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1
Q

what leads to closure of ductus arteriosus after birth

A

decreased levels of prostaglandin E2

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2
Q

what are the 2 right to left shunts

A

foramen ovale and ductus arteriosus

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3
Q

what is the left to right shunt

A

ductus venosis

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4
Q

what does the foramen ovale connect

A

right atrium to left atrium

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5
Q

what does the ductus arteriosus connect

A

pulmonary artery to aorta

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6
Q

what does the ductus arteriosus connect

A

umbilical vein to IVC (bypass liver)

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7
Q

what are the types of congential heart disease

A

acyanotic, obstructive lesions, cyanotic

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8
Q

what are most common defects

A

bicupsid aortiv calce, VSD, ASD (PFO), tetralgy of fallot

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9
Q

what are some risk factors for congenital heart dz

A

premature, fx, genetic syndrmoes, maternal factors (diabetes, HTN,PKU), meds during prego, smoking, alc, infection in utero

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10
Q

how to detect heart dz prenatal

A

US

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11
Q

how to detect heart dz postnatal

A

during birth often present with serious life threatening findings

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12
Q

when do you refer

A

if positive pulse ox screen, genetic disorder ass iwth cards deform, sx concerning , abnormal findings , pulse, CXR or EKG

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13
Q

what are the acyantoic diseases

A

patent ductus arteriosus, ventricular septal defect, atrial septal defect

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14
Q

what is patent ductus arteriosus

A

failure of DA to close

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15
Q

what are sx of PDA

A

depends on shunt size, small may be asc, mod- large CHF, pulm htn

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16
Q

what does murmur sounds like for PDA

A

continous machine like murmur - at left intraclaviucalr area

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17
Q

how to dx PDA

A

echo

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18
Q

how to mang PDA

A

mod-severe - surgical or percutaneous closure

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19
Q

how to mang PDA in prematures

A

prostaglandsin inhibitors tried first

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20
Q

what is ventricular septal defect

A

common congenetial heart dx - 50% of all have this

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21
Q

what at sx of ventricular septal defect

A

diaphoresis, particular with feeds, difficulty feeding, failure to thrive, CHF< irrevesible pul vascular changes with 6-12 months

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22
Q

what is classic sign with VSD

A

holosytolic murmur heard best at the mid to LLSB

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23
Q

what is shown on EKG with VSD

A

LAE, LVH

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24
Q

what is shown on CXR for VSD

A

cardiomegaly, enlarged left ventricle, increased pulm vascular markings

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25
what confirms dx of VSD
echo
26
how to mang VSD: mild
oral diuretics
27
how to mang VSD: mod
lasix +/- aldactone, max caloric intake
28
how to mang VSD: severe
inpt, IV diuresis, max nutrtional support, stabilize condition for surgery
29
what are the differnt atrial septal defects (PFO)
primum, secundum, sinus venosus
30
what is primum in PFO
septum primum does not fuse with endocardial cushions, usu ass with other abnormalities
31
what is secundum in PFO
hole near the formaen oval, F>M, usu isolated, can be ass with genetic disorders
32
what is sinus venosus in PFO
malposition of insertion of vena cava
33
how does PFO present
asx early, normally present on PE, impact depends on size/locations, infants with large can present wtih CHF, FTT
34
what is classic finding with PFO
wide, fixed split S2, mid systolic ejection murmur
35
how to dx PFO
clinic suspicion, EKG may show incomplete R BBB
36
what is shown on CXR for PFO
cardiomegaly, increased pulm vascular markings
37
what confirms dx
echo
38
how to mag PFO
many spontaneously close <2 years, repiar
39
what can happen in 30-40s in PFO in left untx
right sided heart failure
40
when do you repair atrial septal defect (PFO)
if R heart enlargement, substantial shunt pulm over circulation
41
which type of atrial septal defect require more urgent surgery
primum and sinus venosus lesions
42
what are the acyantoic hearts
coarctation of the aorta, aortic stenosis, pulmonary stenosis
43
what is coarctation of aorta
narrowing of descending aorta, distal to LSCA
44
in females with coarctatin of aorta what else do you want to look for
turner syndrome
45
what at sx of coarctation of aorta
depends on degree of obstruction - classic findings: htn in UE, low/unobtainable BP in LEs, deceased/delayed femoral pulses, possible mumur caused by collateral blood flow
46
how to dx coarctation of aorta
prenatal difficult due to low C.O, output via aorta, ECHO
47
what is mang of coarctation of aorta
rapid initiation of prostaglandin E - once stable - surgery
48
what are sx of aortic stenosis
depends on degree, most or ASX, if severe -- central cyanosis and shock
49
what does murmur sound like for aortic stenosis
harsh systolic ejection murmur, systolic ejection click
50
how to DX aortic stenosis
PE and ECH, cardiac cath
51
how do you tx aortic stenosis
prostaglandin if critical, balloon vavluloplasty, surgery,
52
what can you see with aortic stenosis recurrently
obstruction and aortic regurg
53
what are sx of pulmonary stenosis
depend on degree of obstruciton mild to mod ususal asx, detected in childhood on routine visit
54
what does murmur sound like in pulm stenosis
normal 1st heat sound followed by a click, systolic ejection murmur at 2nd L ICS
55
what is dx of pulm stenosis
PE and ECHO, cardiac cath
56
what if sx of pulm stenosis
if severe - balloon valvuloplasty
57
what is cyanotic heart dz
right to left shunts, usually present in neonates
58
what are the 5 T's of cyanotic heart dz
truncus arteriosus, tranposition of great vessles, tricupsid, tetralogy of fallot, total anomalous pulmonary venous return
59
how do you dx cyanotic heart dz
echo, prenatally dx, confirm postnatal
60
how do you mang cyanotic heart dz
surgery, not always immediate may need to treat heart failure first
61
what is truncus arteriosus
single great vessels - always has associated VSD
62
what are sx of truncus arteriosus
cyanosis, resp distress/signs CHF, +/- murmur
63
hat does mumur sound like in truncus arteriosus
systolic ejection mumur with click, S2 is loud and single
64
when does truncus arteriosus develop heart failure
over days to weeks
65
how does transposition of great vessels present
cyanosis, may or may not have murmur
66
when does trasposition of great vessels present
newborn period most commonly
67
what can detect even mild cyanosis
oximetry
68
how do you tx transpositin of great vessels
prostaglandin to keep PDA intact, blloon atrial septostomy, surgery
69
what happens if transposition of great vessels is left untx
most die within 1st year of life
70
what is tricuspid atresia
abesent tricuspid valve - no communication between R atrium and ventricle
71
sx of tricuspid atresia
if dz prenatally transfer for deliver at heart center - immediate initital of prostaglandin, complex, stage surgery
72
what results in tetralogy of fallot
abnomral spetation of the truncys arteriosus into the aorta and pulmonary artery that occurs early in gestation
73
what are the 4 components of tetralogy
ventricular septal defect, pulmonary valve stenosis, overriding aorta, right ventricular hypertrophy
74
sx of tetralogy: severe
cyanosis - present with heart failure
75
sx of tetralogy: mod
murmur
76
sx of tetralogy: mild
may not be cyanotic, present with heart failure
77
what are tet spells
intermittent, sudden increased in cyanosis, restless, agitated, inconsolable, prolonged spells can lead to syncope, convulsions, stroke
78
what does mumur sound like in tetralogy
crescendo-decrescendo systolic murmur heard best in left mid to upper sternal border, single S2
79
what is shown on CXR of tetralogy
boot shaped heart
80
how do you tx tetralogy
surgery
81
what is total anomalous pulm venous return
none of the pulmonary veins connect to the left atrium, all return via systemic venous circulation
82
what are sx of total anomalous pulm venous return
depends on presence or absence of pulm venous obstruction
83
what are sx of severe total anomalous pulm venous return
cyanosis, shock, resp distress at birth
84
what are sx of unobstructed anamousl pulm venous return
subtle cyanosis, may be detected, heart failure
85
murmur of total anomalous pulm venous return
systolic ejection murmur, widely split S2
86
tx severe total anomalous pulm venous return
prostaglandin, other med stabilization
87
hypoplastic left heart syndrome
underdeveloped left herat, nomral great vessels, varying degree of valvular abnormalities
88
abnormalities of hypoplastic left heart syndrome
mitral and aortic valve atresia (most severe), combo of atresia and stenosis, varying degrees of stenosis
89
what is survival dependent on in hypoplastic left heart syndrome
PDA and ASD
90
sx of hypoplastic left heart syndrome
cyanosis, resp distress, usu no murmur, poor distal pulses, cool extremities
91
if no asd: hypoplastic left heart syndrome sx
severe resp distress, cyanosis, cardiogenic shock at birth
92
patent ductus and norestricitve ASD: hypoplastic left heart syndrome sx
may be OK while ductus is open and pulm resitant is still high, develop sx as ductus arteriosus closed and pulm vascular resistance falls
93
mang hypoplastic left heart syndrome initial
prostaglanidn E2 - keep PD open
94
defniitive mang of hypoplastic left heart syndrome
surgery
95
hypoplastic left heart syndrome survival
95% will die if untx

Pediatrics clin med (7 decks)

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