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Peds > Peds CV uworld > Flashcards

Peds CV uworld Flashcards

1
Q 
Digeorge syndrome
Path
Clinical features
First step when suspected
F/u
A

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

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2
Q

Congenital heart disease
Low set ears
Duodenal atresia

Think…

A

Downs syndrome

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3
Q

Neural tube defects
cardiac anomaly
orofacial cleft

In a newborn

Think…

A

Folic acid antagonists like

Phenytoin
Methotrexate

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4
Q

Congenital heart disease
Thrombocytopenia

In a newborn

Think…

A

Congenital rubella syndrome

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5
Q 
Peds myocarditis
Path
Pres
Dx
Tx
Prog
A

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

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6
Q

Viral myocarditis more frequent and severe with higher mortality in This age group Because

A

Newborns

Because immature myocardium less adaptable to acute insult

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7
Q

Newborn viral myocarditis often misdiagnosed as …

Because…

A

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)

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8
Q

viral prodrome
Followed by respiratory distress
Holosystolic murmur
Hepatomegaly

Think…

A

Viral myocarditis

Any age but more freq and severe in newborns

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9
Q

Normal cardiothoracic ratio on cxr

A

v60% infants v1yo

v50% children ^2yo… and adults?

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10
Q

Peds viral myocarditis findings on

Cxr
Ekg
Echo
Labs

Gold standard for dx

A

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

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11
Q

Treatment of peds myocarditis

A

Supportive inotropes amd diuretics

Monitoring in icu for per risk of shock and fatal arrhythmias

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12
Q

How is strep throat different in kids v3yo

A

It is uncommon v3yo

Because fewer epithelial attachment sites in the young throat… per uworld

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13
Q

TF

Kids v3yo are higher risk for acute rheumatic fever

A

F
ARF is uncommon v3yo

Because strep throat (gas pharyngitis) is uncommon

Because fewer epithelial attachment sites in the young throat… per uworld

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14
Q

Typical first manifestation of acute rheumatic fever

A

Arthritis

2-4 wks after incompletely treated group a strep pharyngitis

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15
Q

Clinical diagnostic criteria of kawasaki’s

A 
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)

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16
Q

Age and ancestry of kawasaki

A

v5yo (90% of cases)

Asian ancestry

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17
Q

Describe the conjunctivitis of kawasaki

A

Bilateral

Non-exudative

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18
Q

Describe the mucositis of kawasakis

A

Injected or fissured lips or pharynx

Strawberry tongue

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19
Q

Describe the lymphadenopathy of kawasaki

A

1+ cervical lymph node ^1.5 cm

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20
Q

Define morbilliform

A

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.

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21
Q

Define perineum

A

Surface area between pubic symphisis and coccyx

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22
Q

Describe kawasaki rash

A

erythematous, polymorphous, generalized

Perineal erythema and desquamation

Morbilliform (measle-like… 2-10mm erythematous macules sometimes confluent)

Involves trunk and extremities

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23
Q

Treat kawasaki

A

Aspirin

Ivig

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24
Q

Complications of kawasaki

A

**Coronary artery aneurysm

MI and ischemia

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25
Q

Kawasaki disease aka

A

Mucocutaneous lymph node syndrome

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26
Q

How does a kawasaki kid appear, good or bad

A

Irritable

Miserable

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27
Q

TF

Kawasaki can occur late in childhood

A

T

But 90% of cases occur v5yo

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28
Q

The most serious complication of kawasaki

A

Coronary artery aneurysm

Can occur in 20% untreated
MI and death are possible consequences

So give aspirin and ivig

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29
Q

_____ should be performed in every pt suspected of kawasaki disease and repeated ______ to monitor for changes

A

Baseline echo
(for coronary artery aneurysm)

Repeat 6-8wks later to monitor for changes

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30
Q

When to give aspirin to kids

A

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)

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31
Q

Quick and dirty reye syndrome

A

Rare but life threatening hepatic encephalopathy when aspirin given for flu or varicella in kids

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32
Q

Oropharyngeal exam findings in strep throat

A

Pharyngeal erythema

Tonsilar exudates

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33
Q

Scarlett fever
Should be on your ddx for…
Is a complication of…
Classic exam finding is…

A

Ddx for fever and rash

Complication of untreated strep pharyngitis

Classic sand-paper texture rash that spares palms and soles

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34
Q

Classic signs and symptoms and tx for rocky mountain spotted fever

A

Headache
Gastrointestinal symptoms
Rash on palms and soles

Doxycycline 5-7 days

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35
Q

What lab studies or biopsies are needed to diagnose kawasaki disease

A

None, if classic presentation

It is a clinical diagnosis

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36
Q

Mildly accentuated peripheral pulses in an otherwise healthy 6-month-old and continuois flow murmur on hesrt auscultation think…

A

PDA (small if asymptomatic…)

Wide pulse pressure because left to right shunting aorts to pulmonary artery

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37
Q

Endocardial cushing defect
E.g. …
Commonly associated with…

A

asd or vsd

Commonly associated with downs syndrome

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38
Q

Cardiac abnormalities associated with williams syndrome

A

Supravalvular aortic stenosis
Pulmonic stenosis
Septal defects

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39
Q

Cardiovascular anomalies associsted with turner syndrome

A

Bicuspid aortic valve

Coarctation of the aorta

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40
Q

Turner syndrome features

A 
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
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41
Q

Turner syndrome karyotype

A

45X

complete or partial loss of an X chromosome

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42
Q

45X karyotype aka

CV workup necessary

because…

A

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
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43
Q

mitral valve prolapse in kids is more prevalent in what disorders

A

connective tissue disorders

marfan, ehlers danlos, osteogenesis imperfecta

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44
Q

patent ductus arteriosus symptoms

A

asymptomatic
or
exertional dyspnea, CHF

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45
Q

pda assoc w what peds syndromes

A

congenital rubella syndrome

char syndrome

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46
Q

tetrology of fallot assoc w what peds syndromes

A

downs syndrome

digeorge syndrome

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47
Q

VSDs assoc w what peds syndromes

A

aneuploidy

-trisomy 13 (Patau) 18 (Edwards) 21 (Down)

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48
Q

exam and xray findings for transposition of the great vessels

A 
single S2 (aorta anterior to pulmonary artery)
\+/- VSD murmur

“egg on a string” heart hanging from narrow mediastinum

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49
Q

exam and xray findings for tetrology of fallot

A

harsh pulmonic stenosis murmur
vsd murmur
single S2 (pulmonic valve closing is silent)

boot-shaped heart - enlarged right ventricle

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50
Q

exam and xray findings for tricuspid atresia

A

single S2
vsd murmur

minimal pulmonary blood flow

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51
Q

exam and xray findings for truncus arteriosus

A

single S2
systolic ejection murmur (high flow thru trunk valve)

increased pulmonary blood flow, edema

52
Q

exam and xray findings for total anomalous pulmonary venous return with obstruction

A

severe cyanosis
respiratory distress

pulmonary edema
“snowman sign” - enlarged supracardiac veins and SVC

53
Q

the most common congenital cyanotic heart disease in newborns

A

transposition of teh great vessels

54
Q

transposition of the great vessels
presentation
exam
xray

A

cyanosis within 24 hours of life
single S2 because aorta anterior to pulmonic valve
egg on a string (narrow mediastinum)

55
Q

how does transposition of great vessels survive

and how can you differentiate the mechanism by physical exam

A

mixing of deox and ox blood
PDA - murmur on exam
VSD - murmur on exam

PFO - no murmur on exam

56
Q

workup and treatment of transposition of great vessels

A

give prostaglandin E to kEEP the PDA open for mixing and survival

get cardiac echo

57
Q

TF

atrial septal defect is a cyanotic heart lesion

A

F

58
Q

TF

ventricular septal defect as is a cyanotic heart lesion

A

F

59
Q

coarctation of aorta… cyanotic?

A

maybe some cyanosis

but more often pale and mottled from shock

60
Q

most common cause of cyanotic heart disease in
neonatal period
vs
after the neonatal period

A

neonatal - transposition of great vessels

after-neonate - tetrology of fallot

61
Q

cyanotic 2mo with 2/6 systolic ejection maneuver

likely dx
physical exam maneuver to try to improve symoptoms
and how does it work

A

tetrology of fallot
knees to chest
kink femoral arteries, increase svr so more blood flow across stenosed pulmonary artery to get oxygenated

62
Q

what determines degree of symptoms in tetrology of fallot

A

degree of pulmonary artery stenosis

minimal to profround hypoxemia

63
Q

define “tet spell”

and explain why it happens

A

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

64
Q

what does crescendo-decrescendo systolic ejection murmur in tetrology of fallot represent

A

right ventricular outflow obstruction

65
Q

what does single S2 in tetrology of fallot represent

A

aortic valve closing

(pulmonary component of S2 inaudible… high resistance, low flow, minimal open/close

66
Q

treat tet spell

A

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

67
Q

Normal ekg in newborn is different from normal adult ekg how and why

A

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)

68
Q

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…

A

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
69
Q

risk factors for tricuspid valve atresia include

A 
any congenital heart disease risk factors
eg
congenital rubella syndrome
down syndrome
maternal diabetes
fh congenital heart disease
70
Q

treat tricuspid valve atresia

prognosis with and without this treatment

A

surgical repair

without, most die v1yo
with, 10y survival 80%

71
Q

complete atrioventricular canal defect

strong association with…
cxr findings… because…

A

strong assoc w down’s syndrome

cxr findings

  • increased pulmonary vasc markings (excess pulm flow)
  • cardiomegaly (biventricular volume overload)
72
Q

ebstein anomaly

define
association
ekg findings
cxr findings

A

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

73
Q

total anomalous pulmonary venous return

define
survival depends on
ECG findings
CXR findings

A

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

74
Q

truncus arteriosus

strong assoc w…
ECG findings…
CXR findings…

A

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

75
Q

TF

Left axis deviation can be normal in neonate

A

F
if anything, infant should have R axis deviation (pumping against high pulm resistance in utero) and R waves in precordial V1-V3

76
Q

in hypertrophic cardiomyopathy
physiologic and murmur effects of:

valsalva
abrupt standing
nitroglycerin
sustained hand grip
squatting
leg raise
A

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)

77
Q

hypertrophic cardiomyopathy

define

more common in…
inheritance pattern
symptoms

physical exam

A

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

78
Q

why dual carotid upstroke in hypertrophic cardiomyopathy

A

midsystolic left ventricular outflow obstruction during cardiac contraction

79
Q

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

A

will increase the murmurs in aortic regurge, mitral regurg, vsd
essentially anything that increases left ventricular size will…

80
Q 
pericardiotomy syndrome
define
path
pres
workup
tx
A

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

81
Q

Beck’s triad

A

distant/muffled heart sounds
distended jugular veins (or scalp veins in infant)
hypotension

for cardiac tamponade

82
Q

tf

congenital heart disease increases risk for bacterial endocarditis

A

T

83
Q

presentation of bacterial endocarditis

A

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)
84
Q

pulmonary embolism
presenting symptoms
CXR

A

tachycardia, tachypnea, hypoxia, right heart failure

normal cxr vs atelectasis, opacities, or pleural effusion

85
Q

name for
inflammation, reactive pericarditis, pericardial effusion, tamponade
days-months after cardiac surgery or injury

A

pericardiotomy syndrome

86
Q

4 major causes of sudden cardiac death

A

CAD
HOCM or other cardiomyopathy
Long QT or other arrhythmia
Congenital heart defect

87
Q

Most common cause of sudden cardiac death in adolescents

What % have not had symptoms before

A

HOCM

50% no prior symptoms

88
Q

Pathogenesis of sudden death from HOCM in teen

A

myocyte hypertrophy and fibrosis peaks during puberty and can precipitate a acute left ventricular outflow obstruction

can also precipitate fatal arrhythmia like V-fib

89
Q

Inheritance pattern of HOCM

A

Autosomal dominant

90
Q

Who to screen for HOCM

and how to screen

A

kid with family history of hocm or sudden death v50yo

Usually at preparticipation sports physical eg in adolescence with ECHO and ECG

91
Q

What dies HOCM look like on ECG

A

LVH - tall R wave in avL, deep S wave in v3

repolarization changes (inverted T wave) in anterolateral leads I avL v4-v6

92
Q

Adolescent positive for hocm on preparticipation sports physical by family history, echo, and ecg…

Next step…

A

Advise refrain from sports per risk of sudden cardiac death from outflow obstruction or arrhythmia eg v-tach

93
Q

tf

hypertensive, valvular, ischemic causes of cardiac hypertrophy are considered cardiomyopathy

A

F

94
Q

Autopsy of peds pt who died of sudden cardiac death yields no structural problem

what else to check for
how
why

A

Long QT syndrome
via genetic testing
because heritable

95
Q

Incidence of hocm vs long qt syndrome

Both on the differential in peds when…

A

hocm 1/500
long qt 1/2500

on ddx when sudden death in teenor family history of sudden desth v50yo

96
Q

commotio cordis

define

A

Fatal v-fib after chest trauma, most common during basketball, rare

97
Q

Typical presentstion of PE in peds

A

chest pain, dyspnea

in setting of prolongued inactivity, central venous catheter, or inherited hypercoagulable state

98
Q

aortic aneurysm in peds is rare without history of…

A

Marfan syndrome

99
Q

Common cause of aortic aneurysm in peds vs adults

A

Marfan peds
vs
Atherosclerosis adults

100
Q

dysmorphysms in trisomy 18 Edwards syndrome

prognosis

A 
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

101
Q 
microcephaly
micrognathia
prominent occiput
low set ears
heart defects
clenched hands with overlapping fingers
renal defects
limited hip abduction
rocker-bottom feet

think…

A

trisomy 18 Edwards syndrome

102
Q

most common congenital heart defect in trisomy 18 Edward syndrome

A

vsd

103
Q

trisomy 18 aka

A

Edward syndrome

104
Q

vsd on auscultation

A

holosystolic murmur

lower left sternal border

105
Q

tf

asd can present with a systolic ejection murmur at the left upper sternal border

A

T

due to increased blood flow across pulmonic valve

106
Q

congenital heart block
physical exam
common association and presentation of that

A

bradycardia on exam

assoc w neonatal lupus - typically erythematous annular rash on scalp and periorbital region

107
Q

newborn erythematous annular rash on scalp and periorbital region

bradycardia

think…

A

neonatal lupus with congenital heart block

common association

108
Q

PDA on auscultation

A

continuous flow murmur

heard best at left subclavicular region

109
Q

most common congenital heart defect in downs syndrome

pathophys

A

complete atrioventricular septal defect

failure of endocardial cushions to merge

110
Q

coarctation of the aorta

congenital disease association

A

turner syndrome

111
Q

ebstein anomaly

presentation, auscultory findings, and their related pathopys

A

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

112
Q

does tetrology of fallot typically occur sporadically without other anomalies or in association with another condition?

A

majority tof is sporadic

only 15% with e.g. Down syndrome or DiGeorge syndrome

113
Q

truncus arteriosus and transposition of the great arteries are strongly associated with…

A 
DiGeorge syndrome
(conotruncal defects?)
114
Q

neonatal cyanosis, heart failure, systolic ejection murmur with loud ejection click

think…

A

truncus arteriosus

look for other signs of DiGeorge syndrome…

115
Q

tachypnea
poor weight gain
sweating with feeds

suggests cardiac shunting in which direction
ddx

A

left to right shunting

(the body is not getting adequate perfusion)

asd, vsd, pda

116
Q

cyanosis

suggests cardiac shunting in which direction
ddx

A

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)
117
Q

pallor, shock, severe acidosis

suggests what cardiac issue

A

interrupted left ventricular output

the body is acutely not getting adequate perfusion

118
Q

first step in cyanotic newborn with clear lung fields

why

next step after that

A

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

119
Q

drug to close pda

A

indomethacin

120
Q

what does a trial of 100% oxygen tell you about a cyanotic newborn

A

not a lung disease

concern for congenital heart defect (R-L shunt)

121
Q

tf

intubate baby cyanotic from R-L shunting from congenital heart defect

A

F

additional pressure will not improve hypoxia, lungs are not the problem

122
Q

tf

anemia can cause cyanosis and low pulse-ox

A

F
does not affect cyanosis
does not affect pulse-ox (can still sat available Hb irrespective of how much Hb)

123
Q

pathogenesis of coarcted aorta

A

thickening of tunica media of aortic arch near ductus arteriosus

124
Q

why can you get low O2 sats in lower extremities compared to upper with coarcted aorta

A

if coarct is pre PDA then flow thru PDA is R-L and flow to lower extremities is deoxygenated coming from the pulmonary artery

125
Q

coarcted aorta

murmur and where to listen for it

A

systolic ejection murmur at left interscapular area

126
Q

in coarcted aorta, want PDA open or closed?

A

open

coarct usually pre-PDA, need to perfuse lower extremities somehow, even if with less oxygenated blood

Peds (12 decks)

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