Peds CV uworld Flashcards
Digeorge syndrome Path Clinical features First step when suspected F/u
CATCH22q11.2 in the pharyngeal pouch
22q11.2 deletion
Pharyngeal pouch defective development
Conotruncal cardiac defects (truncus arteriousus, TOF, septal defects...) Abnormal facies Thymic aplasia/hypoplasia Cleft palate Hypocalcemia (parathyroid hypoplasia)
Serum calcium levels and EKG
(Assess immediately for hypocalcemia)
Follow up t cell lymphopenia (viral fungal risk… bacterial too if t cell activation of b cells compromised)
-get routine vaccinations maybe excepting mmrv, intranasal flu, oral polio, rota, live attenuated depending on degree of immunodeficiency
Congenital heart disease
Low set ears
Duodenal atresia
Think…
Downs syndrome
Neural tube defects
cardiac anomaly
orofacial cleft
In a newborn
Think…
Folic acid antagonists like
Phenytoin
Methotrexate
Congenital heart disease
Thrombocytopenia
In a newborn
Think…
Congenital rubella syndrome
Peds myocarditis Path Pres Dx Tx Prog
Cocksackie b virus
Adenovirus
Other infections, toxins, autoimmune…
Direct myocite injury and autoimmune inflammation, myonecrosis, systolic and dystolic dysfunction
Viral prodrome
Heart failure (sob syncope tachyc vomiting)
hepatomegaly
Cxr cardiomegaly pulm edema
EKG sinus tach
Echo dec ej fraction, diffuse hypokinesia
**Endomyocardial bx gold standard - inflammatory infiltrate w myocite necrosis
75% newborn mortality
25% infant and chidren mortality
If survive 66% full recovery 2-3 mos
33% dilated cardiomyopathy / chronic heart failure
Viral myocarditis more frequent and severe with higher mortality in This age group Because
Newborns
Because immature myocardium less adaptable to acute insult
Newborn viral myocarditis often misdiagnosed as …
Because…
Asthma
Pneumonia
Because viral prodrome
Followed by respiratory distress
(L heart failure)
Think viral myocarditis if holosystolic murmur (dilated cardiomyopathy)
Hepatomegaly (congestion from now R heart failure)
viral prodrome
Followed by respiratory distress
Holosystolic murmur
Hepatomegaly
Think…
Viral myocarditis
Any age but more freq and severe in newborns
Normal cardiothoracic ratio on cxr
v60% infants v1yo
v50% children ^2yo… and adults?
Peds viral myocarditis findings on
Cxr
Ekg
Echo
Labs
Gold standard for dx
Cxr - cardiomegaly +- pulm edema depending on severity
Ekg - unhelpful sinus tach and
Non-specific t wave changes
Echo - global hypokinesia
Labs
viral studies
cradiac injury studies - ckmb, troponins
Inflammatory markers - cbc, esr, crp
Gold standard for dx - endomyocardial bx
Treatment of peds myocarditis
Supportive inotropes amd diuretics
Monitoring in icu for per risk of shock and fatal arrhythmias
How is strep throat different in kids v3yo
It is uncommon v3yo
Because fewer epithelial attachment sites in the young throat… per uworld
TF
Kids v3yo are higher risk for acute rheumatic fever
F
ARF is uncommon v3yo
Because strep throat (gas pharyngitis) is uncommon
Because fewer epithelial attachment sites in the young throat… per uworld
Typical first manifestation of acute rheumatic fever
Arthritis
2-4 wks after incompletely treated group a strep pharyngitis
Clinical diagnostic criteria of kawasaki’s
5 day fever Conjunctivitis bilat non-exudative Mucositis Cervical lymph node ^1.5cm Rash of any kind pretty much Swelling and/or erythema of palms and soles
(Need fever + 4/5 of the others to dx)
Age and ancestry of kawasaki
v5yo (90% of cases)
Asian ancestry
Describe the conjunctivitis of kawasaki
Bilateral
Non-exudative
Describe the mucositis of kawasakis
Injected or fissured lips or pharynx
Strawberry tongue
Describe the lymphadenopathy of kawasaki
1+ cervical lymph node ^1.5 cm
Define morbilliform
morbilliform refers to a rash that looks like measles - macular lesions that are red and usually 2–10 mm in diameter but may be confluent in places.
Define perineum
Surface area between pubic symphisis and coccyx
Describe kawasaki rash
erythematous, polymorphous, generalized
Perineal erythema and desquamation
Morbilliform (measle-like… 2-10mm erythematous macules sometimes confluent)
Involves trunk and extremities
Treat kawasaki
Aspirin
Ivig
Complications of kawasaki
**Coronary artery aneurysm
MI and ischemia
Kawasaki disease aka
Mucocutaneous lymph node syndrome
How does a kawasaki kid appear, good or bad
Irritable
Miserable
TF
Kawasaki can occur late in childhood
T
But 90% of cases occur v5yo
The most serious complication of kawasaki
Coronary artery aneurysm
Can occur in 20% untreated
MI and death are possible consequences
So give aspirin and ivig
_____ should be performed in every pt suspected of kawasaki disease and repeated ______ to monitor for changes
Baseline echo
(for coronary artery aneurysm)
Repeat 6-8wks later to monitor for changes
When to give aspirin to kids
For kawasaki disease
(Maybe a few others too…)
For antiplatelet and antiinflammatory effects
Usually avoid in kids for reye syndrome
(Rare but life threatening hepatic encephalopathy when using aspirin for flu or varicella in kids)
Quick and dirty reye syndrome
Rare but life threatening hepatic encephalopathy when aspirin given for flu or varicella in kids
Oropharyngeal exam findings in strep throat
Pharyngeal erythema
Tonsilar exudates
Scarlett fever
Should be on your ddx for…
Is a complication of…
Classic exam finding is…
Ddx for fever and rash
Complication of untreated strep pharyngitis
Classic sand-paper texture rash that spares palms and soles
Classic signs and symptoms and tx for rocky mountain spotted fever
Headache
Gastrointestinal symptoms
Rash on palms and soles
Doxycycline 5-7 days
What lab studies or biopsies are needed to diagnose kawasaki disease
None, if classic presentation
It is a clinical diagnosis
Mildly accentuated peripheral pulses in an otherwise healthy 6-month-old and continuois flow murmur on hesrt auscultation think…
PDA (small if asymptomatic…)
Wide pulse pressure because left to right shunting aorts to pulmonary artery
Endocardial cushing defect
E.g. …
Commonly associated with…
asd or vsd
Commonly associated with downs syndrome
Cardiac abnormalities associated with williams syndrome
Supravalvular aortic stenosis
Pulmonic stenosis
Septal defects
Cardiovascular anomalies associsted with turner syndrome
Bicuspid aortic valve
Coarctation of the aorta
Turner syndrome features
Short stature Narrow high-arched palate Low hair line Webbed neck Bicuspid aortic valve Coarctation of the aorta Broad chest spaced nipples Horseshoe kidney Streak ovaries amenorrhea infertility
Turner syndrome karyotype
45X
complete or partial loss of an X chromosome
45X karyotype aka
CV workup necessary
because…
Turner syndrome
4 extremity blood pressure
echocardiography
because prevalence of CV abnorms
- bicuspid aortic valve
- coarcted aorta
- aortic root dilation and risk of aortic dissection
mitral valve prolapse in kids is more prevalent in what disorders
connective tissue disorders
marfan, ehlers danlos, osteogenesis imperfecta
patent ductus arteriosus symptoms
asymptomatic
or
exertional dyspnea, CHF
pda assoc w what peds syndromes
congenital rubella syndrome
char syndrome
tetrology of fallot assoc w what peds syndromes
downs syndrome
digeorge syndrome
VSDs assoc w what peds syndromes
aneuploidy
-trisomy 13 (Patau) 18 (Edwards) 21 (Down)
exam and xray findings for transposition of the great vessels
single S2 (aorta anterior to pulmonary artery) \+/- VSD murmur
“egg on a string” heart hanging from narrow mediastinum
exam and xray findings for tetrology of fallot
harsh pulmonic stenosis murmur
vsd murmur
single S2 (pulmonic valve closing is silent)
boot-shaped heart - enlarged right ventricle
exam and xray findings for tricuspid atresia
single S2
vsd murmur
minimal pulmonary blood flow
exam and xray findings for truncus arteriosus
single S2
systolic ejection murmur (high flow thru trunk valve)
increased pulmonary blood flow, edema
exam and xray findings for total anomalous pulmonary venous return with obstruction
severe cyanosis
respiratory distress
pulmonary edema
“snowman sign” - enlarged supracardiac veins and SVC
the most common congenital cyanotic heart disease in newborns
transposition of teh great vessels
transposition of the great vessels
presentation
exam
xray
cyanosis within 24 hours of life
single S2 because aorta anterior to pulmonic valve
egg on a string (narrow mediastinum)
how does transposition of great vessels survive
and how can you differentiate the mechanism by physical exam
mixing of deox and ox blood
PDA - murmur on exam
VSD - murmur on exam
PFO - no murmur on exam
workup and treatment of transposition of great vessels
give prostaglandin E to kEEP the PDA open for mixing and survival
get cardiac echo
TF
atrial septal defect is a cyanotic heart lesion
F
TF
ventricular septal defect as is a cyanotic heart lesion
F
coarctation of aorta… cyanotic?
maybe some cyanosis
but more often pale and mottled from shock
most common cause of cyanotic heart disease in
neonatal period
vs
after the neonatal period
neonatal - transposition of great vessels
after-neonate - tetrology of fallot
cyanotic 2mo with 2/6 systolic ejection maneuver
likely dx
physical exam maneuver to try to improve symoptoms
and how does it work
tetrology of fallot
knees to chest
kink femoral arteries, increase svr so more blood flow across stenosed pulmonary artery to get oxygenated
what determines degree of symptoms in tetrology of fallot
degree of pulmonary artery stenosis
minimal to profround hypoxemia
define “tet spell”
and explain why it happens
increased cyanosis in tetrology of fallot baby when feeding, crying, or hyperventilating
maybe some increased o2 demand with exertion
but also “infundibular spasm” - increasing right ventricular outflow obstruction
what does crescendo-decrescendo systolic ejection murmur in tetrology of fallot represent
right ventricular outflow obstruction
what does single S2 in tetrology of fallot represent
aortic valve closing
(pulmonary component of S2 inaudible… high resistance, low flow, minimal open/close
treat tet spell
knees to chest
(kink femoral arteries, increase svr so more blood flow across stenosed pulmonary artery to get oxygenated)
inhaled O2
(stimulates pulm vasodilation, decreasing pulmonary vascular resistance)
maybe IVF
to improve R ventricular filling and pulmonary flow
Normal ekg in newborn is different from normal adult ekg how and why
physiologic R axis deviation
-large right ventricle because pumping against high pulmonary resistance as fetus and supplying majority of systemic blood via pda
physiologic R waves in precordial leads (V1-V3)
cyanotic infant with left axis deviation and small or absent R waves in precordial leads
suspect…
might also see on EKG…
might also see on cxr…
might hear on auscultation…
tricuspid atresia
(if anything, infant should have R axis deviation (pumping against high pulm resistance in utero) and R waves in precordial V1-V3)
- on EKG might also see peaked P waves because hypertrophic R atrium pumping against sealed tricuspid and through PFO
- on CXR might see decreased pulmonary vascular markings because low pulmonary flow
- on auscultation might also hear holosystolic murmur via VSD allowing survival
risk factors for tricuspid valve atresia include
any congenital heart disease risk factors eg congenital rubella syndrome down syndrome maternal diabetes fh congenital heart disease
treat tricuspid valve atresia
prognosis with and without this treatment
surgical repair
without, most die v1yo
with, 10y survival 80%
complete atrioventricular canal defect
strong association with…
cxr findings… because…
strong assoc w down’s syndrome
cxr findings
- increased pulmonary vasc markings (excess pulm flow)
- cardiomegaly (biventricular volume overload)
ebstein anomaly
define
association
ekg findings
cxr findings
atrialization of righ ventrical from displaced and malformed tricuspid valve causing severe tricuspid regurgitation and right atrial enlargement
assoc w maternal lithium use during pregnancy
tall P waves and r axis deviation
extreme cardiomegaly from heart failure
total anomalous pulmonary venous return
define
survival depends on
ECG findings
CXR findings
all 4 pulmonary veins drain into right atrium instead of left
need ASD VSD and/or PDA to survive
ECG R axis deviation and R ventricular hypertrophy that may be difficult to distinguish from normal neonate ecg
CXR increased pulmonary vascular markings from overcirculation
truncus arteriosus
strong assoc w…
ECG findings…
CXR findings…
strong assoc w DiGeorge syndrome
ECG can be normal in neonate
CXR cardiomegaly and increased pulmonary vascular markings from right heart failure and pulmonary overcirculation
TF
Left axis deviation can be normal in neonate
F
if anything, infant should have R axis deviation (pumping against high pulm resistance in utero) and R waves in precordial V1-V3
in hypertrophic cardiomyopathy
physiologic and murmur effects of:
valsalva abrupt standing nitroglycerin sustained hand grip squatting leg raise
valsalva - dec preload, inc murmur
abrupt standing - dec preload, inc murmur
nitroglycerin - dec preload, inc murmur
(ALL DECREASE LEFT VENTRICLE SIZE)
sustained hand grip - inc afterload, dec murmur
squatting - inc afterload and preload, dec murmur
leg raise - inc preload, dec murmur
(ALL INCREASE LEFT VENTRICLE SIZE)
hypertrophic cardiomyopathy
define
more common in…
inheritance pattern
symptoms
physical exam
asymmetrical left ventricular hypertrophy causing left ventricular outflow obstruction… with normal chamber size and without a clear etiology (e.g. not hypertension, aortic stenosis, infiltrative disease)
- more common in African Americans
- Autosomal Dominant inheritance
- asymptomatic vs dyspnea, chest pain, palpitations, syncope, sudden cardiac arrest
-carotid pulse with dual upstroke (midsystolic obstruction during cardiac contraction), systolic ejection murmur along left sternal border, strong apical impulse
why dual carotid upstroke in hypertrophic cardiomyopathy
midsystolic left ventricular outflow obstruction during cardiac contraction
how will increasing systemic venous return and/or systemic vascular resistance (e.g. leg raise, squatting, supine positioning) affect murmurs caused by:
aortic regurgitation
mitral regurgitation
ventricular septal defect
will increase the murmurs in aortic regurge, mitral regurg, vsd
essentially anything that increases left ventricular size will…
pericardiotomy syndrome define path pres workup tx
pleuropericardial disease days-months after cardiac surgery or injury
inflammation from surgical intervention, reactive pericarditis, pericardial effusion, tamponade
infant abdominal pain, vomiting, dec appetite
older kid pericardial friction rub, pleuritic chest pain worse w breathing or supine
-progress to tamponade with Beck’s triad (distant/muffled heart sounds, distended jugular veins vs scalp veins in infants, hypotension)
-compensatory tachycardia, tachypnea, dsypnea
-pulsus paradozus
CXR enlarged cardiac silhouette
pericardiocentiesis or pericardiectomy
Beck’s triad
distant/muffled heart sounds
distended jugular veins (or scalp veins in infant)
hypotension
for cardiac tamponade
tf
congenital heart disease increases risk for bacterial endocarditis
T
presentation of bacterial endocarditis
fever
new murmur
- petechiae
- splinter hemorrhages (tiny blood clots that tend to run vertically under the nails)
- Janeway lesions (non-tender, small erythematous or haemorrhagic macular or nodular lesions on the palms or soles only a few millimeters in diameter)
- Osler nodes (similar to Janeway lesions… erythematous or hemorrhagic nodular lesions on palms or soles but TENDER and origin IMMUNOLOGIC)
- Roth spots (hemorrhage with white center in retina)
pulmonary embolism
presenting symptoms
CXR
tachycardia, tachypnea, hypoxia, right heart failure
normal cxr vs atelectasis, opacities, or pleural effusion
name for
inflammation, reactive pericarditis, pericardial effusion, tamponade
days-months after cardiac surgery or injury
pericardiotomy syndrome
4 major causes of sudden cardiac death
CAD
HOCM or other cardiomyopathy
Long QT or other arrhythmia
Congenital heart defect
Most common cause of sudden cardiac death in adolescents
What % have not had symptoms before
HOCM
50% no prior symptoms
Pathogenesis of sudden death from HOCM in teen
myocyte hypertrophy and fibrosis peaks during puberty and can precipitate a acute left ventricular outflow obstruction
can also precipitate fatal arrhythmia like V-fib
Inheritance pattern of HOCM
Autosomal dominant
Who to screen for HOCM
and how to screen
kid with family history of hocm or sudden death v50yo
Usually at preparticipation sports physical eg in adolescence with ECHO and ECG
What dies HOCM look like on ECG
LVH - tall R wave in avL, deep S wave in v3
repolarization changes (inverted T wave) in anterolateral leads I avL v4-v6
Adolescent positive for hocm on preparticipation sports physical by family history, echo, and ecg…
Next step…
Advise refrain from sports per risk of sudden cardiac death from outflow obstruction or arrhythmia eg v-tach
tf
hypertensive, valvular, ischemic causes of cardiac hypertrophy are considered cardiomyopathy
F
Autopsy of peds pt who died of sudden cardiac death yields no structural problem
what else to check for
how
why
Long QT syndrome
via genetic testing
because heritable
Incidence of hocm vs long qt syndrome
Both on the differential in peds when…
hocm 1/500
long qt 1/2500
on ddx when sudden death in teenor family history of sudden desth v50yo
commotio cordis
define
Fatal v-fib after chest trauma, most common during basketball, rare
Typical presentstion of PE in peds
chest pain, dyspnea
in setting of prolongued inactivity, central venous catheter, or inherited hypercoagulable state
aortic aneurysm in peds is rare without history of…
Marfan syndrome
Common cause of aortic aneurysm in peds vs adults
Marfan peds
vs
Atherosclerosis adults
dysmorphysms in trisomy 18 Edwards syndrome
prognosis
microcephaly micrognathia prominent occiput low set ears heart defects (VSD most commonly) clenched hands with overlapping fingers renal defects limited hip abduction rocker-bottom feet
poor prognosis, death within 1 month, may survive with severe intellectual deficit if heart defect repaired
microcephaly micrognathia prominent occiput low set ears heart defects clenched hands with overlapping fingers renal defects limited hip abduction rocker-bottom feet
think…
trisomy 18 Edwards syndrome
most common congenital heart defect in trisomy 18 Edward syndrome
vsd
trisomy 18 aka
Edward syndrome
vsd on auscultation
holosystolic murmur
lower left sternal border
tf
asd can present with a systolic ejection murmur at the left upper sternal border
T
due to increased blood flow across pulmonic valve
congenital heart block
physical exam
common association and presentation of that
bradycardia on exam
assoc w neonatal lupus - typically erythematous annular rash on scalp and periorbital region
newborn erythematous annular rash on scalp and periorbital region
bradycardia
think…
neonatal lupus with congenital heart block
common association
PDA on auscultation
continuous flow murmur
heard best at left subclavicular region
most common congenital heart defect in downs syndrome
pathophys
complete atrioventricular septal defect
failure of endocardial cushions to merge
coarctation of the aorta
congenital disease association
turner syndrome
ebstein anomaly
presentation, auscultory findings, and their related pathopys
cyanosis and heart failure due to severe tricuspid regurgitation
holosystolic or early systolic murmur at lower left sternal border from tricuspid regurgitation
triple or quadruple gallop
-widely split S1 and S2 sounds… from pulmonic shutting extra extra quick?…. plus loud S3 from heart failure or S4 from hypertrophy
does tetrology of fallot typically occur sporadically without other anomalies or in association with another condition?
majority tof is sporadic
only 15% with e.g. Down syndrome or DiGeorge syndrome
truncus arteriosus and transposition of the great arteries are strongly associated with…
DiGeorge syndrome (conotruncal defects?)
neonatal cyanosis, heart failure, systolic ejection murmur with loud ejection click
think…
truncus arteriosus
look for other signs of DiGeorge syndrome…
tachypnea
poor weight gain
sweating with feeds
suggests cardiac shunting in which direction
ddx
left to right shunting
(the body is not getting adequate perfusion)
asd, vsd, pda
cyanosis
suggests cardiac shunting in which direction
ddx
right to left shunting
(blood is not being oxygenated shunted R-L)
transposition of great vessels
tetrology of fallot
tricuspid atresia
anomalous pulmonary venous return (severe L-R?) truncus arteriosus (severe L-R?.. causes pulm htn.. R-L)
pallor, shock, severe acidosis
suggests what cardiac issue
interrupted left ventricular output
the body is acutely not getting adequate perfusion
first step in cyanotic newborn with clear lung fields
why
next step after that
prostaglandin E1
to kEEp the PDA open
because cyanotic newborn suggests R-L shunt and PDA keeps at least some compensatory L-R pulmonary flow to get blood oxygenated and sustain life
then get echo to determine exact nature of malformation
drug to close pda
indomethacin
what does a trial of 100% oxygen tell you about a cyanotic newborn
not a lung disease
concern for congenital heart defect (R-L shunt)
tf
intubate baby cyanotic from R-L shunting from congenital heart defect
F
additional pressure will not improve hypoxia, lungs are not the problem
tf
anemia can cause cyanosis and low pulse-ox
F
does not affect cyanosis
does not affect pulse-ox (can still sat available Hb irrespective of how much Hb)
pathogenesis of coarcted aorta
thickening of tunica media of aortic arch near ductus arteriosus
why can you get low O2 sats in lower extremities compared to upper with coarcted aorta
if coarct is pre PDA then flow thru PDA is R-L and flow to lower extremities is deoxygenated coming from the pulmonary artery
coarcted aorta
murmur and where to listen for it
systolic ejection murmur at left interscapular area
in coarcted aorta, want PDA open or closed?
open
coarct usually pre-PDA, need to perfuse lower extremities somehow, even if with less oxygenated blood
