycardia
What are the 2 factors that help infective endocarditis develop?
bacteremia & structural abnormality of heart/great vessels w/ significant flow turbulence or pressure gradient (VSD, TOF, IHSS (HOCM))
What is the major Duke criteria for dx infective endocarditis?
1-blood cultures (+)
2-evidence of endocardial involvement
What is the leading cause of acquired heart disease in children?
Kawasaki disease
What is one of the MC vasculitis in childhood?
Kawasaki disease
What age is most likely to get Kawasaki disease?
6 mo - 5 yo
*more frequent in those of Asian ancestry
T/F: Kawasaki is a contagious disease
False
What is the etiology of Kawasaki?
unknown, thought to be an immune rxn
Why do you need to act fast if Kawasaki disease is suspected?
affects the heart muscles and valves early
What are the sx of Kawasaki disease?
persistent high fever (> 5 days) + 2 or more of the following:
strawberry tongue, cherry red lips
changes in hands and feet
diffuse rash (worse in diaper area)
bloodshot eyes w/o d/c
swollen glands
*may not all be present at the same time
Pneumonic for remembering Kawasaki sx
FEARS ME
Fever x 5 days, Eyes -conjunctivitis, Arthralgia, Rash, Skin desquamation (hands/feet), Mucosal involvement (strawberry tongue, dry lips), Elevated ESR/Plts
What are the cardiac manifestations of Kawasaki disease?
coronary artery aneurysms, HF, depressed contractility, MI, arrhythmias, peripheral arterial occlusion
How are coronary artery aneurysms dx?
Echo & angiography
When are coronary artery aneurysms of concern?
*> 50% resolve w/in 2 years
giant aneurysms >8mm are more likely to cause MI
What are signs that the Kawasaki infxn is affecting the heart?
tachycardia out of proportion to fever, gallop heart sounds, muffled heart sounds if effusion, risk of coronary artery aneurysms (highest risk in infants)
What lab values are suggestive of Kawasaki?
elevated WBC, inc plts, inc acute-phase reactants (CRP, ESR, fibrinogen), anemia, pyuria
What is the Kobayashi score used for?
predict pts w/ KD at high risk of failing to respond to primary IVIG tx
What is the tx for Kawasaki?
IVIG
most effective in 1st 7-10 days onset
5x reduction in CA vs untx
high risk of resistance → add glucocorticoids
Aspirin
30-50g/kg daily divided into 4 doses
continue until plt, ESR, CRP are normal
What are the most common causes of Myocarditis in children?
Coxsackie B, echovirus
How do you prevent RHD?
proper tx of strep pharyngitis w/ abx
What infxns inc risk of CHD?
TORCHES CLAP
toxoplasmosis, other (zika), rubella, CMV, Herpes & Hepatitis, Entero virus, Syphilis, Chicken pox, Lyme disease, AIDs, Parvovirus B19
What CHDs are acyanotic?
CoA, AS, PDA, VSD, ASD, A-V canal defect, PS
What does Lithium use during pregnancy cause?
Epstein anomaly
What heart defect does Fetal Alcohol Syndrome cause?
ASD or VSD
What would a CXR show in pts w/ a VSD?
cardiomegaly, LAE, inc pulm vasculature
What CHD is common in Down’s syndrome pts?
ASD
What murmur is present w/ ASD?
soft systolic ejection @ ULSB; wide (fixed) split S2
What murmur is associated with a PDA?
pansystolic, machinery-like w/ bounding peripheral pulses & wide PP
What is the tx for a PDA?
Indomethacin or Ibuprofen OR surgery
What is the triad of sx associated w/ AS?
SAD: syncope, angina, dyspnea
How does CoA present in infants?
CHF or shock
How does CoA present in older children?
HTN
What are the MC clinically significant defects associated w/ CoA?
PDA, VSD, AS
What PE findings are associated with CoA?
SEM @ ULSB; bounding radial pulses, diminished femoral pulses, BP elevated in UE & lower in LE, cardiogenic shock
What is the tx for CoA?
surgery/cath = definitive
severe: prostaglandins to temp. reverse PDA closure
What would you see on a CXR of CoA?
figure 3
What are cyanotic CHD?
ToF, TGA, HLH, tricuspid atresia, truncus arteriosus, total anomalous pulm venous connection
What are the 4 components of ToF?
PROV: PS, RVH, Overriding aorta, VSD
What are sx of ToF?
“tet spells” in infants (hypoxic episodes, thromboembolism, squatting, polycythemia
What would you see on a CXR of ToF?
normal size heart; boot-shaped heart; dec pulm vasculature
What do see on a CXR of TGA?
cardiomegaly “egg-on-string”; inc pulm vasculature; narrow mediastinum
What would you see on a CXR of TAPVC?
cardiomegaly, “snowman” appearance, inc pulm vasculature
What is a Temper tantrum?
Child cries and/or harms him/herself to manipulate the parents/guardians
When do temper tantrums usually resolve?
By age 5
How should you handle tantrums?
redirect child to something else, avoid provoking situation, ignoring the tantrum
What are breath holding spells?
Child holds their breath and suddenly loses consciousness
Are cyanotic or pallid breath holding spells more common and is related to a temper tantrum?
Cyanotic
What is ADHD?
Chronic pattern of attention difficulties, impulsivity, and/or hyperactivity that interferes with daily life
What is the diagnostic criteria for ADHD?
6+ sx x min 6 months (inattention & hyperactivity), inconsistent w/ developmental level, have negative impacts on social/academic activities, present in 2+ settings
What is the gold standard questionnaire for diagnosis of ADHD?
*> 90% sensitivity & specificity
Conner's scale
What questionnaire is designed for Teacher/Parents and commonly used to dx ADHD?
*high sensitivity & specificity if both used
Vanderbilt ADHD Diagnostic rating scale
Which questionnaire is good for monitoring the effectiveness of medications in children with ADHD?
Conner's scale
What stimulant is commonly used for ADHD?
Methylphenidate (Ritalin)
Which ADHD medications are stimulants?
Ritalin, Concerta, Adderall, Vyvanse
What is a common side effect of Ritalin?
reduced growth, wt loss, poor weight gain
Which stimulant is less addictive than Adderall and Concerta?
Vyvanse
Which ADHD medications have less appetite suppression?
Non-stimulants: Stattera and Intuniv
What is Oppositional defiant disorder?
Pattern of negative, hostile, and defiant behavior that is excessive compared with other children of the same age for at least 6 months
What is the ODD-DSM criteria?
A: pattern of negativistic, hostile, and defiant behavior x 6 months
At least 4 sx: loses temper, argues w/ adults, actively defies requests/rules, deliberately annoys ppl, blames mistakes on others, easily annoyed, angry/resentful, spiteful/vindictive
B: disturbance causes impairment in social, academic, occupation
C: do NOT occur during the course of Psychotic/Mood disorder
D: criteria for Conduct disorder (18+ Antisocial personality disorder) are not met
What is Conduct disorder?
Repetitive/persistent pattern of behavior in which the basic rights of others or major age-appropriate societal norms and rules are violated
What is the DSM criteria for Conduct disorder?
A: 3 criteria w/in 12 months, at least 1 w/in the past 6 months
Categories:
Aggression to people & animals
Destruction of property
Deceitfulness or theft
Serious violations of rules
B: behavior impairs social, academic, occupational fxning
C: if 18+, criteria for Antisocial Personality Disorder are not met
ODD or CD:
Defiance of rules and argumentative verbal interactions
ODD
ODD or CD:
Symptoms emerge during pre-school years and persist for at least 6 months
ODD
ODD or CD:
Deliberate aggression, destruction, deceit, and serious rule violations, such as staying out all night or chronic school truancy
CD
ODD or CD:
Clear pattern of behavior established over a 6 month period
CD
What are the signs of Depression that you need to watch for?
SIGECAPS
Sleep changes, Interest loss, Guilt, Energy low, Concentration low, Appetite change (inc/dec), Psychomotor fxn (agitation/hopeless), Suicide
Which parasomnia:
Child awakens screaming from stage N3 sleep with dilated pupils, sweating, & tachycardia (usually 18 mo - 6 yo)
Night terrors
Does the child remember a night terror?
No
What conditions may precipitate night terrors?
Stress, Illness, Sleep deprivation
Night terrors or Nightmares:
Stage 4 sleep, No recall, First 1/3 of night, Not consolable, confused/agitated
Night terrors
Night terrors or Nightmares:
REM sleep, May recall, Consolable, Second 1/2 of night, upset but awake
Nightmares
What factors can exacerbate restless leg syndrome?
Caffeine, Alcohol, Nicotine, Iron deficiency, Pregnancy
Normal VS for a newborn
Temp: 97.7-99.5 F
RR: 35-60 bpm
HR: 120-160 bpm (lower in sleep)
Pulse Ox: 98%
BP: not obtained usually
How does weight change immediately following birth?
lose up to 10% of birthweight and gain it back by week 3 of life
What is Caput Succedaneum?
superficial edema/ecchymosis that crosses the suture line; reconfigure in ~1wk
What may cause a fontanelle to bulge?
inc ICP
What may cause a fontanelle to be sunken?
dehydration
What does an absent red light reflex indicate?
retinoblastoma, cataract, opacified cornea
What is CHARGE syndrome?
Coloboma, Heart anomalies, Atresia of choanae, Retardation of growth, GU abnormalities, Ear anomalies
What are key signs of Turner’s syndrome?
wideset nipples, excessive nuchal skin, lymphedema
What might weak LE pulses indicate?
coarctation of aorta/L ventricular tract obstruction
What does Scaphoid abdomen indicate?
diaphragmatic hernia
What are RF for developmental hip dysplasia?
breech position in utero, FMH, Females
What is the Barlow maneuver?
*used for hip dysplasia
infant lying supine w/ knees flexed → grab thigh along great trochanter w/ thumb on inner thigh → ADDUCT and apply downward pressure to promote dislocation